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Sample records for systemic al amyloidosis

  1. Systemic AL amyloidosis in a Beech Marten (Martes foina).

    PubMed

    Scaglione, F E; Mignone, W; Ferrero, E; Poggi, M; Biolatti, B; Bollo, E

    2013-10-01

    A wild Beech Marten (Martes foina), was referred for necropsy to the Department of Animal Pathology of the University of Turin (Italy). At gross examination, whitish and firm masses, 10-mm in diameter, were found on the heart and in the kidney. Spleen showed lighter color and greater consistency, and the cut surface of the liver appeared scattered with whitish-yellow coalescing foci homogeneously distributed. Amyloid deposits were present in the perivascular and intercellular spaces of the visceral organs, such as the heart, liver, and kidneys. Amyloid stained positively with Congo red with and without 5% potassium permanganate pretreatment and showed green birefringence observable under polarized light. A diagnosis of systemic AL amyloidosis was made. This is the first description of systemic AL amyloidosis in a wild Stone Marten.

  2. Primary Systemic Al Amyloidosis Presenting as Temporal Arteritis

    PubMed Central

    Emmungil, Hakan; Kalfa, Melike; Başarık, Burcu; Kahraman, Hasip; Tanhan, Ferhat; Yaman, Banu; Öztürk, Atiye; Erdemir, Zehra; Kandiloğlu, Gülşen; İnal, Vedat; Kabasakal, Yasemin

    2014-01-01

    Temporal arteritis is most common vasculitis in elderly and imitated by miscellaneous disorders. Temporal artery biopsy is the gold standard test in the diagnosis of giant cell arteritis (GCA). Hereby, we describe a case of a 67-year-old man who presented initially with temporal arteritis; however, a lip biopsy then revealed AL amyloidosis. In this respect, temporal artery biopsy should be performed for definitive diagnosis of GCA particularly patients with systemic symptoms and treatment resistant. PMID:24551471

  3. AL Amyloidosis and Agent Orange

    MedlinePlus

    ... for survivors' benefits . Research on AL amyloidosis and herbicides The Health and Medicine Division (formally known as ... to the compounds of interest found in the herbicide Agent Orange and AL amyloidosis." VA made a ...

  4. Clinical profile and treatment outcome of older (>75 years) patients with systemic AL amyloidosis

    PubMed Central

    Sachchithanantham, Sajitha; Offer, Mark; Venner, Christopher; Mahmood, Shameem A.; Foard, Darren; Rannigan, Lisa; Lane, Thirusha; Gillmore, Julian D.; Lachmann, Helen J.; Hawkins, Philip N.; Wechalekar, Ashutosh D.

    2015-01-01

    Systemic AL amyloidosis, a disease with improving outcomes using novel therapies, is increasingly recognized in the elderly but treatment and outcomes have not been systematically studied in this group of patients in whom comorbidities and frailty may compound morbidity and mortality. We report the outcomes of 295 patients with systemic AL amyloidosis ≥75 years seen at the UK National Amyloidosis Centre from 2005–2012. The median age was 78.5 years. The median overall survival was 20 months. Two hundred and thirty-eight patients received chemotherapy and 57 elected for supportive care only (overall survival – 24 and 8.4 months, respectively). On intention-to-treat analysis, 44% achieved a hematologic response including a very good partial response or better in 23%. The median overall survival was 6.2 years in patients achieving very good partial response or better at the 6-month landmark analysis and 1.5 years in non-responders. Factors independently indicating a poor prognosis were: cardiac involvement, performance status ≥2; systolic blood pressure <100 mmHg and, on landmark analysis, achieving less than a very good partial response. Treatment of systemic AL amyloidosis in the elderly is challenging. Deep clonal responses are associated with excellent survival and organ responses. Achieving a response to the first-line regimen appears particularly important as outcomes of non-responders are similar to those of untreated patients. Prospective trials with lower toxicity, outpatient treatment regimens are needed. PMID:26294730

  5. Systemic kappaAL amyloidosis associated with bovine leukocyte adhesion deficiency.

    PubMed

    Taniyama, H; Yamamoto, S; Sako, T; Hirayama, K; Higuchi, H; Nagahata, H

    2000-01-01

    Histopathologic and immunohistochemical examinations were conducted on a 5-year-old Holstein-Friesian cow with systemic kappaAL amyloidosis associated with bovine leukocyte adhesion deficiency. Amyloid deposits were present in the perivascular and intercellular spaces of the visceral organs, such as the liver, kidneys, pancreas, adrenal glands, and upper alimentary tract. Amyloid was stained positively with Congo red with or without 5% potassium permanganate pretreatment and had green birefringence observed under polarized light. Immunohistochemically, amyloid reacted strongly against anti-bovine IgG (H+L) and anti-bovine kappa-light chain and reacted weakly against bovine X-light chain antibodies but was negative for anti-human amyloid AA antibody. This is the first description of AL amyloidosis immunohistochemically related to immunoglobulin kappa-light chains of precursor protein in cattle. PMID:10643989

  6. 77 FR 6466 - Schedule for Rating Disabilities; AL Amyloidosis (Primary Amyloidosis)

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-02-08

    ... hemic and lymphatic systems to include AL amyloidosis. This regulatory action is necessary to add AL... lymphatic systems, 38 CFR 4.117, by creating diagnostic code 7717. The schedule previously did not contain a... amyloidosis because it was the first available diagnostic code in the Hemic and Lymphatic Systems listed...

  7. Systemic AA amyloidosis: epidemiology, diagnosis, and management

    PubMed Central

    Real de Asúa, Diego; Costa, Ramón; Galván, Jose María; Filigheddu, María Teresa; Trujillo, Davinia; Cadiñanos, Julen

    2014-01-01

    The term “amyloidosis” encompasses the heterogeneous group of diseases caused by the extracellular deposition of autologous fibrillar proteins. The global incidence of amyloidosis is estimated at five to nine cases per million patient-years. While amyloid light-chain (AL) amyloidosis is more frequent in developed countries, amyloid A (AA) amyloidosis is more common in some European regions and in developing countries. The spectrum of AA amyloidosis has changed in recent decades owing to: an increase in the median age at diagnosis; a percent increase in the frequency of primary AL amyloidosis with respect to the AA type; and a substantial change in the epidemiology of the underlying diseases. Diagnosis of amyloidosis is based on clinical organ involvement and histological evidence of amyloid deposits. Among the many tinctorial characteristics of amyloid deposits, avidity for Congo red and metachromatic birefringence under unidirectional polarized light remain the gold standard. Once the initial diagnosis has been made, the amyloid subtype must be identified and systemic organ involvement evaluated. In this sense, the 123I-labeled serum amyloid P component scintigraphy is a safe and noninvasive technique that has revolutionized the diagnosis and monitoring of treatment in systemic amyloidosis. It can successfully identify anatomical patterns of amyloid deposition throughout the body and enables not only an initial estimation of prognosis, but also the monitoring of the course of the disease and the response to treatment. Given the etiologic diversity of AA amyloidosis, common therapeutic strategies are scarce. All treatment options should be based upon a greater control of the underlying disease, adequate organ support, and treatment of symptoms. Nevertheless, novel therapeutic strategies targeting the formation of amyloid fibrils and amyloid deposition may generate new expectations for patients with AA amyloidosis. PMID:25378951

  8. Amyloidosis.

    PubMed

    Pinney, Jennifer H; Hawkins, Philip N

    2012-05-01

    The term amyloid describes the deposition in the extracellular space of certain proteins in a highly characteristic, insoluble fibrillar form. Amyloidosis describes the various clinical syndromes that occur as a result of damage by amyloid deposits in tissues and organs throughout the body. The clinical significance of amyloid varies enormously, ranging from incidental asymptomatic deposits to localized disease through to rapidly fatal systemic forms that can affect multiple vital organs. Currently available therapy is focused on reducing the supply of the respective amyloid fibril precursor protein and supportive medical care, which together have greatly improved survival. Chemotherapy and anti-inflammatory treatment for the disorders that underlie AL and AA amyloidosis are guided by serial measurements of the respective circulating amyloid precursor proteins, i.e. serial serum free light chains in AL and serum amyloid A protein in AA type. Quality of life and prognosis of some forms of hereditary systemic amyloidosis can be improved by liver and other organ transplants. Various new therapies, ranging from silencing RNA, protein stabilizers to monoclonal antibodies, aimed at inhibiting fibril precursor supply, fibril formation or the persistence of amyloid deposits, are in development; some are already in clinical phase. PMID:22402917

  9. 75 FR 65279 - Schedule for Rating Disabilities; AL Amyloidosis (Primary Amyloidosis)

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-10-22

    ... the schedule of ratings for the hemic and lymphatic systems to include AL amyloidosis. This regulatory... diagnostic code in the Hemic and Lymphatic Systems listed in Sec. 4.117. VA proposes to assign a 100 percent... diagnostic code 7716, to read as follows: Sec. 4.117 Schedule of ratings--hemic and lymphatic systems....

  10. Systemic transthyretin amyloidosis in a patient with bent spine syndrome.

    PubMed

    Rezania, Kourosh; Pytel, Peter; Smit, Laurel J; Mastrianni, James; Dina, Michelle A; Highsmith, W Edward; Dogan, Ahmet

    2013-06-01

    Wild-type and mutant transthyretin (TTR) are implicated in systemic amyloidosis (ATTR). Myopathy is a rare complication of ATTR amyloidosis, however no patient with bent spine syndrome secondary to ATTR amyloidosis has been reported so far. We present the first case of bent spine syndrome in a patient with wild-type ATTR amyloidosis who also had concomitant Alzheimer's disease.

  11. Long-term follow-up from a phase 1/2 study of single-agent bortezomib in relapsed systemic AL amyloidosis.

    PubMed

    Reece, Donna E; Hegenbart, Ute; Sanchorawala, Vaishali; Merlini, Giampaolo; Palladini, Giovanni; Bladé, Joan; Fermand, Jean-Paul; Hassoun, Hani; Heffner, Leonard; Kukreti, Vishal; Vescio, Robert A; Pei, Lixia; Enny, Christopher; Esseltine, Dixie-Lee; van de Velde, Helgi; Cakana, Andrew; Comenzo, Raymond L

    2014-10-16

    CAN2007 was a phase 1/2 study of once- and twice-weekly single-agent bortezomib in relapsed primary systemic amyloid light chain amyloidosis (AL) amyloidosis. Seventy patients were treated, including 18 and 34 patients at the maximum planned doses on the once- and twice-weekly schedules. This prespecified final analysis provides mature response and long-term outcomes data after 3-year additional follow-up since the last report. In the once-weekly 1.6 mg/m(2) and twice-weekly 1.3 mg/m(2) bortezomib groups, final hematologic response rates were 68.8% and 66.7%; 80% of patients in each group sustained their response for ≥1 year. One-year progression-free rates were 72.2% and 76.8%. Median overall survival (OS) was 62.1 months and not reached; 4-year OS rates were 75.0% and 63.0%. Low baseline difference in κ/λ free light-chain level was associated with higher hematologic complete response rates and longer OS. At data cutoff, 40 (57%) patients had received subsequent therapy, including 19 (27%) retreated with bortezomib, 11 (58%) of whom achieved complete or partial hematologic responses. Four patients received prolonged bortezomib for between 3.5 and 5.6 years, with no new safety concerns, highlighting the feasibility of long-term therapy. Single-agent bortezomib produced durable hematologic responses and promising long-term OS in relapsed AL amyloidosis. This trial was registered at www.clinicaltrials.gov as #NCT00298766. PMID:25202139

  12. Recurrent Syncope and Cardiac Arrest in a Patient with Systemic Light Chain Amyloidosis Treated with Bortezomib.

    PubMed

    Jaipaul, Navin; Pi, Alexander; Zhang, Zhiwei

    2016-05-10

    About 10-15% of patients with multiple myeloma develop light chain (AL) amyloidosis. AL amyloidosis is a systemic disease that may involve multiple organs, often including the heart. It may present clinically with bradyarrhythmia and syncope. The proteasome inhibitor bortezomib has been used with clinical efficacy in treating patients with AL amyloidosis but also implicated as a possible cause of cardiomyocyte injury. We report a case of a 48-year-old man with AL amyloidosis and increased frequency of syncope and cardiac arrest after starting bortezomib. The biologic and clinical plausibility of a heightened risk for cardiac arrest in patients with cardiac AL amyloidosis and history of syncope being treated with bortezomib is a possibility that is not well documented in the medical literature and warrants further investigation. PMID:27499835

  13. Recurrent Syncope and Cardiac Arrest in a Patient with Systemic Light Chain Amyloidosis Treated with Bortezomib

    PubMed Central

    Jaipaul, Navin; Pi, Alexander; Zhang, Zhiwei

    2016-01-01

    About 10-15% of patients with multiple myeloma develop light chain (AL) amyloidosis. AL amyloidosis is a systemic disease that may involve multiple organs, often including the heart. It may present clinically with bradyarrhythmia and syncope. The proteasome inhibitor bortezomib has been used with clinical efficacy in treating patients with AL amyloidosis but also implicated as a possible cause of cardiomyocyte injury. We report a case of a 48-year-old man with AL amyloidosis and increased frequency of syncope and cardiac arrest after starting bortezomib. The biologic and clinical plausibility of a heightened risk for cardiac arrest in patients with cardiac AL amyloidosis and history of syncope being treated with bortezomib is a possibility that is not well documented in the medical literature and warrants further investigation. PMID:27499835

  14. [Cardiac amyloidosis].

    PubMed

    Hoyer, Caroline; Angermann, Christiane E; Knop, Stefan; Ertl, Georg; Störk, Stefan

    2008-03-15

    Amyloidoses are a heterogeneous group of multisystem disorders, which are characterized by an extracellular deposition of amyloid fibrils. Typically affected are the heart, liver, kidneys, and nervous system. More than half of the patients die due to cardiac involvement. Clinical signs of cardiac amyloidosis are edema of the lower limbs, hepatomegaly, ascites and elevated jugular vein pressure, frequently in combination with dyspnea. There can also be chest pain, probably due to microvessel disease. Dysfunction of the autonomous nervous system or arrhythmias may cause low blood pressure, dizziness, or recurrent syncope. The AL amyloidosis caused by the deposition of immunoglobulin light chains is the most common form. It can be performed by monoclonal gammopathy. The desirable treatment therapy consists of high-dose melphalan therapy twice followed by autologous stem cell transplantation. Due to the high peritransplantation mortality, selection of appropriate patients is mandatory. The ATTR amyloidosis is an autosomal dominant disorder caused by the amyloidogenic form of transthyretin, a plasmaprotein that is synthesized in the liver. Therefore, liver transplantation is the only curative therapy. The symptomatic treatment of cardiac amyloidosis is based on the current guidelines for chronic heart failure according to the patient's New York Heart Association (NYHA) state. Further types of amyloidosis with possible cardiac involvement comprise the senile systemic amyloidosis caused by the wild-type transthyretin, secondary amyloidosis after chronic systemic inflammation, and the beta(2)-microglobulin amyloidosis after long-term dialysis treatment. PMID:18344065

  15. Stabilisation of Laryngeal AL Amyloidosis with Long Term Curcumin Therapy.

    PubMed

    Golombick, Terry; Diamond, Terrence H; Manoharan, Arumugam; Ramakrishna, Rajeev

    2015-01-01

    Multiple myeloma (MM), smoldering myeloma (SMM), and monoclonal gammopathy of undetermined significance (MGUS) represent a spectrum of plasma cell dyscrasias (PCDs). Immunoglobulin light chain amyloidosis (AL) falls within the spectrum of these diseases and has a mortality rate of more than 80% within 2 years of diagnosis. Curcumin, derived from turmeric, has been shown to have a clinical benefit in some patients with PCDs. In addition to a clinical benefit in these patients, curcumin has been found to have a strong affinity for fibrillar amyloid proteins. We thus administered curcumin to a patient with laryngeal amyloidosis and smoldering myeloma and found that the patient has shown a lack of progression of his disease for a period of five years. This is in keeping with our previous findings of clinical benefits of curcumin in patients with plasma cell dyscrasias. We recommend further evaluation of curcumin in patients with primary AL amyloidosis. PMID:26199769

  16. Stabilisation of Laryngeal AL Amyloidosis with Long Term Curcumin Therapy

    PubMed Central

    Golombick, Terry; Diamond, Terrence H.; Manoharan, Arumugam; Ramakrishna, Rajeev

    2015-01-01

    Multiple myeloma (MM), smoldering myeloma (SMM), and monoclonal gammopathy of undetermined significance (MGUS) represent a spectrum of plasma cell dyscrasias (PCDs). Immunoglobulin light chain amyloidosis (AL) falls within the spectrum of these diseases and has a mortality rate of more than 80% within 2 years of diagnosis. Curcumin, derived from turmeric, has been shown to have a clinical benefit in some patients with PCDs. In addition to a clinical benefit in these patients, curcumin has been found to have a strong affinity for fibrillar amyloid proteins. We thus administered curcumin to a patient with laryngeal amyloidosis and smoldering myeloma and found that the patient has shown a lack of progression of his disease for a period of five years. This is in keeping with our previous findings of clinical benefits of curcumin in patients with plasma cell dyscrasias. We recommend further evaluation of curcumin in patients with primary AL amyloidosis. PMID:26199769

  17. [Systemic amyloidosis secondary to xanthogranulomatous pyelonephritis].

    PubMed

    Almirall, J; López, T; Sáez, A; Gratacós, J; Prats, J

    2001-01-01

    Secondary amyloidosis is a relatively common pathology in which chronic infectious diseases are common causes, especially infected bronchiectasis, osteomyelitis or chronic ulcers. The association of xanthogranulomatous pyelonephritis and systemic amyloidosis is extremely rare. To our knowledge, despite innumerable cases of xanthogranulomatous pyelonephritis reported in the literature, this association has been reported in only 8 previous cases. Patients usually complain of fever, back or flank pain and urinary tract symptoms. A long lasting evolution of the process is frequent. We report a 70 year old patient who developed amyloidosis secondary to xanthogranulomatous pyelonephritis. As well as the rarity of this association, this case is exceptional in its clinical presentation, without any urinary tract symptoms that could suggest the diagnosis.

  18. Amyloidosis

    PubMed Central

    Khan, M; Falk, R

    2001-01-01

    Amyloidosis is not a single disease but a series of diseases in which there is extracellular deposition of a protein which, although it may be derived from different and unrelated sources, folds into a β pleated sheet.
There have recently been significant advances in elucidating the pathogenesis and in the treatment of this group of disorders. By identifying the source of precursor protein, treatment is aimed at eliminating or reducing the extent of deposition and is tailored for each patient.
Early diagnosis is required for the optimal effect of treatment on patient survival and quality of life. An increased awareness among physicians of the spectrum of the disease and tools to aid its diagnosis is of increasing importance.


Keywords: amyloidosis PMID:11677276

  19. Soluble suppression of tumorigenicity 2 (sST2), but not galactin-3, adds to prognostication in patients with systemic AL amyloidosis independent of NT-proBNP and troponin T.

    PubMed

    Dispenzieri, Angela; Gertz, Morie A; Saenger, Amy; Kumar, Shaji K; Lacy, Martha Q; Buadi, Francis K; Dingli, David; Leung, Nelson; Zeldenrust, Steven; Hayman, Suzanne R; Kapoor, Prashant; Grogan, Martha; Hwa, Lisa; Russell, Stephen J; Go, Ronald S; Rajkumar, S Vincent; Kyle, Robert A; Jaffe, Allan

    2015-06-01

    The use of soluble cardiac biomarkers such as N-terminal pro-brain natriuretic peptide (NT-proBNP) and troponin has revolutionized prognostication for patients with AL amyloidosis. Soluble ST2 (sST2) and galectin-3 have also been reported to have prognostic value in other cardiac patient populations. We identified 502 patients with AL amyloidosis, who provided a research sample and consent to review their medical records between 1/1/2006-12/31/2010 within 90 days of their diagnosis. Samples were assayed for sST2 and galectin-3. Within this AL amyloidosis population, overall survival (OS) was 25.5 months (95% CI 18, 35.7 months). Receiver operating curve analyses were done to detect the best cut-points for sST2 and galectin-3 to predict both 1- and 5-year OS. The respective cut points for sST2 were 30 and 29.7 ng/mL, while the median sST2 for the entire population was 31 ng/mL (IQR 19.8, 53.6). The respective cut points for galectin-3 were 11 and 10.4 ng/mL while the median for the entire population was 16.6 ng/mL (IQR 11.5, 24.0). Although on univariate analysis, both sST2 and galectin-3 were prognostic, upon multivariate analysis, only sST2 was independent of troponin, NT-proBNP, serum immunoglobulin free light chain, and blood pressure. Not only did sST2 add to previously reported prognostication systems, but a novel prognostication 5-point system including sST2 was possible. The addition of sST2 - but not galectin-3 - to existing prognostication systems for patients with AL amyloidosis strengthens the ability to predict for death.

  20. Bilateral Chronic Subdural Nontraumatic Hematoma in a Woman Affected by AL Amyloidosis

    PubMed Central

    Luongo, M.; Grassi, M.; Godano, U.

    2015-01-01

    Chronic subdural hematoma (CSDH) is a condition frequently seen in neurosurgical practice, especially among the elderly. It is often preceded by head injury, even a trivial trauma. Light chain (AL) amyloidosis is a disorder involving extracellular tissue deposition of misfolded native proteins called amyloids. The several types of amyloidosis differ by source of proteins, organ involvement, treatment, and prognosis. We describe the case of a 59-year-old woman affected by AL amyloidosis, harboring surgically treated bilateral chronic nontraumatic subdural hematoma, and we suggest a possible correlation between the clinical entities of CSDH and AL amyloidosis. PMID:26251797

  1. Idiopathic systemic AA-amyloidosis in a skunk (Mephitis mephitis).

    PubMed

    Elhensheri, Mohamed; Linke, Reinhold P; Blankenburg, Anja; Beineke, Andreas

    2012-03-01

    This report describes a case of systemic amyloidosis in a captive striped skunk. At necropsy, bilateral alopecia, as well as reno-, hepato-, and splenomegaly were present. Congo red staining and immunohistochemistry revealed depositions of AA-amyloid in different organs. The lack of a predisposing disease is suggestive of idiopathic systemic AA-amyloidosis. PMID:22448530

  2. Idiopathic systemic AA-amyloidosis in a skunk (Mephitis mephitis).

    PubMed

    Elhensheri, Mohamed; Linke, Reinhold P; Blankenburg, Anja; Beineke, Andreas

    2012-03-01

    This report describes a case of systemic amyloidosis in a captive striped skunk. At necropsy, bilateral alopecia, as well as reno-, hepato-, and splenomegaly were present. Congo red staining and immunohistochemistry revealed depositions of AA-amyloid in different organs. The lack of a predisposing disease is suggestive of idiopathic systemic AA-amyloidosis.

  3. Atypical immunoglobulin light chain amyloidosis

    PubMed Central

    Wu, Xia; Feng, Jun; Cao, Xinxin; Zhang, Lu; Zhou, Daobin; Li, Jian

    2016-01-01

    Abstract Background: Primary immunoglobulin light chain amyloidosis (AL amyloidosis) is a plasma cell disorder which mainly affects heart, kidneys, liver, and peripheral nervous system. Cases of atypical AL amyloidosis presented as spontaneous vertebral compression fractures have been rarely reported, and data about the management and clinical outcomes of the patients are scarce. Methods: Herein, we present 3 new cases of AL amyloidosis with spontaneous vertebral compression fracture and review 13 cases retrieved from the literature. Results: Moreover, we observed overrepresentations of liver involvement and bone marrow involvement in AL amyloidosis with spontaneous vertebral compression fracture. Conclusion: We believe that better awareness of the rare clinical presentation as spontaneous vertebral compression fracture of AL amyloidosis can facilitate earlier diagnosis and earlier treatment. PMID:27603350

  4. Neurologic complications of primary systemic amyloidosis.

    PubMed

    Kelly, John J

    2006-01-01

    Amyloid is a botanical term that denotes a waxy, amorphous, eosinophilic material and is used to describe similar pathological findings in humans. The deposits in primary systemic amyloidosis are derived from monoclonal serum proteins in plasma cell dyscrasia, which are degraded locally in tissues and deposited in insoluble sheets that damage organs. The cause of organ damage in amyloidosis is unclear but most likely due to the direct toxic effects of amyloid. Symptoms of the disorder include neuropathy, myopathy, and cardiac or renal insufficiency; there is often multiple-organ involvement. Congestive heart failure and nephrotic syndrome are predominant causes of death. Differential difficulties exist in diagnosing the disorder, and familial amyloid polyneuropathy directly mimics the disease. Diagnostic tools include electromyography, laboratory testing for abnormalities in serum and urine, and histological investigation of appropriate tissue. The median survival of patients is 2 years. However, melphalan and prednisone treatment for at least 1 year has resulted in increased survival rates. There have also been reports of benefit from high-dose chemotherapy followed by peripheral blood stem cell transplantation. Without early therapy, however, the disease has a dismal prognosis, and peripheral neuropathy usually persists or worsens despite therapy and improvement in other organs.

  5. Localized axillary milia en plaque: a rare cutaneous case presentation of systemic amyloidosis.

    PubMed

    Dickison, P; Howard, V; Wylie, B; Smith, S D

    2016-10-01

    Systemic AL amyloidosis is known to be associated with plasma cell dyscrasias, including multiple myeloma. The cutaneous manifestations of systemic AL amyloidosis are varied, but typically include waxy plaques or subcutaneous nodules. We report a woman who presented with bilateral eruptions of hyperpigmented plaques in her axillae, which were diagnosed as milia en plaque. She had a history of multiple myeloma, for which she was under the care of a haematologist. This is the first documented case, to our knowledge, of an eruption in the axillae being milia en plaque.

  6. Localized axillary milia en plaque: a rare cutaneous case presentation of systemic amyloidosis.

    PubMed

    Dickison, P; Howard, V; Wylie, B; Smith, S D

    2016-10-01

    Systemic AL amyloidosis is known to be associated with plasma cell dyscrasias, including multiple myeloma. The cutaneous manifestations of systemic AL amyloidosis are varied, but typically include waxy plaques or subcutaneous nodules. We report a woman who presented with bilateral eruptions of hyperpigmented plaques in her axillae, which were diagnosed as milia en plaque. She had a history of multiple myeloma, for which she was under the care of a haematologist. This is the first documented case, to our knowledge, of an eruption in the axillae being milia en plaque. PMID:27663153

  7. AL amyloidosis with temporal artery involvement simulates giant-cell arteritis.

    PubMed

    Audemard, Alexandra; Boutemy, Jonathan; Galateau-Salle, Françoise; Macro, Margaret; Bienvenu, Boris

    2012-03-01

    Light-chain (AL) amyloidosis may present with features suggesting vasculitis, including giant-cell arteritis (GCA). We describe a case of an 80-year-old man, who initially presented with bilateral jaw claudication, bi-temporal headache and arthralgia, however a temporal-artery biopsy then revealed AL amyloidosis. A diagnosis of AL amyloidosis complicating multiple myelome simulates GCA and polymyalgia rheumatica was established. The patient was successfully treated with melphalan and dexamethasone: the free kappa light chains decreased, the patient's jaw claudication and headache disappeared. Then we discuss similarities between GCA and AL amyloidosis and potential confusion in diagnosis. We suggest that, in patients with clinical features of GCA without any temporal-artery typical findings, specimens are stained with Congo red, which then results in a different diagnosis and treatment.

  8. AA amyloidosis associated with systemic-onset juvenile idiopathic arthritis.

    PubMed

    Saha, Abhijeet; Chopra, Yogiraj; Theis, Jason D; Vrana, Julie A; Sethi, Sanjeev

    2013-10-01

    We report a 12-year-old boy with nephrotic syndrome due to renal AA amyloidosis. The AA amyloidosis was associated with a 3-year history of systemic-onset juvenile idiopathic arthritis. The presence of serum amyloid A protein was confirmed by laser microdissection of Congo Red-positive glomeruli and vessels followed by liquid chromatography and tandem mass spectrometry; this analysis excluded hereditary and familial amyloidosis. Aggressive management of the systemic-onset juvenile idiopathic arthritis resulted in improvement in clinical and laboratory parameters. The case represents an unusual cause of nephrotic syndrome in children. Early diagnosis of renal amyloidosis and management of systemic-onset juvenile idiopathic arthritis is paramount to preventing progression of kidney disease.

  9. Clinical benefits of bortezomib-containing regimens for newly diagnosed AL amyloidosis with severe cardiac impairment.

    PubMed

    Tsukune, Yutaka; Yahata, Yuriko; Sasaki, Makoto; Hiki, Makoto; Tsutsui, Miyuki; Hamano, Yasuharu; Itoh, Seigo; Miyazaki, Tetsuro; Dohi, Tomotaka; Maruyama, Masaki; Gotoh, Akihiko; Komatsu, Norio

    2016-08-01

    Cardiac amyloid light-chain amyloidosis (AL amyloidosis) is a rare disease with a very poor prognosis, associated with plasma cell dyscrasias such as monoclonal gammopathy of undetermined significance and multiple myeloma. Though bortezomib-containing regimens have achieved high hematologic response rates, there are still few reports describing the outcomes of Japanese patients. Six patients with severe cardiac AL amyloidosis were treated with bortezomib-containing regimens. Involved free light chain (iFLC) decreased immediately in most of these cases. However, the condition of heart failure and N-terminal pro-B-type natriuretic peptide (NT-proBNP) worsened in the early phase of this treatment and then improved several months later. At 29 months, the median duration of follow-up (2-47months), all patients remain alive except one who died of sudden cardiac arrest. Bortezomib-containing regimens are considered to be among the effective treatments for severe cardiac AL amyloidosis. PMID:27599413

  10. Clinical benefits of bortezomib-containing regimens for newly diagnosed AL amyloidosis with severe cardiac impairment.

    PubMed

    Tsukune, Yutaka; Yahata, Yuriko; Sasaki, Makoto; Hiki, Makoto; Tsutsui, Miyuki; Hamano, Yasuharu; Itoh, Seigo; Miyazaki, Tetsuro; Dohi, Tomotaka; Maruyama, Masaki; Gotoh, Akihiko; Komatsu, Norio

    2016-08-01

    Cardiac amyloid light-chain amyloidosis (AL amyloidosis) is a rare disease with a very poor prognosis, associated with plasma cell dyscrasias such as monoclonal gammopathy of undetermined significance and multiple myeloma. Though bortezomib-containing regimens have achieved high hematologic response rates, there are still few reports describing the outcomes of Japanese patients. Six patients with severe cardiac AL amyloidosis were treated with bortezomib-containing regimens. Involved free light chain (iFLC) decreased immediately in most of these cases. However, the condition of heart failure and N-terminal pro-B-type natriuretic peptide (NT-proBNP) worsened in the early phase of this treatment and then improved several months later. At 29 months, the median duration of follow-up (2-47months), all patients remain alive except one who died of sudden cardiac arrest. Bortezomib-containing regimens are considered to be among the effective treatments for severe cardiac AL amyloidosis.

  11. AL (Light-Chain) Cardiac Amyloidosis: A Review of Diagnosis and Therapy.

    PubMed

    Falk, Rodney H; Alexander, Kevin M; Liao, Ronglih; Dorbala, Sharmila

    2016-09-20

    The amyloidoses are a group of protein-folding disorders in which ≥1 organ is infiltrated by proteinaceous deposits known as amyloid. The deposits are derived from 1 of several amyloidogenic precursor proteins, and the prognosis of the disease is determined both by the organ(s) involved and the type of amyloid. Amyloid involvement of the heart (cardiac amyloidosis) carries the worst prognosis of any involved organ, and light-chain (AL) amyloidosis is the most serious form of the disease. The last decade has seen considerable progress in understanding the amyloidoses. In this review, current and novel approaches to the diagnosis and treatment of cardiac amyloidosis are discussed, with particular reference to AL amyloidosis in the heart. PMID:27634125

  12. Survey on spontaneous systemic amyloidosis in aging mice.

    PubMed

    Majeed, S K

    1993-02-01

    The incidence of systemic amyloidosis in CD-1 mice (Charles River, caesarian derived) obtained from long term studies over more than a 15-year period is reported. The survey included samples of visceral organs, peripheral and central nervous tissues, bone and bone marrow. The total incidence in all mice of this survey did not show any clear evidence of a difference between males and females. Amyloidosis deposits were mainly seen in the stomach (glandular), heart, small intestines, kidney, liver, spleen, thyroid, parathyroid, adrenals, salivary glands and ovaries, but not in the brain, spinal cord, bone or bone marrow. The survey showed that amyloidosis in CD-1 mice was spontaneous, systemic and it is age-related. Amyloid deposition was extracellular and it stained positively with Congo Red and also stained positively with Oil Red O and Alcian blue. In general, amyloidosis in CD-1 mice, was higher in comparison with B6C3F (cross between C57BL/C6 NCRLB and C3H/HEN NCRLB, (bred by Charles River), CFLP strain (hysterectomy derived strain of Swiss origin) and MAGF: TIF (SPF). This survey also showed that spontaneous systemic amyloidosis in CD-1 mice, was one of the major factors contributory to death in aging CD-1 mice. PMID:8457241

  13. Systemic immunoglobulin light-chain amyloidosis presenting hematochezia as the initial symptom.

    PubMed

    Kon, Tetsuo; Nakagawa, Naoki; Yoshikawa, Fumitsugu; Haba, Kazunao; Kitagawa, Nagako; Izumi, Michihiro; Kumazaki, Setsuo; Ishida, Satoshi; Aikawa, Ryuichi

    2016-08-01

    Immunoglobulin light-chain (AL) amyloidosis is characterized by the deposition of insoluble fibrils composed of immunoglobulin light chains secreted by monoclonal plasma cells. Given the recent advances in the therapy of AL amyloidosis, it is important to diagnose this disease as early as possible. Herein, we describe the case of a 62-year-old man with hepatitis C virus (HCV)-related cirrhosis presenting with hematochezia. Colonoscopy showed multiple submucosal hematomas within the region ranging from the transverse colon to the sigmoid colon. Kappa immunoglobulin light-chain amyloid deposition was also detected. Bone marrow examination revealed a monoclonal abnormal plasma cell population. Thus, the patient was diagnosed with systemic immunoglobulin light-chain amyloidosis. The hematochezia was conservatively managed. However, because of liver failure caused by liver cirrhosis, the patient developed massive pleural effusion and died of respiratory failure. Postmortem examination revealed amyloid deposition in the esophagus, stomach, duodenum, ileum, descending colon, pancreas, heart, and lung. In these organs, amyloid deposition was limited to the vascular wall. We concluded that AL amyloidosis can present hematochezia arising from submucosal hematoma in the large colon before other systemic symptoms appear.

  14. Systemic immunoglobulin light-chain amyloidosis presenting hematochezia as the initial symptom.

    PubMed

    Kon, Tetsuo; Nakagawa, Naoki; Yoshikawa, Fumitsugu; Haba, Kazunao; Kitagawa, Nagako; Izumi, Michihiro; Kumazaki, Setsuo; Ishida, Satoshi; Aikawa, Ryuichi

    2016-08-01

    Immunoglobulin light-chain (AL) amyloidosis is characterized by the deposition of insoluble fibrils composed of immunoglobulin light chains secreted by monoclonal plasma cells. Given the recent advances in the therapy of AL amyloidosis, it is important to diagnose this disease as early as possible. Herein, we describe the case of a 62-year-old man with hepatitis C virus (HCV)-related cirrhosis presenting with hematochezia. Colonoscopy showed multiple submucosal hematomas within the region ranging from the transverse colon to the sigmoid colon. Kappa immunoglobulin light-chain amyloid deposition was also detected. Bone marrow examination revealed a monoclonal abnormal plasma cell population. Thus, the patient was diagnosed with systemic immunoglobulin light-chain amyloidosis. The hematochezia was conservatively managed. However, because of liver failure caused by liver cirrhosis, the patient developed massive pleural effusion and died of respiratory failure. Postmortem examination revealed amyloid deposition in the esophagus, stomach, duodenum, ileum, descending colon, pancreas, heart, and lung. In these organs, amyloid deposition was limited to the vascular wall. We concluded that AL amyloidosis can present hematochezia arising from submucosal hematoma in the large colon before other systemic symptoms appear. PMID:27318996

  15. Alopecia in Systemic Amyloidosis: Trichoscopic-Pathologic Correlation

    PubMed Central

    Miteva, Mariya; Wei, Erin; Milikowski, Clara; Tosti, Antonella

    2015-01-01

    Alopecia in systemic amyloidosis is very rare and has been described as individual cases of diffuse nonscarring alopecia and a case of alopecia universalis. We report the trichoscopic findings in alopecia associated with systemic amyloidosis. The most prominent feature was a salmon colored halo (0.3-1 mm in diameter) surrounding the follicular ostia. Other features included broken hairs and black dots. The salmon colored halo correlated on pathology with the perifollicular deposition of amyloid. The horizontal sections showed that the sebaceous glands were preserved which supports the nonscarring pattern of the alopecia. PMID:26903748

  16. Presumptive service connection for disease associated with exposure to certain herbicide agents: AL amyloidosis. Final rule.

    PubMed

    2009-05-01

    This document amends the Department of Veterans Affairs (VA) adjudication regulations concerning presumptive service connection for a certain disease based on the most recent National Academy of Sciences (NAS) Institute of Medicine committee report, "Veterans and Agent Orange: Update 2006" (Update 2006). This amendment is necessary to implement a decision of the Secretary of Veterans Affairs that there is a positive association between exposure to herbicides used in the Republic of Vietnam during the Vietnam era and the subsequent development of AL amyloidosis. The intended effect of this amendment is to establish presumptive service connection for AL amyloidosis based on herbicide exposure. PMID:19507326

  17. Familial and primary (AL) cardiac amyloidosis: echocardiographically similar diseases with distinctly different clinical outcomes.

    PubMed Central

    Dubrey, S. W.; Cha, K.; Skinner, M.; LaValley, M.; Falk, R. H.

    1997-01-01

    OBJECTIVE: To determine whether patients with myocardial amyloidosis due either to AL (primary) amyloid or familial amyloid have distinguishing echocardiographic or electrocardiographic features; and to compare the prevalence of heart failure and survival in the two types of amyloidosis in relation to echocardiographic findings. DESIGN: Blinded group comparison of randomly selected cases of cardiac amyloidosis. SETTING: International referral centre for amyloid research and treatment. PATIENTS: 36 patients with cardiac amyloid heart disease, of whom 12 had familial and 24 had primary AL amyloidosis. RESULTS: Familial and AL echocardiograms were morphologically indistinguishable, with similar left ventricular wall thickness, mean (SD) 15.4 (2.3) nu 15.8 (2.5) mm, respectively; right ventricular wall thickness was also similar between amyloid types: 9.6 (2.8) nu 9.7 (6.5) mm, respectively. Doppler indices of left and right ventricular function, left ventricular volume, and ejection fraction were also similar. Low voltage electrocardiograms (< 0.5 mV) were more common in the AL (16/24, 67%) than in the familial group (4/12, 25%), P < 0.05. The one year survival for familial and AL forms was 92% (11/12) nu 38% (6/24), respectively, with virtually all deaths due to cardiac causes. CONCLUSIONS: Although cardiac involvement is echocardiographically indistinguishable, cardiac mortality is very different between the two forms of amyloidosis. Preservation of electrocardiographic voltage in familial amyloidosis suggests that the particular biochemical characteristics of distinct types of amyloid fibril have different pathological effects on the myocardium. This distinction becomes critical in the evaluation, treatment, and management of patients who have a diagnosis within the spectrum of the protein deposition diseases. Images PMID:9290406

  18. Short- and long-term outcomes of AL amyloidosis patients admitted into intensive care units.

    PubMed

    Guinault, Damien; Canet, Emmanuel; Huart, Antoine; Jaccard, Arnaud; Ribes, David; Lavayssiere, Laurence; Venot, Marion; Cointault, Olivier; Roussel, Murielle; Nogier, Marie-Béatrice; Pichereau, Claire; Lemiale, Virginie; Arnulf, Bertrand; Attal, Michel; Chauveau, Dominique; Azoulay, Elie; Faguer, Stanislas

    2016-09-01

    Amyloidosis is a rare and threatening condition that may require intensive care because of amyloid deposit-related organ dysfunction or therapy-related adverse events. Although new multiple myeloma drugs have dramatically improved outcomes in AL amyloidosis, the outcomes of AL patients admitted into intensive care units (ICUs) remain largely unknown. Admission has been often restricted to patients with low Mayo Clinic staging and/or with a complete or very good immunological response at admission. In a retrospective multicentre cohort of 66 adult AL (n = 52) or AA (n = 14) amyloidosis patients, with similar causes of admission to an ICU, the 28-d and 6-month survival rates of AA patients were significantly higher compared to AL patients (93% vs. 60%, P = 0·03; 71% vs. 45%, P = 0·02, respectively). In AL patients, the simplified Index of Gravity Score (IGS2) was the only independent predictive factor for death by day 28, whereas the Mayo-Clinic classification stage had no influence. In Cox's multivariate regression model, only cardiac arrest and on-going chemotherapy at ICU admission significantly predicted death at 6 months. Short-term outcomes of AL patients admitted into an ICU were mainly related to the severity of the acute medical condition, whereas on-going chemotherapy for active amyloidosis impacted on long-term outcomes.

  19. Cardiac Amyloidosis

    MedlinePlus

    ... abbreviation AL stands for Amyloidosis formed from Light chains, and it is a disease of the bone ... proteins that make up antibodies, known as light chains. These light chains circulate in the blood and ...

  20. [Amyloidosis: Up-to-date].

    PubMed

    Magy-Bertrand, N

    2016-08-01

    Amyloidosis is mainly a systemic disease belonging to protein-folding diseases. The past 10 years have shown significant progress in typing and the clinical management of amyloidosis, in the identification of novel prognostic markers for risk-stratification, and also in the development of new therapeutic agents. Biological molecular techniques are now able to type amyloidosis which were unidentified. Cardiac MRI and biomarkers allow a precise risk-stratification, especially in AL amyloidosis. If necessary, this prognostic evaluation may lead to rapid changes in the chemotherapy treatment. Emerging treatments rely on biotherapies, gene therapy, immunotherapy and blocking analogous agents. They give hope about an increase of survival of patients with systemic amyloidosis.

  1. Bilateral kidney infarction due to primary Al amyloidosis: a first case report.

    PubMed

    Mihout, Fabrice; Joseph, Laure; Brocheriou, Isabelle; Leblond, Véronique; Varnous, Shaïda; Ronco, Pierre; Plaisier, Emmanuelle

    2015-05-01

    Primary Amyloid Light-chain (AL) amyloidosis is a rare form of plasma cell dyscrasia characterized by tissue deposition of monoclonal immunoglobulin light chain. Kidney involvement is the most frequent manifestation, and patients usually present with glomerular disease.We report an exceptional case of bilateral kidney infarcts caused by AL amyloidosis. A 34-years-old man presented with progressive dyspnea, loin pain, recurrent macroscopic hematuria, and acute kidney injury. Computed tomography showed bilateral kidney infarcts.The diagnosis of AL amyloidosis was established on the kidney biopsy with the characterization of major vascular amyloid deposits that selectively stained with antilambda light chain antibody. An amyloid restrictive cardiomyopathy was also present, responsible for the life-threatening conduction disturbance, but without patent cardioembolic disease. The patient then underwent emergency heart transplantation, followed by a conventional chemotherapy with bortezomib, melphalan, and dexamethasone. More than 3 years later, the patient has subnormal renal function, a well-functioning heart transplant, and a sustained hematologic response.In addition to the very uncommon presentation, this case illustrates the tremendous progress that has occurred in the management of severe forms of AL amyloidosis.

  2. Bilateral kidney infarction due to primary Al amyloidosis: a first case report.

    PubMed

    Mihout, Fabrice; Joseph, Laure; Brocheriou, Isabelle; Leblond, Véronique; Varnous, Shaïda; Ronco, Pierre; Plaisier, Emmanuelle

    2015-05-01

    Primary Amyloid Light-chain (AL) amyloidosis is a rare form of plasma cell dyscrasia characterized by tissue deposition of monoclonal immunoglobulin light chain. Kidney involvement is the most frequent manifestation, and patients usually present with glomerular disease.We report an exceptional case of bilateral kidney infarcts caused by AL amyloidosis. A 34-years-old man presented with progressive dyspnea, loin pain, recurrent macroscopic hematuria, and acute kidney injury. Computed tomography showed bilateral kidney infarcts.The diagnosis of AL amyloidosis was established on the kidney biopsy with the characterization of major vascular amyloid deposits that selectively stained with antilambda light chain antibody. An amyloid restrictive cardiomyopathy was also present, responsible for the life-threatening conduction disturbance, but without patent cardioembolic disease. The patient then underwent emergency heart transplantation, followed by a conventional chemotherapy with bortezomib, melphalan, and dexamethasone. More than 3 years later, the patient has subnormal renal function, a well-functioning heart transplant, and a sustained hematologic response.In addition to the very uncommon presentation, this case illustrates the tremendous progress that has occurred in the management of severe forms of AL amyloidosis. PMID:25929920

  3. Primary systemic amyloidosis as a real diagnostic challenge – case study

    PubMed Central

    Jerzykowska, Sonia; Gil, Lidia A.; Balcerzak, Andrzej; Pupek-Musialik, Danuta; Komarnicki, Mieczysław A.

    2014-01-01

    Primary amyloidosis (AL) is a rare variety of plasma cell dyscrasia, the diagnosis of which is often difficult to establish. Pathogenesis of amyloidosis involves extracellular deposition of insoluble protein fibrils in tissues, leading to insufficiency of affected organs. According to various sources, mean survival rate of patients with primary amyloidosis ranges from 12 to 24 months, making primary amyloidosis a disease with a very poor prognosis. Survival rate is significantly lowered in case of cardiac manifestation of amyloidosis (about 6 months survival in untreated patients). In recent years a considerable progress in AL treatment has been observed. Nowadays we are able not only to delay progression of amyloidosis, but also to improve the function of the affected organs. Unfortunately as first signs and symptoms of AL are usually nonspecific, the diagnosis of AL is often delayed, resulting in late introduction of optimal therapy. There are many diagnostic tests which can be used in diagnostic process of amyloidosis, i.e. electrophoresis, serum and urine immunofixation or affected organs and bone marrow biopsy. On establishing the diagnosis in a patient with suspected amyloidosis it should be remembered that particular diagnostic methods vary considerably in sensitivity. The aim of this paper is to present a case report of a 27-year-old patient with primary amyloidosis focusing on diagnostic aspect of this condition. On the basis of this case, the authors would like to emphasize the value of precise diagnostic process, with immunological techniques playing undoubtedly a crucial role. PMID:26155101

  4. Systemic amyloidosis: unusual presentation mistaken for a recurrent scabies infection.

    PubMed

    Haley, Erin M; Nabatian, Adam S; Kopp, Sandra A; Falasca, Gerald F; Haupt, Helen M; Halpern, Analisa V

    2014-06-01

    We report the case of a 63-year-old woman with a history of undifferentiated connective-tissue disease, polyarthritis, and bilateral carpal tunnel syndrome who presented with generalized pruritus and erythematous and excoriated papules on the trunk and extremities. Empiric scabies treatment was unsuccessful. Patch testing and T-cell receptor gene rearrangement studies were unremarkable. The patient was found to have mild interstitial lung disease and hypogammaglobulinemia. Eventually a diagnosis of primary systemic amyloidosis was made after she developed frank lingual hypertrophy despite normal initial serum protein electrophoresis and negative abdominal fat pad aspiration. Diagnosis was confirmed with lingual biopsy. This case demonstrates an unusual presentation of primary systemic amyloidosis consisting of arthritis and intense debilitating pruritus without primary skin lesions for a full year prior to diagnosis of multiple myeloma. The patient responded to treatment with chemotherapy and corticosteroids.

  5. Primary amyloidosis

    MedlinePlus

    ... Swelling in the arms and legs Weak hand grip Weight loss Other symptoms that may occur with ... kidney involvement may lead to organ failure and death. Body-wide ( systemic ) amyloidosis can lead to death ...

  6. Amyloidosis involving the respiratory system: 5-year's experience of a multi-disciplinary group's activity.

    PubMed

    Scala, Raffaele; Maccari, Uberto; Madioni, Chiara; Venezia, Duccio; La Magra, Lidia Calogera

    2015-01-01

    Amyloidosis may involve the respiratory system with different clinical-radiological-functional patterns which are not always easy to be recognized. A good level of knowledge of the disease, an active integration of the pulmonologist within a multidisciplinary setting and a high level of clinical suspicion are necessary for an early diagnosis of respiratory amyloidosis. The aim of this retrospective study was to evaluate the number and the patterns of amyloidosis involving the respiratory system. We searched the cases of amyloidosis among patients attending the multidisciplinary rare and diffuse lung disease outpatients' clinic of Pulmonology Unit of the Hospital of Arezzo from 2007 to 2012. Among the 298 patients evaluated during the study period, we identified three cases of amyloidosis with involvement of the respiratory system, associated or not with other extra-thoracic localizations, whose diagnosis was histo-pathologically confirmed after the pulmonologist, the radiologist, and the pathologist evaluation. Our experience of a multidisciplinary team confirms that intra-thoracic amyloidosis is an uncommon disorder, representing 1.0% of the cases of rare and diffuse lung diseases referred to our center. The diagnosis of the disease is not always easy and quick as the amyloidosis may involve different parts of the respiratory system (airways, pleura, parenchyma). It is therefore recommended to remind this orphan disease in the differential diagnosis of the wide clinical scenarios the pulmonologist may intercept in clinical practice.

  7. Primary Systemic Amyloidosis with Unusual Dermatological Manifestations: A Rare Case Report

    PubMed Central

    Vyas, Kapil; Morgaonkar, Manjaree; Gupta, Savera; Jain, Suresh Kumar

    2016-01-01

    Amyloidosis is a group of heterogeneous diseases characterized by pathological deposition of proteinaceous substance extracellularly in various tissues. The clinical presentation depends on the site of amyloid deposition, with predominant involvement of mesenchymal elements and cutaneous findings in 30–40% of patients in case of primary systemic amyloidosis. We present a case of idiopathic primary systemic amyloidosis presenting with an unusual finding of nodulo-ulcerative lesion over tongue along with multiple skin-colored nodules, mimicking squamous cell carcinoma of tongue with secondary cutaneous metastasis, as well as lacking the classical presentation of purpura, macroglossia, waxy papules, and plaques. PMID:27057028

  8. Primary localized cutaneous amyloidosis with lichen and poikiloderma-like lesions and an excellent response to systemic acitretin*

    PubMed Central

    MA, Han; Su, Xiangyang; Zhu, Guoxing; Yin, Songchao; Lu, Chun; Lai, Wei

    2016-01-01

    Primary localized cutaneous amyloidosis is a skin-limited amyloidosis that does not involve internal organs. It is clinically subclassified into 3 general categories and some rare variants. However, there is considerable overlap within the classification. Though there are a variety of therapeutic measures, the treatment is often unsatisfactory, particularly when the disease is severe and extensive. We describe a rare case of primary localized cutaneous amyloidosis with lichen and poikiloderma-like lesions that showed an excellent response to systemic acitretin.

  9. Malnutrition, pharmaconutrition and other considerations in AL amyloidosis, a rare disease with masquerading symptoms and usually delayed diagnosis.

    PubMed

    Franco-López, Ángeles; Culebras, Jesús M

    2015-06-01

    Amyloid Light-chain (AL) amyloidosis is a very rare disease. Nutritional and pharmaconutrional aspects are described. Nutrition repletion of malnourished AL patients is an essential strategy for improving treatment efficacy and clinical outcomes. Early diagnosis of AL amiloidosis is difficult to establish due to the fact that signs and symptoms appearing mimic other processes that delay the final correct histological diagnosis. Untreated patients with this disease have a dismal outcome, with a median survival of 10-14 months from diagnosis. The sooner the treatment is established the better the results are. Modern chemotherapeutical agents, based primarily in cyclophosphamide, bortethomid and dexametasone, produce a rapid, deep, and durable response in the majority of patients. Autologous stem cell transplantation remains restricted to selected patients who are generally without advanced cardiac amyloidosis.

  10. Bortezomib-based induction for transplant ineligible AL amyloidosis and feasibility of later transplantation.

    PubMed

    Cornell, R F; Zhong, X; Arce-Lara, C; Atallah, E; Blust, L; Drobyski, W R; Fenske, T S; Pasquini, M C; Rizzo, J D; Saber, W; Hari, P N

    2015-07-01

    Recent studies support the use of bortezomib-based therapies in light chain amyloidosis (AL). We performed a retrospective analysis of the safety, efficacy and long-term survival (median follow-up 3 years) after bortezomib-based treatment in 28 consecutive patients with de novo AL deemed ineligible at initial presentation. The first 14 patients received bortezomib and dexamethasone (VD), and the second 14 patients received cyclophosphamide, bortezomib and dexamethasone (CVD; CyBorD). Both regimens were well tolerated with no treatment-related mortality. The overall hematological response (HR) rate was 93% in both the groups. Median time to response was shorter in the CVD group (39 days vs 96 days in the VD group; P=0.002). Hematological and organ responses induced with bortezomib-based therapy enabled 8 (33%) of initially transplant ineligible patients to undergo autologous hematopoietic stem cell transplantation (AHCT), including 4 patients with cardiac stage III or IV. Seven of the eight patients (88%) who underwent subsequent AHCT achieved sustained HR at a median of 33 months posttransplant. These data suggest that bortezomib-based induction followed by AHCT is a viable therapeutic strategy for transplant-ineligible AL. Larger, multicenter prospective trials are necessary to confirm our findings.

  11. Experimental murine amyloidosis: a model system for studying amyloid formation.

    PubMed Central

    Baumal, R.; Wilson, B.; Pass, E.

    1975-01-01

    Myeloma-associated and casein-induced murine amyloidosis were used as models to study the role of lymphocytes and macrophages in amyloid formation. Amyloidosis occurred rarely and in small amounts in Balb/C mice with immunoglobulin (Ig)-producing myeloma tumours but large amounts could be induced by injections of casein. Fluorescent staining of both forms of amyloid deposits by means of anti-casein- and anti-myeloma-amyloid antibodies indicated that they either crossreacted or coexisted. Nor abnormality of Ig biosynthesis was detected in amyloidosis, suggesting that abnormal degradation was responsible for production of the Ig form of amyloid. Although spleen lymphocytes of casein-injected mice with amyloidosis demonstrated diminished cellular immunologic responses, this did not indicate generalized immunologic incompetence. The non-Ig form of amyloid in casein-injected mice was shown to be produced by macrophages, and a technique was developed for increasing the yield of amyloid-containing cells. Images FIG. 1 FIG. 2 FIG. 3 FIG. 6 FIG. 7 FIG. 8 PMID:1080430

  12. Rationale, application and clinical qualification for NT-proBNP as a surrogate end point in pivotal clinical trials in patients with AL amyloidosis

    PubMed Central

    Merlini, G; Lousada, I; Ando, Y; Dispenzieri, A; Gertz, M A; Grogan, M; Maurer, M S; Sanchorawala, V; Wechalekar, A; Palladini, G; Comenzo, R L

    2016-01-01

    Amyloid light-chain (LC) amyloidosis (AL amyloidosis) is a rare and fatal disease for which there are no approved therapies. In patients with AL amyloidosis, LC aggregates progressively accumulate in organs, resulting in organ failure that is particularly lethal when the heart is involved. A significant obstacle in the development of treatments for patients with AL amyloidosis, as well as for those with any disease that is rare, severe and heterogeneous, has been satisfying traditional clinical trial end points (for example, overall survival or progression-free survival). It is for this reason that many organizations, including the United States Food and Drug Administration through its Safety and Innovation Act Accelerated Approval pathway, have recognized the need for biomarkers as surrogate end points. The international AL amyloidosis expert community is in agreement that the N-terminal fragment of the pro-brain natriuretic peptide (NT-proBNP) is analytically validated and clinically qualified as a biomarker for use as a surrogate end point for survival in patients with AL amyloidosis. Underlying this consensus is the demonstration that NT-proBNP is an indicator of cardiac response in all interventional studies in which it has been assessed, despite differences in patient population, treatment type and treatment schedule. Furthermore, NT-proBNP expression is directly modulated by amyloidogenic LC-elicited signal transduction pathways in cardiomyocytes. The use of NT-proBNP will greatly facilitate the development of targeted therapies for AL amyloidosis. Here, we review the data supporting the use of NT-proBNP, a biomarker that is analytically validated, clinically qualified, directly modulated by LC and universally accepted by AL amyloidosis specialists, as a surrogate end point for survival. PMID:27416985

  13. Systemic Amyloid A Amyloidosis in Island Foxes (Urocyon littoralis): Severity and Risk Factors.

    PubMed

    Gaffney, P M; Witte, C; Clifford, D L; Imai, D M; O'Brien, T D; Trejo, M; Liberta, F; Annamalai, K; Fändrich, M; Masliah, E; Munson, L; Sigurdson, C J

    2016-05-01

    Systemic amyloid A (AA) amyloidosis is highly prevalent (34%) in endangered island foxes (Urocyon littoralis) and poses a risk to species recovery. Although elevated serum AA (SAA) from prolonged or recurrent inflammation predisposes to AA amyloidosis, additional risk factors are poorly understood. Here we define the severity of glomerular and medullary renal amyloid and identify risk factors for AA amyloidosis in 321 island foxes necropsied from 1987 through 2010. In affected kidneys, amyloid more commonly accumulated in the medullary interstitium than in the glomeruli (98% [n= 78 of 80] vs 56% [n= 45], respectively;P< .0001), and medullary deposition was more commonly severe (19% [n= 20 of 105]) as compared with glomeruli (7% [n= 7];P= .01). Univariate odds ratios (ORs) of severe renal AA amyloidosis were greater for short- and long-term captive foxes as compared with free-ranging foxes (ORs = 3.2, 3.7, respectively; overall P= .05) and for females as compared with males (OR = 2.9;P= .05). Multivariable logistic regression revealed that independent risk factors for amyloid development were increasing age class (OR = 3.8;P< .0001), San Clemente Island subspecies versus San Nicolas Island subspecies (OR = 5.3;P= .0003), captivity (OR = 5.1;P= .0001), and nephritis (OR = 2.3;P= .01). The increased risk associated with the San Clemente subspecies or captivity suggests roles for genetic as well as exogenous risk factors in the development of AA amyloidosis. PMID:26419399

  14. [The concurrence of light-chain deposition disease, AL-amyloidosis, and cast nephropathy in a patient with multiple myeloma].

    PubMed

    Rekhtina, I G; Zakharova, E V; Stoliarevich, E S; Sinitsina, M N; Denisova, E N

    2015-01-01

    Despite of the fact that their clinical manifestations are similar, AL-amyloidosis (AL-A) and light chain deposition disease (LCDD) are individual nosological entities in view of considerable differences in their pathogenesis and pathomorphology. The paper describes a rare case of the concurrence of LCDD and AL-A in a patient with multiple myeloma. Clinically, there was dialysis-dependent renal failure, flail leg syndrome, myocardiopathy, and rhabdomyolysis. At the disease onset, his nephrobiopsy specimen could diagnose LCDD and myeloma or cast nephropathy. The disease was characterized by an aggressive course. Despite the administration of innovative agents, the patient had a short-term remission and died from disease progression. Autopsy additionally revealed amyloid deposition in the heart and kidney. The development of AL-A in the presence of prior LCDD may reflect the progression of the tumor and the appearance of an additional subclone of plasma cells that produce amyloidogenic light chains. The uncommonness of this case is that renal amyloid was found in the tubular casts and absent in the glomeruli, which may be considered as a special form--tubular AL-amyloidosis. PMID:26281203

  15. Histomorphological patterns of renal amyloidosis: a correlation between histology and chemical type of amyloidosis.

    PubMed

    Looi, L M; Cheah, P L

    1997-07-01

    A retrospective study was conducted to investigate whether there was a correlation between the histological pattern of renal amyloidosis, the chemical type of amyloid protein involved and the clinical presentation. Eighteen consecutive cases of systemic amyloidosis that had renal biopsies processed and examined histopathologically at the Department of Pathology, Faculty of Medicine, University of Malaya, Kuala Lumpur were reviewed. The age range of patients was 25 to 64 yrs (mean, 46 yrs). The male:female ratio was 2.6:1. Three patients were Malay, 9 Chinese, 3 Indian, 1 Indonesian, 1 Iban, and 1 Bisaya. According to the predominant site of amyloid deposition, 14 cases showed a glomerular pattern and 4 a vascular pattern. 8 cases were designated as 2 anti-human amyloid-A (AA) amyloidosis on the basis of permanganate-sensitivity and immunoreactivity of deposits with anti-human AA protein antibody. Ten cases contained deposits that were permanganate-resistant and nonimmunoreactive for AA protein and were designated as AL in type. The histomorphologic pattern of renal amyloidosis did not provide a reliable means of differentiating AA from AL amyloidosis. The glomerular pattern tended to present with renal manifestations such as nephrotic syndrome and chronic renal failure, whereas the vascular pattern tended to present with nonrenal manifestations such as diarrhoea. These findings may have a bearing on the pathophysiology of amyloidosis and provide clues to appropriate management. PMID:9224755

  16. Metabolic studies of radioiodinated serum amyloid P component in normal subjects and patients with systemic amyloidosis.

    PubMed Central

    Hawkins, P N; Wootton, R; Pepys, M B

    1990-01-01

    125I-Serum amyloid P component (SAP), injected intravenously into 10 normal subjects, remained predominantly intravascular with mean (SD) T1/2 (half time) in plasma of 24.5 (5.9) h. The fractional catabolic rate of 68 (19)% of the plasma pool per day was more rapid than other reported human plasma proteins. All radioactivity was excreted in the urine by 14 d. In 16 patients with monoclonal gammopathy or chronic inflammatory diseases, but without amyloidosis, 125I-SAP metabolism was normal. However, among 45 patients with biopsy-proven systemic amyloidosis (25, amyloid A type; 20, amyloid L type), 125I-SAP was cleared from the plasma more rapidly, accumulated in the amyloid deposits, and persisted there. The T1/2 in amyloid, measured directly with 131I-SAP, was 24 d. Repeat studies after 6-18 mo were notably consistent in normals but changed significantly in amyloid patients, generally correlating with clinical signs of disease progression. Measurements of 125I-SAP turnover may thus be of value for diagnosis and monitoring of amyloidosis. Analysis of SAP metabolism in amyloidosis suggests that plasma SAP is in dynamic equilibrium with a very large amyloid pool, and in two autopsies the total mass of SAP in the amyloid deposits was 2,100 and 21,000 mg, respectively. Images PMID:2254450

  17. Serum transthyretin levels in senile systemic amyloidosis: effects of age, gender and ethnicity.

    PubMed

    Buxbaum, Joel; Koziol, James; Connors, Lawreen H

    2008-12-01

    Serum transthyretin (TTR) levels are reduced in familial amyloidotic polyneuropathy (FAP). A single study of patients with senile systemic amyloidosis (SSA) in Sweden found that those individuals also had a significantly lower mean serum TTR concentration than age- and gender-matched controls. To determine if the same phenomenon prevailed in an ethnically more heterogeneous population, we compared the serum TTR levels, as determined by ELISA, in 45 documented SSA patients with congestive heart failure, 20 AL patients with congestive heart failure and population controls. Serum TTR concentrations in the controls were influenced in a statistically significant manner by age, gender and ethnicity. Although it is unlikely that such differences are clinically relevant, they must be considered when assessing the meaning of serum TTR concentrations in any clinically defined population. The serum concentrations in patients with SSA did not differ from age, gender and ethnically matched controls or from a group of AL patients with significant clinical cardiac involvement. We also compared TTR concentrations in 12 African-Americans carrying the TTR V122I allele with those in 826 African-Americans who were homozygous wild type at the TTR locus. The TTR V122I carriers had significantly lower serum TTR concentrations than appropriate controls even though the majority of such individuals had not reached the age of clinical or anatomic risk, i.e. over 60. Thus, as in carriers of other TTR mutations the serum TTR level is lower than normal, despite having a much later appearance of clinical disease. PMID:19065297

  18. [Light Chain Amyloidosis: an Update for Treatment].

    PubMed

    Shen, Kai-Ni; Li, Jian

    2015-06-01

    Systemic light chain amyloidosis (AL amyloidosis) is the most common type of amyloidosis, in which deposition of misfolded monoclonal light chain secreted by underlying clonal plasma cells leads to organ dysfunction. Tissue biopsy of involved organ is needed to confirm the type of amyloid deposits, thus proper treatment could be applied. Laser microdissection followed by mass spectrometry, performed on formalin-fixed paraffin-embedded specimens, has been proven superior to traditional methods on accurate diagnosis of amyloidosis. Prognosis depends on the extent of cardiac involvement. The Mayo staging system using NT-ProBNP, cardiac troponin-T and free light chain, is the most robust method for risk stratification and treatment guidance. The introduction of autologous stem cell transplantation (auto-ASCT) resulted in long-term survival in responders, while treatment-related toxicity substantially limited the number of eligible candidates. Novel agents, especially bortezomib, thalidomide and lenalidomide hold promise to achieve comparable hematological responses with auto-ASCT, which might play significant role in treatment of recurrent or refractory AL amyloidosis. PMID:26117060

  19. An overview of the use of high-dose melphalan with autologous stem cell transplantation for the treatment of AL amyloidosis.

    PubMed

    Sanchorawala, V; Wright, D G; Seldin, D C; Dember, L M; Finn, K; Falk, R H; Berk, J; Quillen, K; Skinner, M

    2001-10-01

    Primary or AL amyloidosis results from a plasma cell dyscrasia in which fibrillar light chain protein deposition leads to organ failure and death. Standard treatment for AL amyloidosis has been oral melphalan and prednisone. However, this form of treatment modifies the natural history of this lethal disease only marginally, extending median survival from 13 months following diagnosis to 17 months. At Boston University Medical Center, we have developed treatment protocols using high-dose intravenous melphalan with autologous peripheral blood stem cell transplantation (HDM/SCT) to treat AL amyloidosis, and we have treated over 200 patients with HDM/SCT during the past six years. This extensive experience has shown that patients with AL amyloidosis, despite multisystem involvement and compromised organ function can tolerate this aggressive form of treatment. Furthermore, HDM/SCT results in durable hematologic responses in a substantial proportion of patients, and such responses are associated with clinical improvement, decreased amyloid-related organ dysfunction, and prolonged survival. However, toxicity from treatment is high (overall peri-transplant mortality, 14%), particularly for those patients with clinically significant cardiac involvement. For this reason, we believe a multidisciplinary management approach is essential when using HDM/SCT for treatment of AL amyloidosis. Based on our experience, we believe that HDM/SCT is the treatment of choice for patients with AL amyloidosis who have a good performance status and limited cardiac involvement at the time of diagnosis. HDM/SCT offers the best chance for hematologic remission, prolongation of survival, and reversal of amyloid-related disease. At the same time, we believe that HDM/SCT should continue to be examined in the context of clinical trials, directed at developing approaches to broaden the applicability of this therapy by minimizing toxicity and to increase the likelihood of complete hematologic responses

  20. Three cases of systemic amyloidosis successfully diagnosed by subcutaneous fat tissue biopsy of the hip.

    PubMed

    Arahata, Masahisa; Shimadoi, Shigeru; Yamatani, Satosi; Hayashi, Shin-Ichi; Miwa, Shigeharu; Asakura, Hidesaku; Nakao, Shinji

    2016-01-01

    Fine-needle aspiration biopsy of the abdominal fat pad is considered to be a minimally invasive procedure for diagnosing systemic amyloidosis. However, this procedure is sometimes difficult and can be dangerous for elderly patients whose abdominal fat layer is thin because of malnutrition. In such cases, alternative diagnostic methods are required. We report three elderly patients with heart failure complicated by malnutrition. In all cases, electrocardiogram showed low voltage in the limb leads and a pseudoinfarct pattern in the chest leads, and echocardiography showed left ventricular wall thickening with granular sparkling appearance. These patients were suspected of having amyloid cardiomyopathy but could not undergo myocardial biopsies because of their poor conditions. After failed attempts at biopsy of the abdominal fat pad or the other organs, subcutaneous fat tissue biopsy over the hip led to the diagnosis of systemic amyloidosis with cardiomyopathy. The resultant diagnosis guided us to choose the appropriate treatment for the patients. This article illustrates that subcutaneous fat tissue biopsy of the hip could be a useful procedure for diagnosing systemic amyloidosis in elderly patients, particularly when a fat tissue biopsy of the abdomen is associated with a high risk of complications because of malnutrition. PMID:27540285

  1. Three cases of systemic amyloidosis successfully diagnosed by subcutaneous fat tissue biopsy of the hip.

    PubMed

    Arahata, Masahisa; Shimadoi, Shigeru; Yamatani, Satosi; Hayashi, Shin-Ichi; Miwa, Shigeharu; Asakura, Hidesaku; Nakao, Shinji

    2016-01-01

    Fine-needle aspiration biopsy of the abdominal fat pad is considered to be a minimally invasive procedure for diagnosing systemic amyloidosis. However, this procedure is sometimes difficult and can be dangerous for elderly patients whose abdominal fat layer is thin because of malnutrition. In such cases, alternative diagnostic methods are required. We report three elderly patients with heart failure complicated by malnutrition. In all cases, electrocardiogram showed low voltage in the limb leads and a pseudoinfarct pattern in the chest leads, and echocardiography showed left ventricular wall thickening with granular sparkling appearance. These patients were suspected of having amyloid cardiomyopathy but could not undergo myocardial biopsies because of their poor conditions. After failed attempts at biopsy of the abdominal fat pad or the other organs, subcutaneous fat tissue biopsy over the hip led to the diagnosis of systemic amyloidosis with cardiomyopathy. The resultant diagnosis guided us to choose the appropriate treatment for the patients. This article illustrates that subcutaneous fat tissue biopsy of the hip could be a useful procedure for diagnosing systemic amyloidosis in elderly patients, particularly when a fat tissue biopsy of the abdomen is associated with a high risk of complications because of malnutrition.

  2. Three cases of systemic amyloidosis successfully diagnosed by subcutaneous fat tissue biopsy of the hip

    PubMed Central

    Arahata, Masahisa; Shimadoi, Shigeru; Yamatani, Satosi; Hayashi, Shin-ichi; Miwa, Shigeharu; Asakura, Hidesaku; Nakao, Shinji

    2016-01-01

    Fine-needle aspiration biopsy of the abdominal fat pad is considered to be a minimally invasive procedure for diagnosing systemic amyloidosis. However, this procedure is sometimes difficult and can be dangerous for elderly patients whose abdominal fat layer is thin because of malnutrition. In such cases, alternative diagnostic methods are required. We report three elderly patients with heart failure complicated by malnutrition. In all cases, electrocardiogram showed low voltage in the limb leads and a pseudoinfarct pattern in the chest leads, and echocardiography showed left ventricular wall thickening with granular sparkling appearance. These patients were suspected of having amyloid cardiomyopathy but could not undergo myocardial biopsies because of their poor conditions. After failed attempts at biopsy of the abdominal fat pad or the other organs, subcutaneous fat tissue biopsy over the hip led to the diagnosis of systemic amyloidosis with cardiomyopathy. The resultant diagnosis guided us to choose the appropriate treatment for the patients. This article illustrates that subcutaneous fat tissue biopsy of the hip could be a useful procedure for diagnosing systemic amyloidosis in elderly patients, particularly when a fat tissue biopsy of the abdomen is associated with a high risk of complications because of malnutrition. PMID:27540285

  3. Hereditary systemic amyloidosis due to Asp76Asn variant β2-microglobulin.

    PubMed

    Valleix, Sophie; Gillmore, Julian D; Bridoux, Frank; Mangione, Palma P; Dogan, Ahmet; Nedelec, Brigitte; Boimard, Mathieu; Touchard, Guy; Goujon, Jean-Michel; Lacombe, Corinne; Lozeron, Pierre; Adams, David; Lacroix, Catherine; Maisonobe, Thierry; Planté-Bordeneuve, Violaine; Vrana, Julie A; Theis, Jason D; Giorgetti, Sofia; Porcari, Riccardo; Ricagno, Stefano; Bolognesi, Martino; Stoppini, Monica; Delpech, Marc; Pepys, Mark B; Hawkins, Philip N; Bellotti, Vittorio

    2012-06-14

    We describe a kindred with slowly progressive gastrointestinal symptoms and autonomic neuropathy caused by autosomal dominant, hereditary systemic amyloidosis. The amyloid consists of Asp76Asn variant β(2)-microglobulin. Unlike patients with dialysis-related amyloidosis caused by sustained high plasma concentrations of wild-type β(2)-microglobulin, the affected members of this kindred had normal renal function and normal circulating β(2)-microglobulin values. The Asp76Asn β(2)-microglobulin variant was thermodynamically unstable and remarkably fibrillogenic in vitro under physiological conditions. Previous studies of β(2)-microglobulin aggregation have not shown such amyloidogenicity for single-residue substitutions. Comprehensive biophysical characterization of the β(2)-microglobulin variant, including its 1.40-Å, three-dimensional structure, should allow further elucidation of fibrillogenesis and protein misfolding. PMID:22693999

  4. Primary amyloidosis A. Immunohistochemical and biochemical characterization.

    PubMed Central

    Picken, M. M.; Pelton, K.; Frangione, B.; Gallo, G.

    1987-01-01

    Primary "idiopathic" amyloidosis is usually related to immunoglobulin light chain (AL) associated with immunocytic dyscrasias, while secondary "reactive" amyloidosis (AA) is related to serum amyloid A protein (SAA) and typically occurs with chronic inflammation, malignancy, or familial Mediterranean fever. In the present study, amyloid fibril protein extracted from frozen and paraffin-embedded tissue from a patient (CAR) with primary systemic amyloidosis proved to be AA protein by immunohistochemical, immunochemical, and amino terminal sequence. Extracts from both frozen and formalin-fixed paraffin-embedded kidney and spleen yielded similar monomers and dimers of the AA protein. The additional high-molecular-weight bands and a distinct 12,000-dalton fragment in the amyloid protein extracted from the formalin-fixed paraffin-embedded lung suggest that different processing of proteins, ie, by polymerization and/or degradation, may occur in different organs. Images Figure 1 Figure 2 Figure 3 PMID:3425691

  5. Hereditary nephropathic systemic amyloidosis caused by a novel variant apolipoprotein A-I.

    PubMed

    Persey, M R; Booth, D R; Booth, S E; van Zyl-Smit, R; Adams, B K; Fattaar, A B; Tennent, G A; Hawkins, P N; Pepys, M B

    1998-02-01

    We report a family with autosomal-dominant hereditary systemic amyloidosis in three generations, presenting with renal involvement. Two members of the current generation received renal transplants for end-stage renal failure 16 and 18 years ago, and remain very well clinically despite massive visceral amyloidosis. Two other members of this generation, aged 32 and 47 years, have massive systemic amyloid but no clinical disability. Individuals known to be affected in previous generations died of renal failure in early adult life. Amyloid deposits in the proband, one of the transplanted individuals, were composed of apolipoprotein A-I (apoA-I), and among living family members there was complete concordance between amyloidosis and the presence of a novel 9 base pair in-frame deletion mutation in exon 4 of the apoA-I gene, causing a loss of residues Glu70Phe71Trp72. This predicts the acquisition of a single extra positive charge by mature apoA-I, and this variant was detected in the plasma of all carriers. All the previously reported amyloidogenic variants of apoA-I also carry an extra positive charge, indicating that this electrostatic change is likely to be relevant to the amyloidogenicity of apoA-I. PMID:9461086

  6. Pulmonary arterial hypertension in primary amyloidosis

    PubMed Central

    Emerson, Lyska L.; Bull, David A.; Hatton, Nathan; Nativi-Nicolai, Jose; Hildebrandt, Gerhard C.; Ryan, John J.

    2016-01-01

    Abstract Amyloidosis involves extravascular deposition of fibrillar proteins within tissues and organs. Primary light chain amyloidosis represents the most common form of systemic amyloidosis involving deposition of monoclonal immunoglobulin light chains. Although pulmonary amyloid deposition is common in primary amyloidosis, clinically significant pulmonary amyloidosis is uncommon, and elevated pulmonary artery pressures are rarely observed in the absence of other underlying etiologies for pulmonary hypertension, such as elevated filling pressures secondary to cardiac amyloid. In this case report, we present a patient with primary light chain amyloidosis and pulmonary arterial hypertension in the setting of pulmonary vascular and right ventricular myocardial amyloid deposition. PMID:27252852

  7. [Heart failure and arterial hypertension disclosing amyloidosis].

    PubMed

    Habbal, R; Noureddine, M; Hachim, K; Zahraoui, M; Azzouzi, L; Fadouach, S; Zaid, D; Chraibi, N

    1997-01-01

    Amyloidosis results from protein infiltration of the extracellular space of organs and tissues. Several amyloidosis proteins have been identified. Protein AL, (deriving from immunoglobulin light chain), protein AA and prealbumin are the most involved in this disease. When AL amyloidosis involves the heart, the illness is often terminal. Most clinical symptoms are heart failure and arrhythmia or block conduction. This case was characterised by the unusual combination of hypertension and amyloidosis. The diagnosis suggested by the echocardiographic but was confirmed by the damaged organ's biopsy. The present case concerns a young woman, who has hypertension and a pulmonary oedema. The echocardiographic scan showed a septal hypertrophy with a shining and granite-like aspect which is compatible with heart amyloidosis. Systolic and diastolic disorder with mitral and aortic regurgitation were also revealed. The kidney and rectum biopsies confirmed amyloidosis AL of the Kappa dysglobulinemia type, without extraosseous plasmocytoma. The heart and kidney failure symptoms disappeared after treatment with diuretics and ACE inhibitors.

  8. Nuclear Imaging of Amyloidosis

    PubMed Central

    Cytawa, Wojciech; Teodorczyk, Jacek; Lass, Piotr

    2014-01-01

    Summary Amyloidosis is a clinical condition caused by deposition of various protein fibrills in extracellular space. The presented symptoms depend on the type of deposits and the organ or organs involved. The correct diagnosis is often difficult, due to lack of nonivasive imaging techniques and insufficiency of morphological imaging procedures delievered by radiology. We presented a list of potential radiopharmaceuticals that can be used in detecting various types of amyloidoses. 123I-SAP proved to have high sensitivity in imaging of AA and AL amyloidosis in visceral organs. 99mTc-Aprotinin was found to be useful in detecting cardiac amyloidosis. A couple of classical radiotracers, such as 201Tl, 123I-mIBG, together with 111In-antimyosin were also tested for accuracy in cardiac imaging, however the main problem was low specificity. Potential applicability was also found in case of some bone-seeking agents and other radiotracers, e.g. 67Ga-citrate and 99mTc-penta-DMSA. High sensitivity and specificity was achieved with β2-microglobulin labeled with 131I or 111In. Among PET tracers, 11C-PIB deserves more attention, because it may have an important role in diagnosing of AD in the near future. Further clinical studies are expected to take place, because noninvasive diagnosing and monitoring of amyloidosis is still a challenge. PMID:25071873

  9. Amyloidosis and Kidney Disease

    MedlinePlus

    ... body has fewer red blood cells than normal. Dialysis-related Amyloidosis People who suffer from kidney failure ... weight loss [ Top ] What are the symptoms of dialysis-related amyloidosis? The symptoms of dialysis-related amyloidosis ...

  10. Nodular pulmonary amyloidosis.

    PubMed Central

    Pinto, A; Rasmussen, S L; Campbell, T M; Lester, W M

    1984-01-01

    An elderly man had a 10-year history of multiple pulmonary nodules that he had refused to have investigated. He died of a ruptured abdominal aortic aneurysm. At autopsy the nodules were shown to consist of amyloid. There was no evidence of systemic amyloidosis. Images Fig. 1 Fig. 2 Fig. 3 Fig. 4 Fig. 1 Fig. 2 PMID:6744167

  11. Transthyretin Leu12Pro is associated with systemic, neuropathic and leptomeningeal amyloidosis.

    PubMed

    Brett, M; Persey, M R; Reilly, M M; Revesz, T; Booth, D R; Booth, S E; Hawkins, P N; Pepys, M B; Morgan-Hughes, J A

    1999-02-01

    We report a middle-aged woman with a novel transthyretin (TTR) variant, Leu12Pro. She had extensive amyloid deposition in the leptomeninges and liver as well as the involvement of the heart and peripheral nervous system which characterizes familial amyloid polyneuropathy caused by variant TTR. Clinical features attributed to her leptomeningeal amyloid included radiculopathy, central hypoventilation, recurrent subarachnoid haemorrhage, depression, seizures and periods of decreased consciousness. MRI showed a marked enhancement throughout her meninges and ependyma, and TTR amyloid deposition was confirmed by meningeal biopsy. The simultaneous presence of extensive visceral amyloid and clinically significant deposits affecting both the peripheral and central nervous system extends the spectrum of amyloid-related disease associated with TTR mutations. The unusual association of severe peripheral neuropathy with symptoms of leptomeningeal amyloid indicates that leptomeningeal amyloidosis should be considered part of the syndrome of TTR-related familial amyloid polyneuropathy. PMID:10071047

  12. Immunoglobulin Light-Chain Amyloidosis: From Basics to New Developments in Diagnosis, Prognosis and Therapy.

    PubMed

    Muchtar, Eli; Buadi, Francis K; Dispenzieri, Angela; Gertz, Morie A

    2016-01-01

    Immunoglobulin amyloid light-chain (AL) amyloidosis is the most common form of systemic amyloidosis, where the culprit amyloidogenic protein is immunoglobulin light chains produced by marrow clonal plasma cells. AL amyloidosis is an infrequent disease, and since presentation is variable and often nonspecific, diagnosis is often delayed. This results in cumulative organ damage and has a negative prognostic effect. AL amyloidosis can also be challenging on the diagnostic level, especially when demonstration of Congo red-positive tissue is not readily obtained. Since as many as 31 known amyloidogenic proteins have been identified to date, determination of the amyloid type is required. While several typing methods are available, mass spectrometry has become the gold standard for amyloid typing. Upon confirming the diagnosis of amyloidosis, a pursuit for organ involvement is essential, with a focus on heart involvement, even in the absence of suggestive symptoms for involvement, as this has both prognostic and treatment implications. Details regarding initial treatment options, including stem cell transplantation, are provided in this review. AL amyloidosis management requires a multidisciplinary approach with careful patient monitoring, as organ impairment has a major effect on morbidity and treatment tolerability until a response to treatment is achieved and recovery emerges.

  13. Prognostic impact of cytogenetic aberrations in AL amyloidosis patients after high-dose melphalan: a long-term follow-up study.

    PubMed

    Bochtler, Tilmann; Hegenbart, Ute; Kunz, Christina; Benner, Axel; Kimmich, Christoph; Seckinger, Anja; Hose, Dirk; Goldschmidt, Hartmut; Granzow, Martin; Dreger, Peter; Ho, Anthony D; Jauch, Anna; Schönland, Stefan O

    2016-07-28

    Cytogenetic aberrations detected by interphase fluorescence in situ hybridization (iFISH) of plasma cells are routinely evaluated as prognostic markers in multiple myeloma. This long-term follow-up study aimed to assess the prognosis of systemic light chain amyloidosis (AL) patients treated with high-dose melphalan (HDM) chemotherapy and autologous stem cell transplantation, depending on iFISH results. Therefore, we analyzed a consecutive cohort of 123 AL patients recruited from 2003 to 2014. HDM was safe, with only 1 of 123 patients dying as a result of treatment-related mortality, and effective, with a complete remission (CR) rate of 34%. Translocation t(11;14) as the most prevalent aberration (59%) led to an improved CR rate after high-dose therapy (41.2% vs 20.0%; P = .02), translating into a prolonged hematologic event-free survival (hemEFS; median, 46.1 vs 28.1 months; P = .05) and a trend for better overall survival (median, not reached vs 93.7 months; P = .07). In multivariate analysis, t(11;14) was confirmed as a favorable prognostic factor regarding hemEFS along with lower values for the difference between involved and uninvolved free light chains. Conversely, deletion 13q14, gain of 1q21, and hyperdiploidy had no significant prognostic impact. The high-risk cytogenetic aberrations t(4;14), t(14;16), and del(17p13) conferred an unfavorable prognosis, although statistical significance was reached only for univariate CR analysis in this small group of 9 patients. Thus, t(11;14) positivity in HDM-treated AL patients conferred superior CR rates and hemEFS. In view of the reduced response of t(11;14) to bortezomib, this highlights the impact of therapy on the prognostic role of cytogenetic aberrations.

  14. Upper Gastrointestinal Bleeding from Gastric Amyloidosis in a Patient with Smoldering Multiple Myeloma

    PubMed Central

    Gjeorgjievski, Mihajlo; Purohit, Treta; Amin, Mitual B.; Kurtin, Paul J.; Cappell, Mitchell S.

    2015-01-01

    Amyloidosis is a common complication of patients with monoclonal gammopathy of undetermined significance (MGUS), smoldering multiple myeloma (SMM), and multiple myeloma (MM). This proteinaceous material can be deposited intercellularly in any organ system, including the gastrointestinal (GI) tract. In the GI tract, amyloidosis affects the duodenum most commonly, followed by the stomach and colorectum. Gastric amyloidosis causes symptoms of nausea, vomiting, early satiety, abdominal pain, and GI bleeding. A case of upper GI bleeding from gastric amyloidosis is presented in a patient with SMM. Esophagogastroduodenoscopy (EGD) revealed a gastric mass. Endoscopic biopsies revealed amyloid deposition in the lamina propria, consistent with gastric amyloidosis. Liquid chromatography tandem mass spectrometry performed on peptides extracted from Congo red-positive microdissected areas of paraffin-embedded stomach specimens revealed a peptide profile consistent with AL- (lambda-) type amyloidosis. Based on this and multiple other case reports, we recommend that patients with GI bleeding and MGUS, SMM, or MM undergo EGD and pathologic examination of endoscopic biopsies of identified lesions using Congo red stains for amyloidosis for early diagnosis and treatment. PMID:26366309

  15. Upper Gastrointestinal Bleeding from Gastric Amyloidosis in a Patient with Smoldering Multiple Myeloma.

    PubMed

    Gjeorgjievski, Mihajlo; Purohit, Treta; Amin, Mitual B; Kurtin, Paul J; Cappell, Mitchell S

    2015-01-01

    Amyloidosis is a common complication of patients with monoclonal gammopathy of undetermined significance (MGUS), smoldering multiple myeloma (SMM), and multiple myeloma (MM). This proteinaceous material can be deposited intercellularly in any organ system, including the gastrointestinal (GI) tract. In the GI tract, amyloidosis affects the duodenum most commonly, followed by the stomach and colorectum. Gastric amyloidosis causes symptoms of nausea, vomiting, early satiety, abdominal pain, and GI bleeding. A case of upper GI bleeding from gastric amyloidosis is presented in a patient with SMM. Esophagogastroduodenoscopy (EGD) revealed a gastric mass. Endoscopic biopsies revealed amyloid deposition in the lamina propria, consistent with gastric amyloidosis. Liquid chromatography tandem mass spectrometry performed on peptides extracted from Congo red-positive microdissected areas of paraffin-embedded stomach specimens revealed a peptide profile consistent with AL- (lambda-) type amyloidosis. Based on this and multiple other case reports, we recommend that patients with GI bleeding and MGUS, SMM, or MM undergo EGD and pathologic examination of endoscopic biopsies of identified lesions using Congo red stains for amyloidosis for early diagnosis and treatment. PMID:26366309

  16. A case report of hereditary apolipoprotein A-I amyloidosis associated with a novel APOA1 mutation and variable phenotype.

    PubMed

    Tougaard, Birgitte G; Pedersen, Katja Venborg; Krag, Søren Rasmus; Gilbertson, Janet A; Rowczenio, Dorota; Gillmore, Julian D; Birn, Henrik

    2016-09-01

    Apolipoprotein A-I (apo A-I) amyloidosis is a non-AL, non-AA, and non-transthyretin type of amyloidosis associated with mutations in the APOA1 gene inherited in an autosomal dominant fashion. It is a form of systemic amyloidosis, but at presentation, can also mimic localized amyloidosis. The renal presentation generally involves interstitial and medullary deposition of apo A-I amyloid protein. We describe the identification of apo A-I amyloidosis by mass spectrometry in a 52-year old male, with no family history of amyloidosis, presenting with nephrotic syndrome and associated with heterozygosity for a novel APOA1 mutation (c.220 T > A) which encodes the known amyloidogenic Trp50Arg variant. Renal amyloid deposits in this case were confined to the glomeruli alone, and the patient developed progressive renal impairment. One year after diagnosis, the patient had a successful kidney transplant from an unrelated donor. Pathogenic mutations in the APOA1 gene are generally associated with symptoms of amyloidosis. In this family however, genotyping of family members identified several unaffected carriers suggesting a variable disease penetrance, which has not been described before in this form of amyloidosis and has implications when counselling those with APOA1 mutations. PMID:27240838

  17. A case report of hereditary apolipoprotein A-I amyloidosis associated with a novel APOA1 mutation and variable phenotype.

    PubMed

    Tougaard, Birgitte G; Pedersen, Katja Venborg; Krag, Søren Rasmus; Gilbertson, Janet A; Rowczenio, Dorota; Gillmore, Julian D; Birn, Henrik

    2016-09-01

    Apolipoprotein A-I (apo A-I) amyloidosis is a non-AL, non-AA, and non-transthyretin type of amyloidosis associated with mutations in the APOA1 gene inherited in an autosomal dominant fashion. It is a form of systemic amyloidosis, but at presentation, can also mimic localized amyloidosis. The renal presentation generally involves interstitial and medullary deposition of apo A-I amyloid protein. We describe the identification of apo A-I amyloidosis by mass spectrometry in a 52-year old male, with no family history of amyloidosis, presenting with nephrotic syndrome and associated with heterozygosity for a novel APOA1 mutation (c.220 T > A) which encodes the known amyloidogenic Trp50Arg variant. Renal amyloid deposits in this case were confined to the glomeruli alone, and the patient developed progressive renal impairment. One year after diagnosis, the patient had a successful kidney transplant from an unrelated donor. Pathogenic mutations in the APOA1 gene are generally associated with symptoms of amyloidosis. In this family however, genotyping of family members identified several unaffected carriers suggesting a variable disease penetrance, which has not been described before in this form of amyloidosis and has implications when counselling those with APOA1 mutations.

  18. [Pulmonary Amyloidosis: A Diagnostic Challenge].

    PubMed

    Alves, Ana; Alfaro, Tiago M; Madama, Daniela; Freitas, Sara; Robalo-Cordeiro, Carlos; Gamboa, Fernanda

    2015-01-01

    Amyloidosis is characterized by amyloid extracellular deposition in organs and tissues. Pulmonary involvement is a rare manifestation of the disease and it can be focal or as part of systemic amyloidosis. We report two cases. Case 1: 71 year-old female with bronchiectasis and Sjogrenâ syndrome, who complained of anorexia, weight loss and a productive cough. The diagnostic study included a surgical lung biopsy and histological examination demonstrated pulmonary amyloidosis. Case 2: 83 year-old male patient, ex-smoker, asymptomatic, whose routine chest x-ray showed a nodular opacity in the right lung field. A transthoracic biopsy revealed an amyloid lung tumor. These cases illustrate a rare disease which in Case 1 also coexisted with Sjögrenâs syndrome and bronchiectasis. The most important differential diagnosis is cancer and so a definitive diagnosis is essential, as amyloidosis is usually benign and indolent.

  19. Genetic factors in amyloidosis.

    PubMed Central

    Thomas, P K

    1975-01-01

    In the absence of biochemical distinctions, the nosography of the inherited amyloidoses must at present depend largely upon clinical subdivisions. In the broad classification adopted here, the disorders have for convenience been grouped according to the anatomical system that is predominantly affected. It is evident that the amyloid syndromes display considerable heterogeneity. However, they overlap. Thus in the Iowa type classified with the hereditary amyloid neuropathies (van Allen et al, 1969; Gimeno et al, 1974), renal involvement was frequent and was the usual cause of death. In the English (Zalin et al, 1974) and Scandinavian (Andersson, 1970) families with neuropathy as the predominant feature, cardiac involvement was a common finding. In certain of the conditions discussed, such as medullary carcinoma of the thyroid and Down's syndrome, amyloid deposition is merely an incidental aspect of the disorder. In those conditions in which generalized or localized amyloid deposition occupies a more central position in the clinical syndrome, an autosomal dominant inheritance has been established or suggested in the majority. An autosomal recessive inheritance has so far only been recognized in familial Mediterranean fever. In the family with hereditary amyloid heart diseases reported by Fredricksen et al (1962), the disorder was confined to a single sibship, raising the possibility of recessive inheritance. This could also be true in sporadic examples of primary amyloidosis. The dominantly inherited amyloidoses comprise a number of geographically widely scattered families with clinical pictures that do not show consistent differences between some families. The families that do not show consistent differences are not necessarily harbouring nutations at the same locus, or the same mutation at any particular locus. However, many of these dominantly inherited clinical syndromes are sufficiently different from each other and the clinical manifestations of each

  20. [Optimization of the immunohistochemical diagnosis of AL amyloidosis using novel antibodies].

    PubMed

    Mikhaleva, L M; Gioeva, Z V; Reken, K

    2015-01-01

    Цель — улучшение иммуногистохимической диагностики AL-амилоидоза путем создания новых пептидных антител, направленных к вариабельному и константному регионам каппа-легких цепей. Материал и методы. В научной литературе, а также в базе данных национального центра биотехнологической информации (NCBI) провели поиск всех амилоидогенных каппа-легких цепей. На основании полученных данных составлена цепь из наиболее часто встречающихся аминокислотных остатков, из которых и были выбраны пептидные регионы для иммунизации. Четыре антитела были генерированы в результате иммунизации кроликов двумя пептидами, которые соответствовали вариабельному и константному регионам каппа-легких цепей. Результаты. Специфичность полученных антител подтверждена на серии из 222 биоптатов, полученных у 193 пациентов с AA-, AL-амилоидозом, транстиретиновым и инсулиновым амилоидозом. Все новые поликлональные пептидные антитела дали позитивную окраску в случаях с AL-каппа-амилоидозом. Заключение. Созданные нами поликлональные антитела, направленные к вариабельному и константному регионам каппа-легких

  1. AA amyloidosis in vaccinated growing chickens.

    PubMed

    Murakami, T; Inoshima, Y; Sakamoto, E; Fukushi, H; Sakai, H; Yanai, T; Ishiguro, N

    2013-01-01

    Systemic amyloid-A (AA) amyloidosis in birds occurs most frequently in waterfowl such as Pekin ducks. In chickens, AA amyloidosis is observed as amyloid arthropathy. Outbreaks of systemic amyloidosis in flocks of layers are known to be induced by repeated inflammatory stimulation, such as those resulting from multiple vaccinations with oil-emulsified bacterins. Outbreaks of fatal AA amyloidosis were observed in growing chickens in a large scale poultry farm within 3 weeks of vaccination with multiple co-administered vaccines. This study documents the histopathological changes in tissues from these birds. Amyloid deposits were also observed at a high rate in the tissues of apparently healthy chickens. Vaccination should therefore be considered as a potential risk factor for the development of AA amyloidosis in poultry.

  2. Risk-adapted autologous stem cell transplantation with adjuvant dexamethasone +/- thalidomide for systemic light-chain amyloidosis: results of a phase II trial.

    PubMed

    Cohen, Adam D; Zhou, Ping; Chou, Joanne; Teruya-Feldstein, Julie; Reich, Lilian; Hassoun, Hani; Levine, Beth; Filippa, Daniel A; Riedel, Elyn; Kewalramani, Tarun; Stubblefield, Michael D; Fleisher, Martin; Nimer, Stephen; Comenzo, Raymond L

    2007-10-01

    High-dose melphalan (MEL) with autologous stem cell transplant (SCT) is an effective therapy for systemic AL amyloidosis (AL), but treatment-related mortality (TRM) has historically been high. We performed a phase II trial of risk-adapted SCT followed by adjuvant dexamethasone (dex) and thalidomide (thal) in an attempt to reduce TRM and improve response rates. Patients (n = 45) with newly diagnosed AL involving < or =2 organ systems were assigned to MEL 100, 140, or 200 mg/m(2) with SCT, based on age, renal function and cardiac involvement. Patients with persistent clonal plasma cell disease 3 months post-SCT received 9 months of adjuvant thal/dex (or dex if there was a history of deep vein thrombosis or neuropathy). Organ involvement was kidney (67%), heart (24%), liver/GI (22%) and peripheral nervous system (18%), with 31% having two organs involved. TRM was 4.4%. Thirty-one patients began adjuvant therapy, with 16 (52%) completing 9 months of treatment and 13 (42%) achieving an improvement in haematological response. By intention-to-treat, overall haematological response rate was 71% (36% complete response), with 44% having organ responses. With a median follow-up of 31 months, 2-year survival was 84% (95% confidence interval: 73%, 94%). Risk-adapted SCT with adjuvant thal/dex is feasible and results in low TRM and high haematological and organ response rates in AL patients. PMID:17897298

  3. Primary breast amyloidosis presenting as microcalcifications only.

    PubMed

    Shim, Youngsub; Kim, Min Jung; Ryu, Han Suk; Park, Sung Hee

    2013-01-01

    Amyloidosis is a rare disease characterized by the formation of pathological protein deposits in organs or tissues. It is typically a systemic disease which can occur in a localized form. Amyloidosis of the breast is uncommon. Common mammographic findings of breast amyloidosis are multiple nodules with or without calcifications. We report a case of primary localized breast amyloidosis presenting suspicious microcalcifications on mammography without associated masses. Mammography in a 72-year-old woman displayed multiple, linearly distributed, irregular and rod-like calcifications in the subareolar area of the left breast. The patient underwent surgical excision under mammo-guided needle localization and the pathology was confirmed to be breast amyloidosis. PMID:24043964

  4. Primary Breast Amyloidosis Presenting as Microcalcifications Only

    PubMed Central

    Shim, Youngsub; Kim, Min Jung; Ryu, Han Suk

    2013-01-01

    Amyloidosis is a rare disease characterized by the formation of pathological protein deposits in organs or tissues. It is typically a systemic disease which can occur in a localized form. Amyloidosis of the breast is uncommon. Common mammographic findings of breast amyloidosis are multiple nodules with or without calcifications. We report a case of primary localized breast amyloidosis presenting suspicious microcalcifications on mammography without associated masses. Mammography in a 72-year-old woman displayed multiple, linearly distributed, irregular and rod-like calcifications in the subareolar area of the left breast. The patient underwent surgical excision under mammo-guided needle localization and the pathology was confirmed to be breast amyloidosis. PMID:24043964

  5. Does AL amyloidosis have a unique genomic profile? Gene expression profiling meta-analysis and literature overview.

    PubMed

    Kryukov, Fedor; Kryukova, Elena; Brozova, Lucie; Kufova, Zuzana; Filipova, Jana; Growkova, Katerina; Sevcikova, Tereza; Jarkovsky, Jiri; Hajek, Roman

    2016-10-15

    Immunoglobulin light chain amyloidosis (ALA) is a plasma cell dyscrasia characterized by deposition of amyloid fibrils in various organs and tissues. The current paper is devoted to clarify if ALA has a unique gene expression profile and to its pathogenetic argumentation. The meta-analysis of ALA patients vs. healthy donors, monoclonal gammopathy of undetermined significance, smoldering and multiple myeloma patients' cohorts have revealed molecular signature of ALA consists of 256 genes representing mostly ribosomal proteins and immunoglobulin regions. This signature appears pathogenetically supported and elucidates for the first time the role of ribosome dysfunction in ALA. In summary of our findings with literature overview, we hypothesize that ALA development is associated not only with changes in genes, coding amyloidogenic protein itself, but with post-transcriptional disbalance as well. Based on our data analysis in ALA, ribosome machinery is impaired and the affected link mainly involves translational initiation, elongation and co-translational protein folding. PMID:27288311

  6. Leukocyte chemotactic factor 2 amyloidosis (ALECT2) is a common form of renal amyloidosis among Egyptians.

    PubMed

    Larsen, Christopher P; Ismail, Wesam; Kurtin, Paul J; Vrana, Julie A; Dasari, Surendra; Nasr, Samih H

    2016-04-01

    Large case series of renal amyloidosis subtypes have recently been published in the United States and Europe showing AL amyloidosis to be the predominant subtype in this part of the world. However, the most common subtypes of renal amyloidosis throughout the rest of the world are unknown. We present here the first large case series detailing the subtypes of renal amyloidosis among Egyptians. In this population, AA amyloidosis was the most common type of amyloidosis on renal biopsy at 48%. The newly described leukocyte chemotactic factor 2 amyloidosis (ALECT2) was the second most common type and represented nearly one-third of renal amyloid cases at 31%. AL accounted for only 20% of cases. The pathologic findings in ALECT2 cases were similar to those previously described in other case series. Thus ALECT2, which was initially thought to affect mainly Hispanics in the United States, appears to represent an important and likely underrecognized etiology of chronic kidney disease among Egyptians and probably in other ethnic groups around the world. PMID:26867784

  7. [New trends in the treatment of amyloidosis].

    PubMed

    Martínez-Valle, Fernando; Gironella-Mesa, Mercedes; Solans-Laqué, Roser

    2012-05-26

    Amyloidosis is a clinical disorder caused by extracellular deposition of proteins that are normally soluble as insoluble fibrils that damage different organs. More than 20 proteins can form amyloid deposits. All types of amyloid fibrils have a secondary structure with a β folded shape that is characteristic and makes them to adopt a green birefringence after stained with Congo red and viewed under cross-polarized light. Amyloidosis can be acquired or hereditary, systemic or localized, and are classified by the fibril precursor protein. Advances in the knowledge of the pathogenesis of amyloidosis allows the development of new diagnostic and therapeutical schemes that are currently under investigation.

  8. Amyloidosis in Retinal Neurodegenerative Diseases.

    PubMed

    Masuzzo, Ambra; Dinet, Virginie; Cavanagh, Chelsea; Mascarelli, Frederic; Krantic, Slavica

    2016-01-01

    As a part of the central nervous system, the retina may reflect both physiological processes and abnormalities related to pathologies that affect the brain. Amyloidosis due to the accumulation of amyloid-beta (Aβ) was initially regarded as a specific and exclusive characteristic of neurodegenerative alterations seen in the brain of Alzheimer's disease (AD) patients. More recently, it was discovered that amyloidosis-related alterations, similar to those seen in the brain of Alzheimer's patients, also occur in the retina. Remarkably, these alterations were identified not only in primary retinal pathologies, such as age-related macular degeneration (AMD) and glaucoma, but also in the retinas of Alzheimer's patients. In this review, we first briefly discuss the biogenesis of Aβ, a peptide involved in amyloidosis. We then discuss some pathological aspects (synaptic dysfunction, mitochondrial failure, glial activation, and vascular abnormalities) related to the neurotoxic effects of Aβ. We finally highlight common features shared by AD, AMD, and glaucoma in the context of Aβ amyloidosis and further discuss why the retina, due to the transparency of the eye, can be considered as a "window" to the brain. PMID:27551275

  9. Amyloidosis in Retinal Neurodegenerative Diseases

    PubMed Central

    Masuzzo, Ambra; Dinet, Virginie; Cavanagh, Chelsea; Mascarelli, Frederic; Krantic, Slavica

    2016-01-01

    As a part of the central nervous system, the retina may reflect both physiological processes and abnormalities related to pathologies that affect the brain. Amyloidosis due to the accumulation of amyloid-beta (Aβ) was initially regarded as a specific and exclusive characteristic of neurodegenerative alterations seen in the brain of Alzheimer’s disease (AD) patients. More recently, it was discovered that amyloidosis-related alterations, similar to those seen in the brain of Alzheimer’s patients, also occur in the retina. Remarkably, these alterations were identified not only in primary retinal pathologies, such as age-related macular degeneration (AMD) and glaucoma, but also in the retinas of Alzheimer’s patients. In this review, we first briefly discuss the biogenesis of Aβ, a peptide involved in amyloidosis. We then discuss some pathological aspects (synaptic dysfunction, mitochondrial failure, glial activation, and vascular abnormalities) related to the neurotoxic effects of Aβ. We finally highlight common features shared by AD, AMD, and glaucoma in the context of Aβ amyloidosis and further discuss why the retina, due to the transparency of the eye, can be considered as a “window” to the brain. PMID:27551275

  10. Pulmonary hyalinizing granulomas in a patient with malignant lymphoma, with development nine years later of multiple myeloma and systemic amyloidosis.

    PubMed

    Drasin, H; Blume, M R; Rosenbaum, E H; Klein, H Z

    1979-07-01

    In our patient, multiple bilateral nodular pulmonary densities appeared on a chest x-ray at the time of diagnosis of stage IV diffuse lymphocytic lymphoma. After localized radiation therapy, the patient received no further systemic therapy. The pulmonary nodules slowly became larger and more numerous. Nine years later the patient developed proven multiple myeloma. Pulmonary hyalinizing granulomas have not heretofore been associated with proven lymphoreticular neoplasia, although this has long been suspected. The occurrence of two B-cell tumors at different points in time associated with systemic amyloidosis is an extremely rare event. The authors discuss the possibility that these conditions represent an abnormality in a common cell of origin with differing expression over time. Coincidence, however, remains a likely explanation for the different immunopathies that occurred in our patient. PMID:582294

  11. Primary sjögren syndrome manifested as localized cutaneous nodular amyloidosis.

    PubMed

    Wey, Shiow-Jiuan; Chen, Yi-Ming; Lai, Po-Ju; Chen, Der-Yuan

    2011-10-01

    Localized cutaneous nodular amyloidosis (LCNA) is the rarest type of cutaneous amyloidosis. Typically presenting as waxy nodules on the lower extremities, it demonstrates localized deposition of AL-type amyloid in immunohistologic study and is often associated with focal plasma cell proliferation. Sjögren syndrome, an autoimmune lymphoproliferative disorder, is characterized by keratoconjunctivitis sicca and xerostomia with lymphocytic infiltration of exocrine glands. As shown in case reports, the association of LCNA with Sjögren syndrome is considerable. Herein, we report a 78-year-old woman with LCNA, who was further surveyed and diagnosed with Sjögren syndrome. In light of the significant relation between these 2 diseases, further examination for coexistence of Sjögren syndrome in addition to systemic amyloidosis is well warranted. Prompt identification of an underlying Sjögren syndrome in LCNA with polyclonal immunoglobulin amyloid may have important therapeutic consequences.

  12. Anterior Orbit and Adnexal Amyloidosis

    PubMed Central

    Al Hussain, Hailah; Edward, Deepak P.

    2013-01-01

    Purpose: To describe six cases of anterior orbital and adnexal amyloidosis and to report on proteomic analysis to characterize the nature of amyloid in archived biopsies in two cases. Materials and Methods: The clinical features, radiological findings, pathology, and outcome of six patients with anterior orbit and adnexal amyloidosis were retrieved from the medical records. The biochemical nature of the amyloid was determined using liquid chromatography/mass spectroscopy archived paraffin-embedded tissue in two cases. Results: Of the six cases, three had unilateral localized anterior orbit and lacrimal gland involvement. Four of the six patients were female with an average duration of 12.8 years from the time of onset to presentation eyelid infiltration by amyloid caused ptosis in five cases. CT scan in patients with lacrimal gland involvement (n = 3) demonstrated calcified deformable anterior orbital masses and on pathological exmaintionamyloid and calcific deposits replaced the lacrimal gland acini. Ptosis repair was performed in three patients with good outcomes. One patient required repeated debulking of the mass and one patient had recurrenct disease. Proteomic analysis revealed polyclonal IgG-associated amyloid deposition in one patient and AL kappa amyloid in the second patient. Conclusion: Amyloidosis of the anterior orbit and lacrimal gland can present with a wide spectrum of findings with good outcomes after surgical excision. The nature of amyloid material can be precisely determined in archival pathology blocks using diagnostic proteomic analysis. PMID:24014979

  13. Amyloidosis: Modern Cross-sectional Imaging.

    PubMed

    Czeyda-Pommersheim, Ferenc; Hwang, Misun; Chen, Sue Si; Strollo, Diane; Fuhrman, Carl; Bhalla, Sanjeev

    2015-01-01

    Amyloidosis is a rare diverse condition caused by the pathologic extracellular deposition of abnormal insoluble proteins throughout the body. It may exist as a primary disease or, more commonly, may be secondary to a wide variety of pathologic processes ranging from chronic infection or inflammation to malignancy. Hereditary forms also exist. On the basis of the structure of the protein deposits, more than two dozen subtypes of amyloidosis have been described. A single organ or multiple organ systems may be affected. The radiologic manifestations of amyloidosis are varied and often nonspecific, making amyloidosis a diagnostic challenge for the radiologist. In the chest, the lungs, mediastinum, pleura, and heart may be involved. Lung involvement may manifest as diffuse reticulonodular interstitial thickening, consolidations, or solitary or multiple parenchymal nodules that may calcify, cavitate, and slowly enlarge. Pleural involvement most commonly manifests as pleural effusions. Tracheobronchial involvement may exhibit concentric airway thickening, mural and intraluminal nodules, submucosal calcification, and airway obstruction. Mediastinal and hilar lymph nodes may enlarge and frequently calcify. At cardiac magnetic resonance (MR) imaging, the left ventricular wall is typically thickened, with associated diastolic dysfunction. Delayed contrast material-enhanced cardiac MR imaging typically shows global transmural or subendocardial enhancement. The pathophysiology, classification, treatment, and prognosis of amyloidosis are reviewed, followed by case examples of the appearance of thoracic and cardiac amyloidosis on chest radiographs, computed tomographic (CT) images, and cardiac MR images. PMID:26230754

  14. Cardiac amyloidosis

    MedlinePlus

    ... the way electrical signals move through the heart (conduction system). This can lead to abnormal heartbeats ( arrhythmias ) ... due to medicine) Sick sinus syndrome Symptomatic cardiac conduction system disease (arrhythmias related to abnormal conduction of ...

  15. Cell Damage in Light Chain Amyloidosis: FIBRIL INTERNALIZATION, TOXICITY AND CELL-MEDIATED SEEDING.

    PubMed

    Marin-Argany, Marta; Lin, Yi; Misra, Pinaki; Williams, Angela; Wall, Jonathan S; Howell, Kyle G; Elsbernd, Laura R; McClure, Megan; Ramirez-Alvarado, Marina

    2016-09-16

    Light chain (AL) amyloidosis is an incurable human disease characterized by the misfolding, aggregation, and systemic deposition of amyloid composed of immunoglobulin light chains (LC). This work describes our studies on potential mechanisms of AL cytotoxicity. We have studied the internalization of AL soluble proteins and amyloid fibrils into human AC16 cardiomyocytes by using real time live cell image analysis. Our results show how external amyloid aggregates rapidly surround the cells and act as a recruitment point for soluble protein, triggering the amyloid fibril elongation. Soluble protein and external aggregates are internalized into AC16 cells via macropinocytosis. AL amyloid fibrils are shown to be highly cytotoxic at low concentrations. Additionally, caspase assays revealed soluble protein induces apoptosis, demonstrating different cytotoxic mechanisms between soluble protein and amyloid aggregates. This study emphasizes the complex immunoglobulin light chain-cell interactions that result in fibril internalization, protein recruitment, and cytotoxicity that may occur in AL amyloidosis. PMID:27462073

  16. Atypical tumour-like involvement of the colon in secondary systemic amyloidosis which vanished after 1 month of observation

    PubMed Central

    Ando, Katuyoshi; Fujiya, Mikihiro; Ito, Takahiro; Sugiyama, Ryuji; Nata, Toshie; Nomura, Yoshiki; Ueno, Nobuhiro; Kashima, Shin; Ishikawa, Chisato; Inaba, Yuhiei; Moriichi, Kentaro; Okamoto, Kotaro; Ikuta, Katsuya; Mizukami, Yusuke; Tokusashi, Yoshihiko; Miyokawa, Naoyuki; Watari, Jiro; Kohgo, Yutaka

    2011-01-01

    Amyloidosis occurs as a result of the extracellular deposition of protein fibrils in organs and tissues, thus causing mild to severe pathophysiological changes. The gastrointestinal tract is a common site of amyloid deposition. While intestinal amyloidosis frequently results in polypoid lesions, ulcerations, nodules and petechial mucosal haemorrhage, tumour-like lesions are rarely developed and infrequently diagnosed before the resection because of the difficulty in differentiating them from colon cancer. The authors herein reported a case of intestinal amyloid A amyloidosis with a complication of a tumour-like lesion endoscopically resembling a malignant lesion, which was completely diminished after 1 month of observation with bowel rest. Such conservative treatment is a feasible option to cure intestinal tumour-like lesions in patients with intestinal amyloidosis when no neoplastic change is histologically detected, possibly decreasing the need for surgery of the fragile mucosa. PMID:22699464

  17. Insulin-derived amyloidosis

    PubMed Central

    Gupta, Yashdeep; Singla, Gaurav; Singla, Rajiv

    2015-01-01

    Amyloidosis is the term for diseases caused by the extracellular deposition of insoluble polymeric protein fibrils in tissues and organs. Insulin-derived amyloidosis is a rare, yet significant complication of insulin therapy. Insulin-derived amyloidosis at injection site can cause poor glycemic control and increased insulin dose requirements because of the impairment in insulin absorption, which reverse on change of injection site and/or excision of the mass. This entity should be considered and assessed by histopathology and immunohistochemistry, in patients with firm/hard local site reactions, which do not regress after cessation of insulin injection at the affected site. Search strategy: PubMed was searched with terms “insulin amyloidosis”. Full text of articles available in English was reviewed. Relevant cross references were also reviewed. Last search was made on October 15, 2014. PMID:25593849

  18. Spontaneous bilateral kidney rupture during autologous stem cell transplantation in a patient affected by amyloidosis

    PubMed Central

    Ferrannini, Michele; Vischini, Gisella; De Angelis, Gottardo; Giannakakis, Konstantinos; Arcese, William

    2011-01-01

    Kidney spontaneous rupture is not a recognized complication neither for amyloidosis nor of autologous stem cell transplantation (ASCT). A 46-year-old white woman, affected by nephrotic syndrome, was diagnosed as AL amyloidosis by renal biopsy. We report the singular case of a bilateral spontaneous kidney rupture during ASCT for AL with renal rescue. PMID:25984105

  19. Abnormal scintigraphic evolution in AA hepatic amyloidosis

    SciTech Connect

    Lomena, F.; Rosello, R.; Pons, F.; Grau, M.; Garcia, A.; Catafau, A.; Setoain, J.

    1988-03-01

    A patient with AA amyloidosis secondary to ankylosing spondylitis showed intense liver uptake of Tc-99m MDP on bone imaging. The biopsy showed hepatic amyloid deposition. A repeat bone scan with Tc-99m MDP 1 year later was negative, although the clinical signs and liver function tests of the patient had not changed. A mechanism might exist, other than the affinity of amyloid to calcium, which would explain the extraosseous uptake of pyrophosphates and diphosphonates in organs and soft tissues affected by systemic amyloidosis.

  20. Prognostic Value of Late Gadolinium Enhancement Cardiovascular Magnetic Resonance in Cardiac Amyloidosis

    PubMed Central

    Fontana, Marianna; Pica, Silvia; Reant, Patricia; Abdel-Gadir, Amna; Treibel, Thomas A.; Banypersad, Sanjay M.; Maestrini, Viviana; Barcella, William; Rosmini, Stefania; Bulluck, Heerajnarain; Sayed, Rabya H.; Patel, Ketna; Mamhood, Shameem; Bucciarelli-Ducci, Chiara; Whelan, Carol J.; Herrey, Anna S.; Lachmann, Helen J.; Wechalekar, Ashutosh D.; Manisty, Charlotte H.; Schelbert, Eric B.; Kellman, Peter; Gillmore, Julian D.; Hawkins, Philip N.

    2015-01-01

    Background— The prognosis and treatment of the 2 main types of cardiac amyloidosis, immunoglobulin light chain (AL) and transthyretin (ATTR) amyloidosis, are substantially influenced by cardiac involvement. Cardiovascular magnetic resonance with late gadolinium enhancement (LGE) is a reference standard for the diagnosis of cardiac amyloidosis, but its potential for stratifying risk is unknown. Methods and Results— Two hundred fifty prospectively recruited subjects, 122 patients with ATTR amyloid, 9 asymptomatic mutation carriers, and 119 patients with AL amyloidosis, underwent LGE cardiovascular magnetic resonance. Subjects were followed up for a mean of 24±13 months. LGE was performed with phase-sensitive inversion recovery (PSIR) and without (magnitude only). These were compared with extracellular volume measured with T1 mapping. PSIR was superior to magnitude-only inversion recovery LGE because PSIR always nulled the tissue (blood or myocardium) with the longest T1 (least gadolinium). LGE was classified into 3 patterns: none, subendocardial, and transmural, which were associated with increasing amyloid burden as defined by extracellular volume (P<0.0001), with transitions from none to subendocardial LGE at an extracellular volume of 0.40 to 0.43 (AL) and 0.39 to 0.40 (ATTR) and to transmural at 0.48 to 0.55 (AL) and 0.47 to 0.59 (ATTR). Sixty-seven patients (27%) died. Transmural LGE predicted death (hazard ratio, 5.4; 95% confidence interval, 2.1–13.7; P<0.0001) and remained independent after adjustment for N-terminal pro-brain natriuretic peptide, ejection fraction, stroke volume index, E/E′, and left ventricular mass index (hazard ratio, 4.1; 95% confidence interval, 1.3–13.1; P<0.05). Conclusions— There is a continuum of cardiac involvement in systemic AL and ATTR amyloidosis. Transmural LGE is determined reliably by PSIR and represents advanced cardiac amyloidosis. The PSIR technique provides incremental information on outcome even after

  1. D18G transthyretin is monomeric, aggregation prone, and not detectable in plasma and cerebrospinal fluid: a prescription for central nervous system amyloidosis?

    PubMed

    Hammarström, Per; Sekijima, Yoshiki; White, Joleen T; Wiseman, R Luke; Lim, Amareth; Costello, Catherine E; Altland, Klaus; Garzuly, Ferenc; Budka, Herbert; Kelly, Jeffery W

    2003-06-10

    Over 70 transthyretin (TTR) mutations facilitate amyloidosis in tissues other than the central nervous system (CNS). In contrast, the D18G TTR mutation in individuals of Hungarian descent leads to CNS amyloidosis. D18G forms inclusion bodies in Escherichia coli, unlike the other disease-associated TTR variants overexpressed to date. Denaturation and reconstitution of D18G from inclusion bodies afford a folded monomer that is destabilized by 3.1 kcal/mol relative to an engineered monomeric version of WT TTR. Since TTR tetramer dissociation is typically rate limiting for amyloid formation, the monomeric nature of D18G renders its amyloid formation rate 1000-fold faster than WT. It is perplexing that D18G does not lead to severe early onset systemic amyloidosis, given that it is the most destabilized TTR variant characterized to date, more so than variants exhibiting onset in the second decade. Instead, CNS impairment is observed in the fifth decade as the sole pathological manifestation; however, benign systemic deposition is also observed. Analysis of heterozygote D18G patient's serum and cerebrospinal fluid (CSF) detects only WT TTR, indicating that D18G is either rapidly degraded postsecretion or degraded within the cell prior to secretion, consistent with its inability to form hybrid tetramers with WT TTR. The nondetectable levels of D18G TTR in human plasma explain the absence of an early onset systemic disease. CNS disease may result owing to the sensitivity of the CNS to lower levels of D18G aggregate. Alternatively, or in addition, we speculate that a fraction of D18G made by the choroid plexus can be transiently tetramerized by the locally high thyroxine (T(4)) concentration, chaperoning it out into the CSF where it undergoes dissociation and amyloidogenesis due to the low T(4) CSF concentration. Selected small molecule tetramer stabilizers can transform D18G from a monomeric aggregation-prone state to a nonamyloidogenic tetramer, which may prove to be a

  2. ALS superbend magnet system

    SciTech Connect

    Zbasnik, J.; Wang, S.T.; Chen, J.Y.; DeVries, G.J.; DeMarco, R.; Fahmie, M.; Geyer, A.; Green, M.A.; Harkins, J.; Henderson, T.; Hinkson, J.; Hoyer, E.H.; Krupnick, J.; Marks, S.; Ottens, F.; Paterson, J.A.; Pipersky, P.; Portmann, G.; Robin, D.A.; Schlueter, R.D.; Steier, C.; Taylor, C.E.; Wahrer, R.

    2000-09-15

    The Lawrence Berkeley National Laboratory is preparing to upgrade the Advanced Light Source (ALS) with three superconducting dipoles (Superbends). In this paper we present the final magnet system design which incorporates R&D test results and addresses the ALS operational concerns of alignment, availability, and economy. The design incorporates conduction-cooled Nb-Ti windings and HTS current leads, epoxy-glass suspension straps, and a Gifford-McMahon cryocooler to supply steady state refrigeration. We also present the current status of fabrication and testing.

  3. What is new in diagnosis and management of light chain amyloidosis?

    PubMed

    Palladini, Giovanni; Merlini, Giampaolo

    2016-07-14

    Light chain (AL) amyloidosis is caused by a usually small plasma cell clone producing a misfolded light chain that deposits in tissues. Survival is mostly determined by the severity of heart involvement. Recent studies are clarifying the mechanisms of cardiac damage, pointing to a toxic effect of amyloidogenic light chains and offering new potential therapeutic targets. The diagnosis requires adequate technology, available at referral centers, for amyloid typing. Late diagnosis results in approximately 30% of patients presenting with advanced, irreversible organ involvement and dying in a few months despite modern treatments. The availability of accurate biomarkers of clonal and organ disease is reshaping the approach to patients with AL amyloidosis. Screening of early organ damage based on biomarkers can help identify patients with monoclonal gammopathy of undetermined significance who are developing AL amyloidosis before they become symptomatic. Staging systems and response assessment based on biomarkers facilitate the design and conduction of clinical trials, guide the therapeutic strategy, and allow the timely identification of refractory patients to be switched to rescue therapy. Treatment should be risk-adapted. Recent studies are linking specific characteristics of the plasma cell clone to response to different types of treatment, moving toward patient-tailored therapy. In addition, novel anti-amyloid treatments are being developed that might be combined with anti-plasma cell chemotherapy. PMID:27053535

  4. A Rare Case of Amyloidosis of the Eyelid and Conjunctiva

    PubMed Central

    Fernando, Bertie

    2016-01-01

    Amyloidosis of the eyelid is uncommon and is typically associated with systemic associations. In contrast, amyloidosis of the conjunctiva is often localised with no other associations. We present a rare case of a 92-year-old gentleman with both cutaneous lid lesions and conjunctival amyloid with no systemic involvement. Biopsy demonstrated the hallmarks of amyloid and treatment has remained conservative. He remains at the department to be monitored for secondary glaucoma. PMID:27752377

  5. Mitral valve involvement as a predominant feature of cardiac amyloidosis

    PubMed Central

    Viswanathan, Girish; Williams, James; Slinn, Simon; Campbell, Philip

    2010-01-01

    Cardiac involvement in systemic amyloidosis carries poor prognosis with a median survival of 5 months.1 The authors report an unusual presentation of cardiac amyloidosis in the form of predominant mitral regurgitation. The patient responded very well to medical therapy with subsequent improvement of mitral valve dysfunction. The authors would like to highlight this multisystem involvement and the presence of a complex overlap of systemic features. PMID:22767536

  6. Amyloidosis of the renal pelvis: a harbinger of mammary carcinoma?

    PubMed

    Grigor, Thomas; Munro, Nicolas

    2015-01-01

    We describe a rare case of light chain immunoglobulin amyloid (AL) accumulation in the central and lower pole renal calyces. Our patient, a woman aged 60, presented with several episodes of gross haematuria. Radiological imaging detected a filling defect in the left renal pelvis. Rigid ureteroscopy showed a corresponding mucosal abnormality resembling transitional cell carcinoma. A definitive preoperative tissue diagnosis could not be reached. Laparoscopic-assisted left nephroureterectomy was indicated. Histopathological examination excluded malignancy, revealing congophilic deposits of submucosal amyloid. A constellation of findings confirmed localised or primary amyloidosis with an AL immunophenotype but no evidence of clonal B-cell disease in the amyloid-associated lymphoplasmacytic cell infiltrate. Investigation for systemic plasma cell dyscrasia and echocardiography and scintigraphy for visceral amyloid deposits were negative for systemic disease. At a follow-up period of 30 months, there is no recurrence. However, our patient was diagnosed with breast cancer 21 months ago. PMID:25596296

  7. [Secondary amyloidosis (AA-type) due to localized cutaneous vasculitis].

    PubMed

    Esteve, V; Ribera, L; Ponz, E; Almirall, J; López, T; Martínez Ocaña, J C; Ibeas, J; Rodríguez Jornet, A; Andreu, X; García, M

    2007-01-01

    We report a case of a 49 year old man, diagnosed soon after the outcome of casual proteinuria, of AA-type amyloidosis in relation to small and medium vessel cutaneous vasculitis without systemic involvement. This combination is a rare entity and only two cases of cutaneous hypersensibility vasculitis complicated with AA-type amyloidosis had been reported. We describe the results of the use of several immunosuppressive drugs during four years follow up with temporally total remission of the disease. PMID:18045042

  8. Combined pulmonary involvement in hereditary lysozyme amyloidosis with associated pulmonary sarcoidosis: a case report.

    PubMed

    McCarthy, Cormac; Deegan, Alexander P; Garvey, John F; McDonnell, Timothy J

    2013-01-01

    Sarcoidosis is a multisystem inflammatory disorder of unknown cause which can affect any organ system. Autosomal dominant lysozyme amyloidosis is a very rare form of hereditary amyloidosis. The Arg64 variant is extraordinarily rare with each family showing a particular pattern of organ involvement, however while Sicca syndrome, gastrointestinal involvement and renal failure are common, lymph node involvement is very rare. In this case report we describe the first reported case of sarcoidosis in association with hereditary lysozyme amyloidosis. PMID:24351625

  9. Amyloid Goiter Associated with Amyloidosis Secondary to Rheumatoid Arthritis

    PubMed Central

    Uzum, Gungor; Kaya, Fatih Oner; Uzum, Ayse Kubat; Kucukyilmaz, Meltem; Duzkoylu, Yigit; Leblebici, Cem; Koc, Oguz

    2013-01-01

    Amyloidosis refers to a variety of conditions in which amyloid proteins are abnormally deposited in organs and/or tissues. The most common forms of systemic amyloidosis are primary amyloidosis (PA) of light chains and secondary amyloidosis (SA) caused by chronic inflammatory diseases such as rheumatoid arthritis (RA). Although involvement of the thyroid gland by amyloid is a relatively common phenomenon, clinically significant enlargement of the thyroid owing to amyloid deposition is a rare occurrence. In SA, the deposition of amyloid associated (AA) protein is associated with atrophy of thyroid follicles. The clinical picture of these patients is characterized by rapid, painless thyroid gland enlargement which may be associated with dysphagia, dyspnea, or hoarseness. Thyroid function is not impaired in most cases. Although amyloid goitre secondary to systemic amyloidosis due to chronic inflammatory diseases is relatively common, specifically related to RA is much more uncommon one and it is reported less in the literature. In this report, A 52-old-year female patient with amyloid goiter associated with amyloidosis secondary to rheumatoid arthritis is presented. PMID:24368922

  10. Amyloidosis in a Captive Zebra Finch (Taeniopygia guttata) Research Colony.

    PubMed

    Shientag, Lisa J; Garlick, David S; Galati, Erin

    2016-01-01

    Five birds in a captive zebra finch research colony were diagnosed with systemic amyloidosis within a 7-mo period by means of postmortem Congo red staining and green birefringence under polarized light. The liver was the most frequently and usually the most seriously affected organ, followed by the spleen and then the kidney. All 5 birds had been clinically affected with various inflammatory, infectious, and neoplastic conditions associated with amyloid A (AA) amyloidosis in humans and animals. Immunohistochemistry using antisera against duck AA protein revealed that tissues from 2 of the 5 birds were positive for the presence of AA protein and systemic inflammation-associated amyloidosis. Although the development of AA amyloidosis has been associated with chronic inflammation, trauma, and various infectious and neoplastic diseases as well as possible genetic predispositions and stresses linked to overcrowding, the root causes for individual cases of AA amyloidosis are incompletely understood. As far as we know, this report is the first description of AA amyloidosis in captive, research zebra finches. PMID:27298248

  11. D25V apolipoprotein C-III variant causes dominant hereditary systemic amyloidosis and confers cardiovascular protective lipoprotein profile.

    PubMed

    Valleix, Sophie; Verona, Guglielmo; Jourde-Chiche, Noémie; Nédelec, Brigitte; Mangione, P Patrizia; Bridoux, Frank; Mangé, Alain; Dogan, Ahmet; Goujon, Jean-Michel; Lhomme, Marie; Dauteuille, Carolane; Chabert, Michèle; Porcari, Riccardo; Waudby, Christopher A; Relini, Annalisa; Talmud, Philippa J; Kovrov, Oleg; Olivecrona, Gunilla; Stoppini, Monica; Christodoulou, John; Hawkins, Philip N; Grateau, Gilles; Delpech, Marc; Kontush, Anatol; Gillmore, Julian D; Kalopissis, Athina D; Bellotti, Vittorio

    2016-01-01

    Apolipoprotein C-III deficiency provides cardiovascular protection, but apolipoprotein C-III is not known to be associated with human amyloidosis. Here we report a form of amyloidosis characterized by renal insufficiency caused by a new apolipoprotein C-III variant, D25V. Despite their uremic state, the D25V-carriers exhibit low triglyceride (TG) and apolipoprotein C-III levels, and low very-low-density lipoprotein (VLDL)/high high-density lipoprotein (HDL) profile. Amyloid fibrils comprise the D25V-variant only, showing that wild-type apolipoprotein C-III does not contribute to amyloid deposition in vivo. The mutation profoundly impacts helical structure stability of D25V-variant, which is remarkably fibrillogenic under physiological conditions in vitro producing typical amyloid fibrils in its lipid-free form. D25V apolipoprotein C-III is a new human amyloidogenic protein and the first conferring cardioprotection even in the unfavourable context of renal failure, extending the evidence for an important cardiovascular protective role of apolipoprotein C-III deficiency. Thus, fibrate therapy, which reduces hepatic APOC3 transcription, may delay amyloid deposition in affected patients. PMID:26790392

  12. D25V apolipoprotein C-III variant causes dominant hereditary systemic amyloidosis and confers cardiovascular protective lipoprotein profile

    PubMed Central

    Valleix, Sophie; Verona, Guglielmo; Jourde-Chiche, Noémie; Nédelec, Brigitte; Mangione, P. Patrizia; Bridoux, Frank; Mangé, Alain; Dogan, Ahmet; Goujon, Jean-Michel; Lhomme, Marie; Dauteuille, Carolane; Chabert, Michèle; Porcari, Riccardo; Waudby, Christopher A.; Relini, Annalisa; Talmud, Philippa J.; Kovrov, Oleg; Olivecrona, Gunilla; Stoppini, Monica; Christodoulou, John; Hawkins, Philip N.; Grateau, Gilles; Delpech, Marc; Kontush, Anatol; Gillmore, Julian D.; Kalopissis, Athina D.; Bellotti, Vittorio

    2016-01-01

    Apolipoprotein C-III deficiency provides cardiovascular protection, but apolipoprotein C-III is not known to be associated with human amyloidosis. Here we report a form of amyloidosis characterized by renal insufficiency caused by a new apolipoprotein C-III variant, D25V. Despite their uremic state, the D25V-carriers exhibit low triglyceride (TG) and apolipoprotein C-III levels, and low very-low-density lipoprotein (VLDL)/high high-density lipoprotein (HDL) profile. Amyloid fibrils comprise the D25V-variant only, showing that wild-type apolipoprotein C-III does not contribute to amyloid deposition in vivo. The mutation profoundly impacts helical structure stability of D25V-variant, which is remarkably fibrillogenic under physiological conditions in vitro producing typical amyloid fibrils in its lipid-free form. D25V apolipoprotein C-III is a new human amyloidogenic protein and the first conferring cardioprotection even in the unfavourable context of renal failure, extending the evidence for an important cardiovascular protective role of apolipoprotein C-III deficiency. Thus, fibrate therapy, which reduces hepatic APOC3 transcription, may delay amyloid deposition in affected patients. PMID:26790392

  13. Leukocyte Cell-Derived Chemotaxin 2-Associated Amyloidosis: A Recently Recognized Disease with Distinct Clinicopathologic Characteristics.

    PubMed

    Nasr, Samih H; Dogan, Ahmet; Larsen, Christopher P

    2015-11-01

    Amyloidosis derived from leukocyte cell-derived chemotaxin 2 is a recently recognized form of amyloidosis, and it has already been established as a frequent form of systemic amyloidosis in the United States, with predominant involvement of kidney and liver. The disease has a strong ethnic bias, affecting mainly Hispanics (particularly Mexicans). Additional ethnic groups prone to develop amyloidosis derived from leukocyte cell-derived chemotaxin 2 include Punjabis, First Nations people in British Columbia, and Native Americans. Most patients are elderly who present with chronic renal insufficiency and bland urinary sediment. Proteinuria is variable, being absent altogether in about one third of patients. Liver involvement is frequently an incidental finding. Amyloidosis derived from leukocyte cell-derived chemotaxin 2 deposits shows a characteristic distribution: in the kidney, there is consistent involvement of cortical interstitium, whereas in the liver, there is a preferential involvement of periportal and pericentral vein regions. Concurrent renal disease is frequent, with diabetic nephropathy and IgA nephropathy being the most common. Patient survival is excellent, likely because of the rarity of cardiac involvement, whereas renal survival is guarded, with a median renal survival of 62 months in those without concurrent renal disease. There is currently no efficacious therapy for amyloidosis derived from leukocyte cell-derived chemotaxin 2 amyloidosis. Renal transplantation seems to be a reasonable treatment for patients with advanced renal failure, although the disease may recur in the allograft. The pathogenesis of amyloidosis derived from leukocyte cell-derived chemotaxin 2 amyloidosis has not yet been elucidated. It could be a result of leukocyte cell-derived chemotaxin 2 overexpression by hepatocytes either constitutively (controlled by yet-uncharacterized genetic defects) or secondary to hepatocellular damage. It is critical not to misdiagnose amyloidosis

  14. Leukocyte Cell-Derived Chemotaxin 2-Associated Amyloidosis: A Recently Recognized Disease with Distinct Clinicopathologic Characteristics.

    PubMed

    Nasr, Samih H; Dogan, Ahmet; Larsen, Christopher P

    2015-11-01

    Amyloidosis derived from leukocyte cell-derived chemotaxin 2 is a recently recognized form of amyloidosis, and it has already been established as a frequent form of systemic amyloidosis in the United States, with predominant involvement of kidney and liver. The disease has a strong ethnic bias, affecting mainly Hispanics (particularly Mexicans). Additional ethnic groups prone to develop amyloidosis derived from leukocyte cell-derived chemotaxin 2 include Punjabis, First Nations people in British Columbia, and Native Americans. Most patients are elderly who present with chronic renal insufficiency and bland urinary sediment. Proteinuria is variable, being absent altogether in about one third of patients. Liver involvement is frequently an incidental finding. Amyloidosis derived from leukocyte cell-derived chemotaxin 2 deposits shows a characteristic distribution: in the kidney, there is consistent involvement of cortical interstitium, whereas in the liver, there is a preferential involvement of periportal and pericentral vein regions. Concurrent renal disease is frequent, with diabetic nephropathy and IgA nephropathy being the most common. Patient survival is excellent, likely because of the rarity of cardiac involvement, whereas renal survival is guarded, with a median renal survival of 62 months in those without concurrent renal disease. There is currently no efficacious therapy for amyloidosis derived from leukocyte cell-derived chemotaxin 2 amyloidosis. Renal transplantation seems to be a reasonable treatment for patients with advanced renal failure, although the disease may recur in the allograft. The pathogenesis of amyloidosis derived from leukocyte cell-derived chemotaxin 2 amyloidosis has not yet been elucidated. It could be a result of leukocyte cell-derived chemotaxin 2 overexpression by hepatocytes either constitutively (controlled by yet-uncharacterized genetic defects) or secondary to hepatocellular damage. It is critical not to misdiagnose amyloidosis

  15. Multifocal fibrosclerosis and renal amyloidosis.

    PubMed Central

    Hamilton, D. V.; Thiru, S.

    1983-01-01

    A 34-year-old female with a 14-year history of multifocal fibrosclerosis developed the nephrotic syndrome due to renal amyloidosis. Although the association between chronic inflammatory processes and amyloidosis is well known, this is the first report of amyloidosis occurring in a patient with multifocal fibrosclerosis. Other interesting features of this case are the involvement of subcutaneous adipose tissue in the inflammatory-fibrotic process and the presence of features of multifocal fibrosclerosis in the patient's twin sister. Images Fig. 1 PMID:6622334

  16. AA amyloidosis in the renal allograft: a report of two cases and review of the literature.

    PubMed

    Rojas, Rebecca; Josephson, Michelle A; Chang, Anthony; Meehan, Shane M

    2012-04-01

    AA amyloidosis is a disorder characterized by the abnormal formation, accumulation and systemic deposition of fibrillary material that frequently involves the kidney. Recurrent AA amyloidosis in the renal allograft has been documented in patients with tuberculosis, familial Mediterranean fever, ankylosing spondylitis, chronic pyelonephritis and rheumatoid arthritis. De novo AA amyloidosis is rarely described. We report two cases of AA amyloidosis in the renal allograft. Our first case is a 47-year-old male with a history of ankylosing spondylitis who developed end-stage renal disease reportedly from tubulointerstitial nephritis from non-steroidal anti-inflammatory agent use. A biopsy was never performed. One year after transplantation, AA amyloidosis was identified in the femoral head and 8 years post-transplantation, AA amyloidosis was identified in the renal allograft. He was treated with colchicine and adalimumab and has stable renal function at 1 year-follow-up. Our second case is a 57-year-old male with a long history of intravenous drug use and hepatitis C infection who developed end-stage kidney disease due to AA amyloidosis. Our second patient's course was complicated by renal adenovirus, pulmonary aspergillosis and hepatitis C with AA amyloidosis subsequently being identified in the allograft 2.5 years post-transplantation. Renal allograft function remains stable 4-years post-transplantation. These reports describe clinical and pathologic features of two cases of AA amyloidosis presenting with proteinuria and focal involvement of the renal allograft.

  17. Dystrophic amyloidosis: a local complication of tissue damage with heterogeneous distribution.

    PubMed

    Looi, L M

    1991-08-01

    Seventeen consecutive patients with dystrophic amyloidosis are reported here (eight Chinese, three Indian, three Iban, two Malay and one Caucasian). Ten were females and seven males, with ages ranging from 12 to 80 years (mean of 48 years). Five instances of dystrophic amyloidosis occurred in areas of tissue damage in the cardiovascular system, including fibrotic cardiac valves and an atheromatous plaque. Three occurred in osteoarthritic joint tissue. Of note were three occurrences in endometriotic cyst walls, four in the fibrotic walls of epidermal cysts, one in a hernial sac and one at the edge of a skin ulcer. All deposits were congophilic and exhibited green-birefringence and permanganate-resistance. Immunohistochemistry did not reveal reactivity for AA protein or immunoglobulin lambda or kappa light-chains. AP protein was detected in 35% of cases. Our results show that, besides the usual sites of osteoarthritic joints and damaged heart valves, dystrophic amyloidosis can complicate other areas of chronic tissue damage and fibrosis such as walls of cysts and ulcers. While the pathogenesis and biochemical nature remain unresolved, immunohistochemistry indicates that neither AA nor AL proteins are present in the deposits, and suggests that a different amyloid protein is involved. PMID:1757071

  18. Corneal amyloidosis associated with keratoconus.

    PubMed

    Stern, G A; Knapp, A; Hood, C I

    1988-01-01

    Nodular, gray-white, central corneal opacities which extended from the subepithelial zone through the anterior four fifths of the stroma developed in a 50-year-old man with a longstanding history of hard contact lens wear for keratoconus. Results of histopathologic analysis of the corneal button obtained at the time of penetrating keratoplasty disclosed that the opacities were composed of amyloid. Corneal amyloidosis is rarely found in association with keratoconus. Although there were some similarities in the pattern of amyloid deposition to that seen in primary familial amyloidosis of the cornea, the authors believe that their patient is more likely to have had a secondary amyloidosis. Corneal amyloidosis should be considered in keratoconus patients with development of unusual forms of central corneal opacification. PMID:3278260

  19. Hereditary Transthyretin Amyloidosis in Eight Chinese Families

    PubMed Central

    Meng, Ling-Chao; Lyu, He; Zhang, Wei; Liu, Jing; Wang, Zhao-Xia; Yuan, Yun

    2015-01-01

    Background: Mutations of transthyretin (TTR) cause the most common type of autosomal-dominant hereditary systemic amyloidosis, which occurs worldwide. To date, more and more mutations in the TTR gene have been reported. Some variations in the clinical presentation are often observed in patients with the same mutation or the patients in the same family. The purpose of this study was to find out the clinicopathologic and genetic features of Chinese patients with hereditary TTR amyloidosis. Methods: Clinical and necessary examination materials were collected from nine patients of eight families with hereditary TTR amyloidosis at Peking University First Hospital from January 2007 to November 2014. Sural nerve biopsies were taken for eight patients and skin biopsies were taken in the calf/upper arm for two patients, for light and electron microscopy examination. The TTR genes from the nine patients were analyzed. Results: The onset age varied from 23 to 68 years. The main manifestations were paresthesia, proximal and/or distal weakness, autonomic dysfunction, cardiomyopathy, vitreous opacity, hearing loss, and glossohypertrophia. Nerve biopsy demonstrated severe loss of myelinated fibers in seven cases and amyloid deposits in three. One patient had skin amyloid deposits which were revealed from electron microscopic examination. Genetic analysis showed six kinds of mutations of TTR gene, including Val30Met, Phe33Leu, Ala36Pro, Val30Ala, Phe33Val, and Glu42Gly in exon 2. Conclusions: Since the pathological examinations of sural nerve were negative for amyloid deposition in most patients, the screening for TTR mutations should be performed in all the adult patients, who are clinically suspected with hereditary TTR amyloidosis. PMID:26521788

  20. Amyloidosis in black-footed cats (Felis nigripes).

    PubMed

    Terio, K A; O'Brien, T; Lamberski, N; Famula, T R; Munson, L

    2008-05-01

    A high prevalence of systemic amyloidosis was documented in the black-footed cat (Felis nigripes) based on a retrospective review of necropsy tissues (n = 38) submitted as part of ongoing disease surveillance. Some degree of amyloid deposition was present in 33 of 38 (87%) of the examined cats, and amyloidosis was the most common cause of death (26/38, 68%). Amyloid deposition was most severe in the renal medullary interstitium (30/33, 91%) and glomeruli (21/33, 63%). Other common sites included the splenic follicular germinal centers (26/31, 84%), gastric lamina propria (9/23, 39%), and intestinal lamina propria (3/23, 13%). Amyloid in all sites stained with Congo red, and in 13 of 15 (87%) cats, deposits had strong immunoreactivity for canine AA protein by immunohistochemistry. There was no association with concurrent chronic inflammatory conditions (P = .51), suggesting that amyloidosis was not secondary to inflammation. Adrenal cortical hyperplasia, a morphologic indicator of stress that can predispose to amyloid deposition, was similarly not associated (P = .09) with amyloidosis. However, adrenals were not available from the majority of cats without amyloidosis; therefore, further analysis of this risk factor is warranted. Heritability estimation suggested that amyloidosis might be familial in this species. Additionally, tissues from a single free-ranging black-footed cat had small amounts of amyloid deposition, suggesting that there could be a predilection for amyloidosis in this species. Research to identify the protein sequence of serum amyloid A (SAA) in the black-footed cat is needed to further investigate the possibility of an amyloidogenic SAA in this species. PMID:18487501

  1. Amyloidosis in black-footed cats (Felis nigripes).

    PubMed

    Terio, K A; O'Brien, T; Lamberski, N; Famula, T R; Munson, L

    2008-05-01

    A high prevalence of systemic amyloidosis was documented in the black-footed cat (Felis nigripes) based on a retrospective review of necropsy tissues (n = 38) submitted as part of ongoing disease surveillance. Some degree of amyloid deposition was present in 33 of 38 (87%) of the examined cats, and amyloidosis was the most common cause of death (26/38, 68%). Amyloid deposition was most severe in the renal medullary interstitium (30/33, 91%) and glomeruli (21/33, 63%). Other common sites included the splenic follicular germinal centers (26/31, 84%), gastric lamina propria (9/23, 39%), and intestinal lamina propria (3/23, 13%). Amyloid in all sites stained with Congo red, and in 13 of 15 (87%) cats, deposits had strong immunoreactivity for canine AA protein by immunohistochemistry. There was no association with concurrent chronic inflammatory conditions (P = .51), suggesting that amyloidosis was not secondary to inflammation. Adrenal cortical hyperplasia, a morphologic indicator of stress that can predispose to amyloid deposition, was similarly not associated (P = .09) with amyloidosis. However, adrenals were not available from the majority of cats without amyloidosis; therefore, further analysis of this risk factor is warranted. Heritability estimation suggested that amyloidosis might be familial in this species. Additionally, tissues from a single free-ranging black-footed cat had small amounts of amyloid deposition, suggesting that there could be a predilection for amyloidosis in this species. Research to identify the protein sequence of serum amyloid A (SAA) in the black-footed cat is needed to further investigate the possibility of an amyloidogenic SAA in this species.

  2. Serum amyloid A and protein AA: molecular mechanisms of a transmissible amyloidosis.

    PubMed

    Westermark, Gunilla T; Westermark, Per

    2009-08-20

    Systemic AA-amyloidosis is a complication of chronic inflammatory diseases and the fibril protein AA derives from the acute phase reactant serum AA. AA-amyloidosis can be induced in mice by an inflammatory challenge. The lag phase before amyloid develops can be dramatically shortened by administration of a small amount of amyloid fibrils. Systemic AA-amyloidosis is transmissible in mice and may be so in humans. Since transmission can cross species barriers it is possible that AA-amyloidosis can be induced by amyloid in food, e.g. foie gras. In mice, development of AA-amyloidosis can also be accelerated by other components with amyloid-like properties. A new possible risk factor may appear with synthetically made fibrils from short peptides, constructed for tissue repair.

  3. Transthyretin Amyloidosis: Chaperone Concentration Changes and Increased Proteolysis in the Pathway to Disease

    PubMed Central

    Ribeiro, Raquel; Gilberto, Samuel; Gomes, Ricardo A.; Ferreira, António; Mateus, Élia; Barroso, Eduardo; Coelho, Ana V.; Freire, Ana Ponces; Cordeiro, Carlos

    2015-01-01

    Transthyretin amyloidosis is a conformational pathology characterized by the extracellular formation of amyloid deposits and the progressive impairment of the peripheral nervous system. Point mutations in this tetrameric plasma protein decrease its stability and are linked to disease onset and progression. Since non-mutated transthyretin also forms amyloid in systemic senile amyloidosis and some mutation bearers are asymptomatic throughout their lives, non-genetic factors must also be involved in transthyretin amyloidosis. We discovered, using a differential proteomics approach, that extracellular chaperones such as fibrinogen, clusterin, haptoglobin, alpha-1-anti-trypsin and 2-macroglobulin are overrepresented in transthyretin amyloidosis. Our data shows that a complex network of extracellular chaperones are over represented in human plasma and we speculate that they act synergistically to cope with amyloid prone proteins. Proteostasis may thus be as important as point mutations in transthyretin amyloidosis. PMID:26147092

  4. Amyloidosis and Waldenström's macroglobulinemia.

    PubMed

    Gertz, Morie A; Merlini, Giampaolo; Treon, Steven P

    2004-01-01

    Primary systemic amyloidosis is an immunoglobulin light chain disorder that is 1/5th as common as multiple myeloma. Amyloidosis is regularly seen in the practice of a hematologist and has recently undergone major advances in terms of the ability to evaluate responses as well as new therapeutic options that were not available when this topic was covered as an education session at the American Society of Hematology meeting 5 years ago. Waldenström macroglobulinemia (WM) is rarer than amyloidosis (1500 per year WM versus 3000 per year amyloid in the US), and recent consensus panels have established the definition of the disease, the diagnostic criteria, criteria for initiation of therapy and a new classification scheme. In this session, new developments in amyloid and macroglobulinemia, from suspicion of the diagnosis to treatment, are covered. In Section I, Dr. Morie Gertz answers four specific questions: (1) When should amyloidosis be suspected? (2) How does one heighten ones index of suspicion for amyloid? (3) How is the diagnosis confirmed and the type classified as primary? (4) What is the prognosis and how is it accurately assessed? Recent findings on cardiac biomarkers, presenting features and use of the free light chain assay are reviewed. Staging for amyloid and recently proposed criteria of response and progression are covered. In Section II, Dr. Giampaolo Merlini comprehensively reviews therapy of amyloidosis from the use of standard melphalan/prednisone to the recently described standard dose therapies including dexamethasone, thalidomide/dexamethasone, melphalan/dexamethasone and IV melphalan/dexamethasone. An extensive discussion of the role of high-dose therapy with stem cell reconstitution follows and includes patient selection, predictors of immediate morbidity and mortality, and survival expectation. Finally, a therapeutic strategy is proposed. In Section III, Drs. Steven Treon and Giampaolo Merlini review the most current information on WM. The

  5. [Laryngeal amyloidosis: a clinical case and review of literature].

    PubMed

    Fries, S; Pasche, P; Brunel, C; Schweizer, V

    2015-09-30

    Amyloidosis consists of different forms of systemic or isolated organ lesions characterised by fibrillary protein deposits in extra-cellular tissue. The isolated involvement of the larynx is the most frequent form in the ENT sphere. We present a clinical case of a 67 year-old woman addressed for a sub-acute laryngitis resistant to conservative treatment, and finally diagnosed with laryngeal amyloidosis. We reviewed its physiopathology, the scientific literature as well as the different possibilities of management. Laryngeal amyloidosis is rare. A thorough additional work-up for the research of multifocal or systemic forms is highly recommended. The treatment aims at a minimal invasive endoscopic surgery with functional organ preservation.

  6. [Hepatic amyloidosis as a rare differential diagnosis of progressive liver failure].

    PubMed

    Bettinger, Dominik; Lutz, Lisa; Schultheiß, Michael; Werner, Martin; Thimme, Robert; Neumann-Haefelin, Christoph

    2016-09-01

    Primary systemic amyloidosis is a rare disorder resulting in extracellular deposition of insoluble fibrils in different organs. Liver involvement has been reported. Since hepatic amyloidosis often presents clinically asymptomatic without specific laboratory or imaging hallmarks, diagnosis is challenging. However, cases of progressive hepatic failure due to liver amyloidosis have been reported. A 63 year old man presented with newly diagnosed ascites to our department. The patient reported occasional alcohol consumption. Viral hepatitis, genetic-metabolic causes as well as hepatic vascular disorders were excluded and ultrasound did not show any signs of liver cirrhosis or intraabdominal malignancy. Initially, alcoholic hepatitis was suspected. Due to the rapid deterioration of liver function, however, transjugular liver biopsy was performed showing light chain amyloidosis of kappa isotype. As diagnosis of hepatic amyloidosis is challenging, early liver biopsy is mandatory in patients with unexplained acute or chronic liver disease to exclude rare diseases with high mortality. PMID:27642740

  7. [Hepatic amyloidosis as a rare differential diagnosis of progressive liver failure].

    PubMed

    Bettinger, Dominik; Lutz, Lisa; Schultheiß, Michael; Werner, Martin; Thimme, Robert; Neumann-Haefelin, Christoph

    2016-09-01

    Primary systemic amyloidosis is a rare disorder resulting in extracellular deposition of insoluble fibrils in different organs. Liver involvement has been reported. Since hepatic amyloidosis often presents clinically asymptomatic without specific laboratory or imaging hallmarks, diagnosis is challenging. However, cases of progressive hepatic failure due to liver amyloidosis have been reported. A 63 year old man presented with newly diagnosed ascites to our department. The patient reported occasional alcohol consumption. Viral hepatitis, genetic-metabolic causes as well as hepatic vascular disorders were excluded and ultrasound did not show any signs of liver cirrhosis or intraabdominal malignancy. Initially, alcoholic hepatitis was suspected. Due to the rapid deterioration of liver function, however, transjugular liver biopsy was performed showing light chain amyloidosis of kappa isotype. As diagnosis of hepatic amyloidosis is challenging, early liver biopsy is mandatory in patients with unexplained acute or chronic liver disease to exclude rare diseases with high mortality.

  8. [Osteoarticular amyloidosis caused by dialysis].

    PubMed

    Orzincolo, C; Bedani, P L; Scutellari, P N; Cardona, P; Farinelli, A; Vita, G

    1988-01-01

    The accumulation of amyloid in the bone and joint system has recently been recognized as a peculiar disease in patients undergoing long-term hemodialysis (5 years at least), especially in those who use cuprophan membranes. The pathology of amyloidosis is characterized by deposits of amyloid (beta 2-microglobulin mainly) in the bone, in the synovia, and in pericapsular soft tissues. The skeleton of 46 long-term hemodialysis patients (19 males and 27 females) was studied by X-ray: bone and joint abnormalities due to amyloid deposition were observed in 45% of cases. The shoulder, hip, and wrist were the most frequently involved joints. Destructive spondyloarthropathy was present in 15% of cases. The radiographic patterns of AOD are generally divided into axial and peripheral lesions. In the appendicular skeleton abnormalities include: well-defined lytic areas (geodes), pathologic fractures, marginal erosions, and periarticular soft tissue swelling. Destructive spondyloarthropathy is frequently present in the cervical spine (85% of our cases), and is characterized by narrowing of the intervertebral space, marginal erosion, and subchondral bone sclerosis of the vertebral body.

  9. Dynamic Modeling of ALS Systems

    NASA Technical Reports Server (NTRS)

    Jones, Harry

    2002-01-01

    The purpose of dynamic modeling and simulation of Advanced Life Support (ALS) systems is to help design them. Static steady state systems analysis provides basic information and is necessary to guide dynamic modeling, but static analysis is not sufficient to design and compare systems. ALS systems must respond to external input variations and internal off-nominal behavior. Buffer sizing, resupply scheduling, failure response, and control system design are aspects of dynamic system design. We develop two dynamic mass flow models and use them in simulations to evaluate systems issues, optimize designs, and make system design trades. One model is of nitrogen leakage in the space station, the other is of a waste processor failure in a regenerative life support system. Most systems analyses are concerned with optimizing the cost/benefit of a system at its nominal steady-state operating point. ALS analysis must go beyond the static steady state to include dynamic system design. All life support systems exhibit behavior that varies over time. ALS systems must respond to equipment operating cycles, repair schedules, and occasional off-nominal behavior or malfunctions. Biological components, such as bioreactors, composters, and food plant growth chambers, usually have operating cycles or other complex time behavior. Buffer sizes, material stocks, and resupply rates determine dynamic system behavior and directly affect system mass and cost. Dynamic simulation is needed to avoid the extremes of costly over-design of buffers and material reserves or system failure due to insufficient buffers and lack of stored material.

  10. The Successful Diagnosis and Typing of Systemic Amyloidosis Using A Microwave-Assisted Filter-Aided Fast Sample Preparation Method and LC/MS/MS Analysis.

    PubMed

    Sun, Weiyi; Sun, Jian; Zou, Lili; Shen, Kaini; Zhong, Dingrong; Zhou, Daobin; Sun, Wei; Li, Jian

    2015-01-01

    Laser microdissection followed by mass spectrometry has been successfully used for amyloid typing. However, sample contamination can interfere with proteomic analysis, and overnight digestion limits the analytical throughput. Moreover, current quantitative analysis methods are based on the spectrum count, which ignores differences in protein length and may lead to misdiagnoses. Here, we developed a microwave-assisted filter-aided sample preparation (maFASP) method that can efficiently remove contaminants with a 10-kDa cutoff ultrafiltration unit and can accelerate the digestion process with the assistance of a microwave. Additionally, two parameters (P- and D-scores) based on the exponentially modified protein abundance index were developed to define the existence of amyloid deposits and those causative proteins with the greatest abundance. Using our protocol, twenty cases of systemic amyloidosis that were well-typed according to clinical diagnostic standards (training group) and another twenty-four cases without subtype diagnoses (validation group) were analyzed. Using this approach, sample preparation could be completed within four hours. We successfully subtyped 100% of the cases in the training group, and the diagnostic success rate in the validation group was 91.7%. This maFASP-aided proteomic protocol represents an efficient approach for amyloid diagnosis and subtyping, particularly for serum-contaminated samples. PMID:25984759

  11. The Successful Diagnosis and Typing of Systemic Amyloidosis Using A Microwave-Assisted Filter-Aided Fast Sample Preparation Method and LC/MS/MS Analysis

    PubMed Central

    Zou, Lili; Shen, Kaini; Zhong, Dingrong; Zhou, Daobin; Sun, Wei; Li, Jian

    2015-01-01

    Laser microdissection followed by mass spectrometry has been successfully used for amyloid typing. However, sample contamination can interfere with proteomic analysis, and overnight digestion limits the analytical throughput. Moreover, current quantitative analysis methods are based on the spectrum count, which ignores differences in protein length and may lead to misdiagnoses. Here, we developed a microwave-assisted filter-aided sample preparation (maFASP) method that can efficiently remove contaminants with a 10-kDa cutoff ultrafiltration unit and can accelerate the digestion process with the assistance of a microwave. Additionally, two parameters (P- and D-scores) based on the exponentially modified protein abundance index were developed to define the existence of amyloid deposits and those causative proteins with the greatest abundance. Using our protocol, twenty cases of systemic amyloidosis that were well-typed according to clinical diagnostic standards (training group) and another twenty-four cases without subtype diagnoses (validation group) were analyzed. Using this approach, sample preparation could be completed within four hours. We successfully subtyped 100% of the cases in the training group, and the diagnostic success rate in the validation group was 91.7%. This maFASP-aided proteomic protocol represents an efficient approach for amyloid diagnosis and subtyping, particularly for serum-contaminated samples. PMID:25984759

  12. Diagnostic approach to cardiac amyloidosis: A case report.

    PubMed

    Fernandes, Andreia; Caetano, Francisca; Almeida, Inês; Paiva, Luís; Gomes, Pedro; Mota, Paula; Trigo, Joana; Botelho, Ana; Cachulo, Maria do Carmo; Alves, Joana; Francisco, Luís; Leitão Marques, António

    2016-05-01

    The authors present a case of systemic amyloidosis with cardiac involvement. We discuss the need for a high level of suspicion to establish a diagnosis, diagnostic techniques and treatment options. Our patient was a 78-year-old man with chronic renal disease and atrial fibrillation admitted with acute decompensated heart failure of unknown cause. The transthoracic echocardiogram revealed severely impaired left ventricular function with phenotypic overlap between hypertrophic and restrictive cardiomyopathy. After an extensive diagnostic workup, which included an abdominal fat pad biopsy, the final diagnosis was amyloidosis.

  13. The Advanced Launch System (ALS)

    NASA Astrophysics Data System (ADS)

    Eldred, Charles H.

    The Advanced Launch System (ALS) is an unmanned vehicle that will achieve low hardware cost by using a reusable booster stage which flies back to the launch site, and a core stage in which the rocket engines and redundant avionics are in a module that is returned to earth and recovered for reuse. The booster's utilization of liquid propellant instead of solid propellant will help lower the consumable costs. The ALS also includes launch processing and flight control facilities, necessary support equipment, and ground- and flight-operations infrastructure. The ALS program studies show that, through the ALS, the United States can launch a major Mars initiative economically and with confidence. It is estimated that the objective ALS can be operational in the late 1990s.

  14. Molecular tweezers targeting transthyretin amyloidosis.

    PubMed

    Ferreira, Nelson; Pereira-Henriques, Alda; Attar, Aida; Klärner, Frank-Gerrit; Schrader, Thomas; Bitan, Gal; Gales, Luís; Saraiva, Maria João; Almeida, Maria Rosário

    2014-04-01

    Transthyretin (TTR) amyloidoses comprise a wide spectrum of acquired and hereditary diseases triggered by extracellular deposition of toxic TTR aggregates in various organs. Despite recent advances regarding the elucidation of the molecular mechanisms underlying TTR misfolding and pathogenic self-assembly, there is still no effective therapy for treatment of these fatal disorders. Recently, the "molecular tweezers", CLR01, has been reported to inhibit self-assembly and toxicity of different amyloidogenic proteins in vitro, including TTR, by interfering with hydrophobic and electrostatic interactions known to play an important role in the aggregation process. In addition, CLR01 showed therapeutic effects in animal models of Alzheimer's disease and Parkinson's disease. Here, we assessed the ability of CLR01 to modulate TTR misfolding and aggregation in cell culture and in an animal model. In cell culture assays we found that CLR01 inhibited TTR oligomerization in the conditioned medium and alleviated TTR-induced neurotoxicity by redirecting TTR aggregation into the formation of innocuous assemblies. To determine whether CLR01 was effective in vivo, we tested the compound in mice expressing TTR V30M, a model of familial amyloidotic polyneuropathy, which recapitulates the main pathological features of the human disease. Immunohistochemical and Western blot analyses showed a significant decrease in TTR burden in the gastrointestinal tract and the peripheral nervous system in mice treated with CLR01, with a concomitant reduction in aggregate-induced endoplasmic reticulum stress response, protein oxidation, and apoptosis. Taken together, our preclinical data suggest that CLR01 is a promising lead compound for development of innovative, disease-modifying therapy for TTR amyloidosis.

  15. Cardiac Amyloidosis: Typical Imaging Findings and Diffuse Myocardial Damage Demonstrated by Delayed Contrast-Enhanced MRI

    SciTech Connect

    Sueyoshi, Eijun Sakamoto, Ichiro; Okimoto, Tomoaki; Hayashi, Kuniaki; Tanaka, Kyouei; Toda, Genji

    2006-08-15

    Amyloidosis is a rare systemic disease. However, involvement of the heart is a common finding and is the most frequent cause of death in amyloidosis. We report the sonographic, scintigraphic, and MRI features of a pathologically proven case of cardiac amyloidosis. Delayed contrast-enhanced MR images, using an inversion recovery prepped gradient-echo sequence, revealed diffuse enhancement in the wall of both left and right ventricles. This enhancement suggested expansion of the extracellular space of the myocardium caused by diffuse myocardial necrosis secondary to deposition of amyloid.

  16. THE AUTOIMMUNE CONSTELLATION IN LICHEN AMYLOIDOSIS.

    PubMed

    Andrese, Elena; Vâţă, D; Ciobanu, Delia; Stătescu, Laura; Solovăstru, Laura Gheucă

    2015-01-01

    Localized cutaneous amyloidosis is a rare disease among white people, being more common in South-Asia, China and South America. The disease is characterized by deposition of amyloid material in the papillary dermis without visceral involvement. Nevertheless, there is a growing list of immune-mediated disorders that have been linked to cutaneous amyloidosis. We present two cases of concomitant occurrence of lichen amyloidosis and autoimmune thyroiditis/atopic dermatitis in two Caucasian women. PMID:26793847

  17. THE AUTOIMMUNE CONSTELLATION IN LICHEN AMYLOIDOSIS.

    PubMed

    Andrese, Elena; Vâţă, D; Ciobanu, Delia; Stătescu, Laura; Solovăstru, Laura Gheucă

    2015-01-01

    Localized cutaneous amyloidosis is a rare disease among white people, being more common in South-Asia, China and South America. The disease is characterized by deposition of amyloid material in the papillary dermis without visceral involvement. Nevertheless, there is a growing list of immune-mediated disorders that have been linked to cutaneous amyloidosis. We present two cases of concomitant occurrence of lichen amyloidosis and autoimmune thyroiditis/atopic dermatitis in two Caucasian women.

  18. Single Nodular Pulmonary Amyloidosis: Case Report

    PubMed Central

    Ko, Young Chun; Jeong, Jong Pil; Park, Chan Woo; Seo, Seok Ho; Kim, Jong Taek; Park, Dae Won; Bak, Cheol Min; Moon, Seung Ki; Jo, Shin Hyoung; Kim, Se Mi; Jung, Ah Lon

    2015-01-01

    Amyloidosis is defined as the presence of extra-cellular deposits of an insoluble fibrillar protein, amyloid. The pulmonary involvement of amyloidosis is usually classified as tracheobronchial, parenchymal nodular, or diffuse alveolar septal. A single nodular lesion can mimic various conditions, including malignancy, pulmonary tuberculosis, and fungal infection. To date, only one case of nodular pulmonary amyloidosis has been reported in Korea, a case involving multiple nodular lesions. Here, we report and discuss the case of a patient having single nodular amyloidosis. PMID:26508930

  19. Extrusion of amyloid fibrils to the extracellular space in experimental mesangial AL-amyloidosis: transmission and scanning electron microscopy studies and correlation with renal biopsy observations.

    PubMed

    Teng, Jiamin; Turbat-Herrera, Elba A; Herrera, Guillermo A

    2014-04-01

    In vitro studies have provided much information regarding the process of glomerular AL-amyloidogenesis. Research efforts have been successful in deciphering how glomerulopathic light chains interact with mesangial cells. The sequential steps involved in the genesis of amyloid fibrils include interactions with surface caveolae in mesangial cells and internalization of the monoclonal light chains through a clathrin-mediated process followed by trafficking in the mesangial cells to the mature lysosomal compartment where fibrils are formed. This manuscript focuses on how mesangial cells, once amyloid has been formed, deliver the fibrils to the extracellular matrix. The delivery of amyloid fibrils to the outside of the cells is carried out by lysosomes, which abut the mesangial cell membranes and extrude their contents into the extracellular space. This final step responsible for the fibrils to be present predominantly in the extracellular space is well demonstrated with scanning electron microscopy.

  20. Persistent Release of IL-1s from Skin Is Associated with Systemic Cardio-Vascular Disease, Emaciation and Systemic Amyloidosis: The Potential of Anti-IL-1 Therapy for Systemic Inflammatory Diseases

    PubMed Central

    Yamanaka, Keiichi; Nakanishi, Takehisa; Saito, Hiromitsu; Maruyama, Junko; Isoda, Kenichi; Yokochi, Ayumu; Imanaka-Yoshida, Kyoko; Tsuda, Kenshiro; Kakeda, Masato; Okamoto, Ryuji; Fujita, Satoshi; Iwakura, Yoichiro; Suzuki, Noboru; Ito, Masaaki; Maruyama, Kazuo; Gabazza, Esteban C.; Yoshida, Toshimichi; Shimaoka, Motomu; Mizutani, Hitoshi

    2014-01-01

    The skin is an immune organ that contains innate and acquired immune systems and thus is able to respond to exogenous stimuli producing large amount of proinflammatory cytokines including IL-1 and IL-1 family members. The role of the epidermal IL-1 is not limited to initiation of local inflammatory responses, but also to induction of systemic inflammation. However, association of persistent release of IL-1 family members from severe skin inflammatory diseases such as psoriasis, epidermolysis bullosa, atopic dermatitis, blistering diseases and desmoglein-1 deficiency syndrome with diseases in systemic organs have not been so far assessed. Here, we showed the occurrence of severe systemic cardiovascular diseases and metabolic abnormalities including aberrant vascular wall remodeling with aortic stenosis, cardiomegaly, impaired limb and tail circulation, fatty tissue loss and systemic amyloid deposition in multiple organs with liver and kidney dysfunction in mouse models with severe dermatitis caused by persistent release of IL-1s from the skin. These morbid conditions were ameliorated by simultaneous administration of anti-IL-1α and IL-1β antibodies. These findings may explain the morbid association of arteriosclerosis, heart involvement, amyloidosis and cachexia in severe systemic skin diseases and systemic autoinflammatory diseases, and support the value of anti-IL-1 therapy for systemic inflammatory diseases. PMID:25119884

  1. Duodenal Amyloidosis Masquerading as Iron Deficiency Anemia

    PubMed Central

    Hurairah, Abu

    2016-01-01

    The present study is a unique illustration of duodenal amyloidosis initially manifesting with iron deficiency anemia. It underscores the importance of clinical suspicion of amyloidosis while performing upper gastrointestinal endoscopy with a biopsy to establish the definite diagnosis in patients with unexplained iron deficiency anemia. PMID:27625911

  2. Tissue distribution of amyloid deposits in Abyssinian cats with familial amyloidosis.

    PubMed

    DiBartola, S P; Tarr, M J; Benson, M D

    1986-07-01

    The tissue distribution of amyloid deposits was studied in 15 related Abyssinian cats with familial amyloidosis. There was interstitial medullary amyloidosis in the kidneys of all 15 cats but only 11 had detectable glomerular involvement. The thyroid glands, stomach and colon were affected in all cats examined. Most of the cats also had amyloid deposits in the small intestine, spleen, heart, adrenals, pancreas, liver, lymph nodes and bladder. In 50 per cent or fewer of the cats examined, there was involvement of the parathyroids, lung and gonads. The central nervous system was not involved in any of the 3 cats evaluated. In 8 of the cats, no concurrent inflammatory disease could be detected. The tissue distribution of amyloid deposits resembled that found in other breeds of domestic cats with systemic amyloidosis. Despite the wide tissue distribution of amyloid deposits, clinical signs were related to renal amyloidosis. Familial amyloidosis in the Abyssinian cat may represent a valuable spontaneous animal model for the study of Familial Mediterranean Fever in man and the pathogenesis of reactive amyloidosis in general. PMID:3734172

  3. Tracheobronchial amyloidosis and confocal endomicroscopy.

    PubMed

    Newton, Richard C; Kemp, Samuel V; Yang, Guang-Zhong; Darzi, Ara; Sheppard, Mary N; Shah, Pallav L

    2011-01-01

    Tracheobronchial amyloidosis is one of many causes of endobronchial stenosis and nodularity, the concrete diagnosis of which currently requires the finding of apple-green birefringence from endobronchial biopsies. Bronchoscopic probe-based confocal endomicroscopy (pCLE) is a novel optical biopsy technique which provides real-time images of the lattice structure of the bronchial basement membrane - a finding lost in malignancy. This case study outlines the imperfect, essentially palliative management of this rare disease, and shows for the first time the unusual dappled in vivo pCLE images of amyloid-affected endobronchium.

  4. Immunoglobulin synthesis in primary and myeloma amyloidosis.

    PubMed Central

    Preud'homme, J L; Ganeval, D; Grünfeld, J P; Striker, L; Brouet, J C

    1988-01-01

    Bone marrow cells from 14 patients with primary amyloidosis and two patients with myeloma amyloidosis were studied by immunofluorescence and biosynthesis experiments after incorporation of radioactive amino acids. Cells from four patients affected with non-myeloma secondary amyloidosis were also studied as controls. In primary amyloidosis, monoclonal plasma cell populations were demonstrated by immunofluorescence in virtually every case, even in patients without serum and urine monoclonal immunoglobulin and with a normal percentage of bone marrow plasma cells. Biosynthesis experiments showed the secretion of large amounts of free light chains, most often of the lambda type, in every primary or myeloma amyloidosis case and the presence of light chain fragments in almost all cases. Special features in certain patients were the synthesis of short gamma chains (two cases), assembly block at the HL half molecule level of a monoclonal IgA (one case) and secretion of decameric abnormally large kappa chains (one case). This is in contrast with non-myelomatous secondary amyloidosis where the distribution of bone marrow plasma cells was normal by immunofluorescence and where normal sized immunoglobulins were synthesized, without free light chain secretion and fragments. These data confirm that primary amyloidosis belongs to plasma cell dyscrasias and emphasize the role of free light chains and light chain fragments in the pathogenesis of amyloid deposition. PMID:3145161

  5. Magnetic resonance imaging in cardiac amyloidosis

    SciTech Connect

    O'Donnell, J.K.; Go, R.T.; Bott-Silverman, C.; Feiglin, D.H.; Salcedo, E.; MacIntyre, W.J.

    1984-01-01

    Primary amyloidosis (AL) involves the myocardium in 90% of cases and may present as apparent ischemia, vascular disease, or congestive heart failure. Two-dimensional echocardiography (echo) has proven useful in the diagnosis, particularly in differentiating AL from constrictive pericarditis. The findings of thickened RV and LV myocardium, normal LV cavity dimension, and a diffuse hyperrefractile ''granular sparkling'' appearance are virtually diagnostic. Magnetic resonance (MR) imaging may improve the resolution of anatomic changes seen in cardiac AL and has the potential to provide more specific information based on biochemical tissue alterations. In this preliminary study, the authors obtained both MR and echo images in six patients with AL and biopsy-proven myocardial involvement. 5/6 patients also had Tc-99 PYP myocardial studies including emission tomography (SPECT). MR studies utilized a 0.6 Tesla superconductive magnet. End diastolic gated images were obtained with TE=30msec and TR=R-R interval on the ECG. 6/6 pts. showed LV wall thickening which was concentric and included the septum. Papillary muscles were identified in all and were enlarged in 3/6. 4/6 pts. showed RV wall thickening but to a lesser degree than LV. Pericardial effusions were present in 4 cases. These findings correlated well with the results of echo although MR gave better RV free wall resolution. PYP scans were positive in 3 pts. but there was no correlation with degree of LV thickening. The authors conclude that there are no identifiable MR findings in patients with cardiac AL which encourage further attempts to characterize myocardial involvement by measurement of MR relaxation times in vivo.

  6. Pneumatosis intestinalis due to gastrointestinal amyloidosis: A case report & review of literature

    PubMed Central

    Khalid, Filza; Kaiyasah, Hadiel; Binfadil, Wafa; Majid, Maiyasa; Hazim, Wessam; ElTayeb, Yousif

    2016-01-01

    Introduction Pneumatosis intestinalis (PI) is not a disease but a radiological finding with a poorly understood pathogenesis. It can be divided into primary/idiopathic (15%) or secondary (85%) Kim et al. 2007, based on the factors thought to play a role in its development. Amongst the rare causes of secondary PI is gastrointestinal (GI) amyloidosis. Presentation of the case We report a case of a 46-year-old gentleman who presented with a one month history of acute on chronic abdominal pain, associated with one episode of melena. Upon further investigation, he was found to have pneumoperitoneum. He was taken to the operating theatre, where he was noted to have features of pneumatosis intestinalis of the small bowel with no evidence of bowel perforation. Postoperatively, he underwent an upper GI endoscopy with biopsies that revealed GI amyloidosis. Discussion One of the rare causes that can lead to secondary PI is GI amyloidosis as proven in our case. Patients with symptomatic gastrointestinal amyloidosis usually present with one of four syndromes: gastrointestinal bleeding, malabsorption, protein-losing gastroenteropathy, and, less often, gastrointestinal dysmotility. Conclusion GI amyloidosis is a rare cause of secondary pneumatosis intestinalis. The presentation of the disease varies from patient to patient, therefore, the management should be tailored accordingly. PMID:27085104

  7. AA-negative and Kappa-positive Amyloidosis in a Patient with Rheumatoid Arthritis.

    PubMed

    Ueno, Toshiharu; Sumida, Keiichi; Hoshino, Junichi; Suwabe, Tatsuya; Mise, Koki; Hazue, Ryo; Hayami, Noriko; Hiramatsu, Rikako; Kawada, Masahiro; Imafuku, Aya; Hasegawa, Eiko; Sawa, Naoki; Takaichi, Kenmei; Kinowaki, Keiichi; Ohashi, Kenichi; Fujii, Takeshi; Nishida, Aya; Ubara, Yoshifumi

    2016-01-01

    A 57-year-old Japanese woman with a 5-year history of rheumatoid arthritis (RA) was admitted to our hospital for an evaluation of nephrotic range proteinuria (4.8 g/day). A renal biopsy led to the diagnosis of amyloidosis according to strong positivity for Congo red staining and the detection of microfibrillar structures on electron microscopy that were negative for AA and positive for kappa light chain. Combination therapy with high-dose melphalan and autologous stem cell transplantation was performed according to the regimen for AL amyloidosis. Her proteinuria and RA subsided, but relapsed after 3 years. This is the first report regarding kappa light chain amyloidosis in an RA patient. PMID:27580556

  8. Amyloidosis: an unusual cause of portal hypertension.

    PubMed

    Takayasu, Vilma; Laborda, Lorena Silva; Bernardelli, Raquel; Pinesi, Henrique Trombini; Silva, Marilia Polo Minguete E; Chiavelli, Viviane; Simões, Angélica Braz; Felipe-Silva, Aloisio

    2016-01-01

    Amyloidosis comprises a group of diseases that occurs in five to nine cases per million patients per year worldwide irrespective of its classification. Although the hepatic involvement in primary amyloidosis is frequent, the clinical manifestations of liver amyloidosis are mild or even absent. The authors report the case of an aged man who complained of diffuse abdominal pain and marked weight loss and presented clinical signs of hepatopathy. Clinical workup revealed portal hypertension with ascites, hemorrhoids, and esophageal varices. The laboratory tests showed the cholestatic pattern of liver enzymes, hyperbilirubinemia, renal insufficiency and massive proteinuria accompanied by the presence of serum pike of monoclonal lambda light chain protein. The outcome was unfavorable, and the patient died. The autopsy findings revealed the diagnosis of amyloidosis predominantly involving the liver and kidneys. The bone marrow examination demonstrated the deposition of amyloid material associated with clonal plasma cells infiltration. The authors call attention to portal hypertension as a rare manifestation of primary amyloidosis. Meanwhile, this diagnosis should be taken into account whenever the hepatopathy is accompanied by laboratory abnormalities consistent with hepatic space-occupying lesions concomitantly with other organs involvement. In the case reported herein, kidney involvement was also present with renal failure, massive proteinuria with monoclonal serum gammopathy, what reinforced the diagnostic possibility of primary amyloidosis. PMID:27547738

  9. Amyloidosis: an unusual cause of portal hypertension

    PubMed Central

    Laborda, Lorena Silva; Bernardelli, Raquel; Pinesi, Henrique Trombini; Silva, Marilia Polo Minguete e; Chiavelli, Viviane; Simões, Angélica Braz; Felipe-Silva, Aloisio

    2016-01-01

    Amyloidosis comprises a group of diseases that occurs in five to nine cases per million patients per year worldwide irrespective of its classification. Although the hepatic involvement in primary amyloidosis is frequent, the clinical manifestations of liver amyloidosis are mild or even absent. The authors report the case of an aged man who complained of diffuse abdominal pain and marked weight loss and presented clinical signs of hepatopathy. Clinical workup revealed portal hypertension with ascites, hemorrhoids, and esophageal varices. The laboratory tests showed the cholestatic pattern of liver enzymes, hyperbilirubinemia, renal insufficiency and massive proteinuria accompanied by the presence of serum pike of monoclonal lambda light chain protein. The outcome was unfavorable, and the patient died. The autopsy findings revealed the diagnosis of amyloidosis predominantly involving the liver and kidneys. The bone marrow examination demonstrated the deposition of amyloid material associated with clonal plasma cells infiltration. The authors call attention to portal hypertension as a rare manifestation of primary amyloidosis. Meanwhile, this diagnosis should be taken into account whenever the hepatopathy is accompanied by laboratory abnormalities consistent with hepatic space-occupying lesions concomitantly with other organs involvement. In the case reported herein, kidney involvement was also present with renal failure, massive proteinuria with monoclonal serum gammopathy, what reinforced the diagnostic possibility of primary amyloidosis. PMID:27547738

  10. In situ characterization of protein aggregates in human tissues affected by light chain amyloidosis: a FTIR microspectroscopy study

    PubMed Central

    Ami, Diletta; Lavatelli, Francesca; Rognoni, Paola; Palladini, Giovanni; Raimondi, Sara; Giorgetti, Sofia; Monti, Luca; Doglia, Silvia Maria; Natalello, Antonino; Merlini, Giampaolo

    2016-01-01

    Light chain (AL) amyloidosis, caused by deposition of amyloidogenic immunoglobulin light chains (LCs), is the most common systemic form in industrialized countries. Still open questions, and premises for developing targeted therapies, concern the mechanisms of amyloid formation in vivo and the bases of organ targeting and dysfunction. Investigating amyloid material in its natural environment is crucial to obtain new insights on the molecular features of fibrillar deposits at individual level. To this aim, we used Fourier transform infrared (FTIR) microspectroscopy for studying in situ unfixed tissues (heart and subcutaneous abdominal fat) from patients affected by AL amyloidosis. We compared the infrared response of affected tissues with that of ex vivo and in vitro fibrils obtained from the pathogenic LC derived from one patient, as well as with that of non amyloid-affected tissues. We demonstrated that the IR marker band of intermolecular β-sheets, typical of protein aggregates, can be detected in situ in LC amyloid-affected tissues, and that FTIR microspectroscopy allows exploring the inter- and intra-sample heterogeneity. We extended the infrared analysis to the characterization of other biomolecules embedded within the amyloid deposits, finding an IR pattern that discloses a possible role of lipids, collagen and glycosaminoglycans in amyloid deposition in vivo. PMID:27373200

  11. In situ characterization of protein aggregates in human tissues affected by light chain amyloidosis: a FTIR microspectroscopy study.

    PubMed

    Ami, Diletta; Lavatelli, Francesca; Rognoni, Paola; Palladini, Giovanni; Raimondi, Sara; Giorgetti, Sofia; Monti, Luca; Doglia, Silvia Maria; Natalello, Antonino; Merlini, Giampaolo

    2016-01-01

    Light chain (AL) amyloidosis, caused by deposition of amyloidogenic immunoglobulin light chains (LCs), is the most common systemic form in industrialized countries. Still open questions, and premises for developing targeted therapies, concern the mechanisms of amyloid formation in vivo and the bases of organ targeting and dysfunction. Investigating amyloid material in its natural environment is crucial to obtain new insights on the molecular features of fibrillar deposits at individual level. To this aim, we used Fourier transform infrared (FTIR) microspectroscopy for studying in situ unfixed tissues (heart and subcutaneous abdominal fat) from patients affected by AL amyloidosis. We compared the infrared response of affected tissues with that of ex vivo and in vitro fibrils obtained from the pathogenic LC derived from one patient, as well as with that of non amyloid-affected tissues. We demonstrated that the IR marker band of intermolecular β-sheets, typical of protein aggregates, can be detected in situ in LC amyloid-affected tissues, and that FTIR microspectroscopy allows exploring the inter- and intra-sample heterogeneity. We extended the infrared analysis to the characterization of other biomolecules embedded within the amyloid deposits, finding an IR pattern that discloses a possible role of lipids, collagen and glycosaminoglycans in amyloid deposition in vivo. PMID:27373200

  12. [Inconsistency between voltage of the electrocardiogram and the left ventricular wall thickness. diagnostic key in cardiac amyloidosis].

    PubMed

    Contreras, A; Beacon, E; Brenna, Eduardo J; Parisi, Gustavo R; Chamale, Roberto A; Gilardi, F; Bürguesser, M V; Salomone, O

    2013-01-01

    Restrictive cardiomyopathy is the least common form of cardiomyopathy, and the disease that most often cause it, is the system amyloidosis. We present a 62-year-old with a history of heart failure, which in its assessment highlights the discrepancy between the low voltage ventricular complexes in the electrocardiogram and the severity of left ventricular wall thickness on echocardiography. This discrepancy was the source of suspicion and subsequent confirmation of systemic amyloidosis with cardiac involvement.

  13. Wild-Type Transthyretin Cardiac Amyloidosis: Novel Insights From Advanced Imaging.

    PubMed

    Narotsky, David L; Castano, Adam; Weinsaft, Jonathan W; Bokhari, Sabahat; Maurer, Mathew S

    2016-09-01

    Amyloidosis is caused by extracellular deposition of abnormal protein fibrils, resulting in destruction of tissue architecture and impairment of organ function. The most common forms of systemic amyloidosis are light-chain and transthyretin-related (ATTR). ATTR can result from an autosomal dominant hereditary transmission of mutated genes in the transthyretin or from a wild-type form of disease (ATTRwt), previously known as senile cardiac amyloidosis. With the aging of the worldwide population, ATTRwt will emerge as the most common type of cardiac amyloidosis that clinicians encounter. Diagnosis of systemic amyloidosis is often delayed, either because of the false assumption that it is a rare disease, or because of misdiagnosis as a result of mistaking it with other conditions. Clinicians must integrate clinical clues from history, physical examination, and common diagnostic tests to raise suspicion for ATTRwt. The historical gold standard for diagnosis of cardiac amyloid is endomyocardial biopsy analysis with pathological distinction of precursor protein type, but this method often results in delayed diagnosis because of the limited availability of expertise to perform and interpret the endomyocardial biopsy specimen. Emerging noninvasive imaging modalities provide easier, accurate screening for ATTRwt. These modalities include advanced echocardiography, using strain imaging and the myocardial contraction fraction; nuclear scintigraphy, which can differentiate between ATTR and light-chain cardiac amyloid; and cardiac magnetic resonance imaging, using extracellular volume measurement, late gadolinium enhancement, and distinct T1 mapping. These novel approaches reveal insights into the prevalence, clinical course, morphological effects, and prognosis of ATTRwt. PMID:27568874

  14. Increased Serum Free Light Chains Precede the Presentation of Immunoglobulin Light Chain Amyloidosis

    PubMed Central

    Weiss, Brendan M.; Hebreo, Joseph; Cordaro, Daniel V.; Roschewski, Mark J.; Baker, Thomas P.; Abbott, Kevin C.; Olson, Stephen W.

    2014-01-01

    Purpose Patients with immunoglobulin light chain amyloidosis (AL amyloidosis) generally present with advanced organ dysfunction and have a high risk of early death. We sought to characterize monoclonal immunoglobulin (M-Ig) light chains before clinical presentation of AL amyloidosis. Patients and Methods We obtained prediagnostic sera from 20 cases with AL amyloidosis and 20 healthy controls matched for age, sex, race, and age of serum sample from the Department of Defense Serum Repository. Serum protein electrophoresis with immunofixation and serum free light chain (FLC) analysis were performed on all samples. Results An M-Ig was detected in 100% of cases and 0% of controls (P < .001). The M-Ig was present in 100%, 80%, and 42% of cases at less than 4 years, 4 to 11 years, and more than 11 years before diagnosis, respectively. The median FLC differential (FLC-diff) was higher in cases compared with controls at all time periods, less than 4 years (174.8 v 0.3 mg/L; P < .001), 4 to 11 years (65.1 v 2.2 mg/L; P < .001), and more than 11 years (4.5 v 0.4 mg/L; P = .03) before diagnosis. The FLC-diff was greater than 23 mg/L in 85% of cases and 0% of controls (P < .001). The FLC-diff level increased more than 10% per year in 84% of cases compared with 16% of controls (P < .001). Conclusion Increase of FLCs, including within the accepted normal range, precedes the development of AL amyloidosis for many years. PMID:25024082

  15. Macular Amyloidosis and Epstein-Barr Virus

    PubMed Central

    Nahidi, Yalda; Tayyebi Meibodi, Naser; Meshkat, Zahra; Nazeri, Narges

    2016-01-01

    Background. Amyloidosis is extracellular precipitation of eosinophilic hyaline material of self-origin with special staining features and fibrillar ultrastructure. Macular amyloidosis is limited to the skin, and several factors have been proposed for its pathogenesis. Detection of Epstein-Barr virus (EBV) DNA in this lesion suggests that this virus can play a role in pathogenesis of this disease. Objective. EBV DNA detection was done on 30 skin samples with a diagnosis of macular amyloidosis and 31 healthy skin samples in the margin of removed melanocytic nevi by using PCR. Results. In patients positive for beta-globin gene in PCR, BLLF1 gene of EBV virus was positive in 23 patients (8 patients in case and 15 patients in the control group). There was no significant difference in presence of EBV DNA between macular amyloidosis (3.8%) and control (23.8%) groups (P = 0.08). Conclusion. The findings of this study showed that EBV is not involved in pathogenesis of macular amyloidosis. PMID:26981113

  16. Systems Engineering Techniques for ALS Decision Making

    NASA Technical Reports Server (NTRS)

    Rodriquez, Luis F.; Drysdale, Alan E.; Jones, Harry; Levri, Julie A.

    2004-01-01

    The Advanced Life Support (ALS) Metric is the predominant tool for predicting the cost of ALS systems. Metric goals for the ALS Program are daunting, requiring a threefold increase in the ALS Metric by 2010. Confounding the problem, the rate new ALS technologies reach the maturity required for consideration in the ALS Metric and the rate at which new configurations are developed is slow, limiting the search space and potentially giving the perspective of a ALS technology, the ALS Metric may remain elusive. This paper is a sequel to a paper published in the proceedings of the 2003 ICES conference entitled, "Managing to the metric: an approach to optimizing life support costs." The conclusions of that paper state that the largest contributors to the ALS Metric should be targeted by ALS researchers and management for maximum metric reductions. Certainly, these areas potentially offer large potential benefits to future ALS missions; however, the ALS Metric is not the only decision-making tool available to the community. To facilitate decision-making within the ALS community a combination of metrics should be utilized, such as the Equivalent System Mass (ESM)-based ALS metric, but also those available through techniques such as life cycle costing and faithful consideration of the sensitivity of the assumed models and data. Often a lack of data is cited as the reason why these techniques are not considered for utilization. An existing database development effort within the ALS community, known as OPIS, may provide the opportunity to collect the necessary information to enable the proposed systems analyses. A review of these additional analysis techniques is provided, focusing on the data necessary to enable these. The discussion is concluded by proposing how the data may be utilized by analysts in the future.

  17. Tracheobronchial Amyloidosis Mimicking Tracheal Tumor

    PubMed Central

    Özgül, Mehmet Akif; Uzun, Oğuz; Yaşar, Zehra; Acat, Murat; Arda, Naciye; Çetinkaya, Erdoğan

    2016-01-01

    Tracheobronchial amyloidosis is a rare presentation and accounts for about 1% of benign tumors in this area. The diagnosis of disease is delayed due to nonspecific pulmonary symptoms. Therapeutic approaches are required to control progressive pulmonary symptoms for most of the patients. Herein, we report a case of a 68-year-old man admitted with progressive dyspnea to our institution for further evaluation and management. He was initially diagnosed with and underwent management for bronchial asthma for two years but had persistent symptoms despite optimal medical therapy. Pulmonary computed tomography scan revealed severe endotracheal stenosis. Bronchoscopy was performed and showed endotracheal mass obstructing 70% of the distal trachea and mimicking a neoplastic lesion. The mass was successfully resected by mechanical resection, argon plasma coagulation (APC), and Nd-YAG laser during rigid bronchoscopy. Biopsy materials showed deposits of amorphous material by hematoxylin and eosin staining and these deposits were selectively stained with Congo Red. Although this is a rare clinical condition, this case indicated that carrying out a bronchoscopy in any patient complaining of atypical bronchial symptoms or with uncontrolled asthma is very important.

  18. Tracheobronchial Amyloidosis Mimicking Tracheal Tumor

    PubMed Central

    Özgül, Mehmet Akif; Uzun, Oğuz; Yaşar, Zehra; Acat, Murat; Arda, Naciye; Çetinkaya, Erdoğan

    2016-01-01

    Tracheobronchial amyloidosis is a rare presentation and accounts for about 1% of benign tumors in this area. The diagnosis of disease is delayed due to nonspecific pulmonary symptoms. Therapeutic approaches are required to control progressive pulmonary symptoms for most of the patients. Herein, we report a case of a 68-year-old man admitted with progressive dyspnea to our institution for further evaluation and management. He was initially diagnosed with and underwent management for bronchial asthma for two years but had persistent symptoms despite optimal medical therapy. Pulmonary computed tomography scan revealed severe endotracheal stenosis. Bronchoscopy was performed and showed endotracheal mass obstructing 70% of the distal trachea and mimicking a neoplastic lesion. The mass was successfully resected by mechanical resection, argon plasma coagulation (APC), and Nd-YAG laser during rigid bronchoscopy. Biopsy materials showed deposits of amorphous material by hematoxylin and eosin staining and these deposits were selectively stained with Congo Red. Although this is a rare clinical condition, this case indicated that carrying out a bronchoscopy in any patient complaining of atypical bronchial symptoms or with uncontrolled asthma is very important. PMID:27594885

  19. Tracheobronchial Amyloidosis Mimicking Tracheal Tumor.

    PubMed

    Tanrıverdi, Elif; Özgül, Mehmet Akif; Uzun, Oğuz; Gül, Şule; Çörtük, Mustafa; Yaşar, Zehra; Acat, Murat; Arda, Naciye; Çetinkaya, Erdoğan

    2016-01-01

    Tracheobronchial amyloidosis is a rare presentation and accounts for about 1% of benign tumors in this area. The diagnosis of disease is delayed due to nonspecific pulmonary symptoms. Therapeutic approaches are required to control progressive pulmonary symptoms for most of the patients. Herein, we report a case of a 68-year-old man admitted with progressive dyspnea to our institution for further evaluation and management. He was initially diagnosed with and underwent management for bronchial asthma for two years but had persistent symptoms despite optimal medical therapy. Pulmonary computed tomography scan revealed severe endotracheal stenosis. Bronchoscopy was performed and showed endotracheal mass obstructing 70% of the distal trachea and mimicking a neoplastic lesion. The mass was successfully resected by mechanical resection, argon plasma coagulation (APC), and Nd-YAG laser during rigid bronchoscopy. Biopsy materials showed deposits of amorphous material by hematoxylin and eosin staining and these deposits were selectively stained with Congo Red. Although this is a rare clinical condition, this case indicated that carrying out a bronchoscopy in any patient complaining of atypical bronchial symptoms or with uncontrolled asthma is very important. PMID:27594885

  20. Transthyretin Cardiac Amyloidosis in Black Americans.

    PubMed

    Shah, Keyur B; Mankad, Anit K; Castano, Adam; Akinboboye, Olakunle O; Duncan, Phillip B; Fergus, Icilma V; Maurer, Mathew S

    2016-06-01

    Transthyretin-related cardiac amyloidosis is a progressive infiltrative cardiomyopathy that mimics hypertensive and hypertrophic heart disease and often goes undiagnosed. In the United States, the hereditary form disproportionately afflicts black Americans, who when compared with whites with wild-type transthyretin amyloidosis, a phenotypically similar condition, present with more advanced disease despite having a noninvasive method for early identification (genetic testing). Although reasons for this are unclear, this begs to consider the inadequate access to care, societal factors, or a biological basis. In an effort to improve awareness and explore unique characteristics, we review the pathophysiology, epidemiology, and therapeutic strategies for transthyretin amyloidosis and highlight diagnostic pitfalls and clinical pearls for identifying patients with amyloid heart disease.

  1. Transthyretin Cardiac Amyloidosis in Black Americans.

    PubMed

    Shah, Keyur B; Mankad, Anit K; Castano, Adam; Akinboboye, Olakunle O; Duncan, Phillip B; Fergus, Icilma V; Maurer, Mathew S

    2016-06-01

    Transthyretin-related cardiac amyloidosis is a progressive infiltrative cardiomyopathy that mimics hypertensive and hypertrophic heart disease and often goes undiagnosed. In the United States, the hereditary form disproportionately afflicts black Americans, who when compared with whites with wild-type transthyretin amyloidosis, a phenotypically similar condition, present with more advanced disease despite having a noninvasive method for early identification (genetic testing). Although reasons for this are unclear, this begs to consider the inadequate access to care, societal factors, or a biological basis. In an effort to improve awareness and explore unique characteristics, we review the pathophysiology, epidemiology, and therapeutic strategies for transthyretin amyloidosis and highlight diagnostic pitfalls and clinical pearls for identifying patients with amyloid heart disease. PMID:27188913

  2. Surgical management of advanced ocular adnexal amyloidosis.

    PubMed

    Patrinely, J R; Koch, D D

    1992-06-01

    Ocular adnexal amyloidosis is characterized by amyloid deposition within the deep connective tissue layers of the eyelids, conjunctiva, and anterior orbit. Management of advanced cases has traditionally been unsatisfactory, with either no surgery offered because of fear of hemorrhage or an en bloc resection performed of the entire involved area. We present two cases of advanced periorbital amyloidosis successfully managed by preserving the anatomic planes of the eyelids and meticulously debulking the deposits with a spooned curette. Lax eyelid tendons and aponeuroses were simultaneously repaired, and no sacrifice of eyelid tissues was necessary. One patient remained asymptomatic for 2 years after surgery before developing early reaccumulation in the lower eyelids. The other patient required additional eyelid debulking and ptosis revision 8 months after surgery, but was in stable condition at follow-up 2 years after surgery. This technique offers safe, easily repeatable, nondestructive treatment for advanced periocular amyloidosis.

  3. Primary localized amyloidosis presenting as diffuse amorphous calcified mass in both orbits: case report.

    PubMed

    Gonçalves, Allan Christian Pieroni; Moritz, Rodrigo Bernal da Costa; Monteiro, Mário Luiz Ribeiro

    2011-01-01

    Primary localized amyloidosis is rare in the orbit. We report the case of a 63-year-old woman that presented with bilateral proptosis and ophthalmoplegia. A computed tomography scan revealed an infiltrative amorphous and markedly calcified mass in both orbits while a magnetic resonance scan showed a heterogeneous hypointense signal on T2-weighted images. A biopsy was performed through an anterior orbitotomy. Microscopy revealed extracellular amorphous and eosinophilic hyaline material which stained pink with Congo red and displayed green birefringence on polarized microscopy, leading to a diagnosis of amyloidosis. The results of the systemic workup were completely normal. A two-year follow-up period without any treatment disclosed no worsening of the condition. While calcification of nonvascular orbital lesions has often been regarded as suggestive of malignant disease, our case is a reminder that it can also be a characteristic presenting sign of orbital amyloidosis.

  4. Topical application of ankaferd hemostat in a patient with gastroduodenal amyloidosis complicated with gastrointestinal bleeding.

    PubMed

    Beyazit, Yavuz; Onder, Fatih Oguz; Torun, Serkan; Tas, Adnan; Purnak, Tugrul; Tenlik, Ilyas; Turhan, Nesrin

    2013-10-01

    Amyloidosis rarely manifests itself as gastrointestinal hemorrhage, especially in the absence of systemic involvement. Despite urgent endoscopic and/or pharmacological therapy, bleeding due to gastric amyloidosis usually recurs after a short period and has considerable morbidity and mortality rates, even in patients undergoing gastrointestinal surgery. For this reason, there is a need for a therapeutic armamentarium for such cases that is effective, easily applicable and has minimal side effects. In this respect, ankaferd blood stopper (ABS) offers a well tolerated and effective alternative approach for these patients. Herein we would like to report a 77-year-old man who had massive bleeding from a gastric ulcer complicating primary gastroduodenal amyloidosis, in whom topical ABS was successfully applied. PMID:23751610

  5. Spontaneous pancreatic islet amyloidosis in 40 baboons.

    PubMed

    Hubbard, G B; Steele, K E; Davis, K J; Leland, M M

    2002-04-01

    Spontaneous amyloidosis occurs in many nonhuman primate species but remains difficult to diagnose and treat. Nonhuman primates continue to offer promise as animal models in which to study amyloidosis in humans. Amyloidosis was not diagnosed clinically but was found histologically in four male and 36 female baboons. The baboons averaged 18 years of age at death (range, 7-28 years). Clinical signs, if present, were hyperglycemia and cachexia. Blood glucose values were elevated in 12 of 30 baboons with available clinical pathology data. Four baboons had been clinically diagnosed as diabetic and three were treated with insulin. Amyloid was found in the islets of Langerhans of the pancreas in 40 baboons; 35 baboons had amyloid only in the islets of Langerhans. Amyloid was found in nonislet tissue of baboons as follows: five, nonislet pancreas; four, intestine and adrenal; three, kidney; two, prostate and spleen; and one each, lymph node, liver, gall bladder, stomach, tongue, urinary bladder, and salivary gland. Sections of paraffin-embedded tissues were evaluated for amyloid with hematoxylin and eosin (HE) and congo red (CR) staining, and using immunohistochemistry for human islet amyloid polypeptide (IAPP), calcitonin gene-related peptide (CGRP), glucagon, pancreatic polypeptide (PP), somatostatin (SS), and porcine insulin. Islet amyloid was positive with HE in 40 baboons, with CR in 39 baboons, and with IAPP and CGRP in 35 baboons. IAPP and CGRP only stained islet amyloid. PP, SS, glucagon, and porcine insulin did not stain amyloid. Islet amyloidosis in the baboon appears to be difficult to diagnose clinically, age-related, and similar to islet amyloidosis in other species. The baboon may be a good model for the study of islet amyloidosis in humans.

  6. Renal amyloidosis in leprosy, an infrequent cause of nephrotic syndrome in Europe.

    PubMed

    Sanz-Martín, Noelia; Samillán-Sosa, Kelly Del Rocío; De Miguel, Julio; Martínez-Miguel, Patricia

    2016-01-01

    Leprosy is a chronic infectious disease caused by Mycobacterium leprae The main clinical manifestations involve the skin and the peripheral nervous system. Several types of nephropathy have been described in leprosy. One frequent form of renal involvement is amyloidosis, especially in patients with lepromatous leprosy. In these patients, end-stage renal disease is an important contributor to morbidity and mortality. Here, we present the case of a patient with nephrotic syndrome caused by secondary amyloidosis, chronic peripheral neuropathy and a history of leprosy. The patient was correctly treated in her youth, which is the best way to avoid renal pathology, but she developed a nephrotic syndrome years later. PMID:27489069

  7. Growth Hormone and Cerebral Amyloidosis.

    PubMed

    Benvenga, S; Guarneri, F

    2016-08-01

    Great interest has recently been focused on a paper reporting characteristic deposits of amyloid-β protein associated with Alzheimer's disease in brains of adults who died of Creutzfeldt-Jakob disease. As they had contracted such disease after treatment with prion-contaminated human growth hormone extracted from cadaver-derived pituitaries, the authors have suggested that interhuman transmission of Alzheimer's disease had occurred. Our previous research led us to find that amyloid-forming peptides share amino acid sequence homology, summarized by a motif. Here, we probed the amino acid sequence of human growth hormone for such a motif, and found that 2 segments fit the motif and are potentially amyloid-forming. This finding was confirmed by Aggrescan, another well-known software for the prediction of amyloidogenic peptides. Our results, taken together with data from the literature that are missing in the aforementioned paper and associated commentaries, minimize the contagious nature of the iatrogenically-acquired coexistence of Creutzfeldt-Jakob disease and Alzheimer's disease. In particular, the above mentioned paper misses literature data on intratumoral amyloidosis in growth hormone- and prolactin-secreting adenomas, tumors relatively frequent in adults, which are often silent. It cannot be excluded that some pituitaries used to extract growth hormone contained clinically silent microadenomas, a fraction of which containing amyloid deposits, and patients might had received a fraction of growth hormone (with or without prolactin) that already was an amyloid seed. The intrinsic amyloidogenicity of growth hormone, in the presence of contaminating prion protein (and perhaps prolactin as well) and amyloid-β contained in some cadavers' pituitaries, may have led to the observed co-occurring of Creutzfeldt-Jakob disease and Alzheimer's disease. PMID:27214308

  8. Study on epidemiology of cutaneous amyloidosis in northern India and effectiveness of dimethylsulphoxide in cutaneous amyloidosis

    PubMed Central

    Krishna, Arvind; Nath, Bhola; Dhir, G. G.; Kumari, Ranjeeta; Budhiraja, Virendra; Singh, Kalpana

    2012-01-01

    Context: Amyloidosis, which is characterized by the extracellular deposition of a proteinaceous substance, is usually associated with considerable tissue dysfunction. However, the etiology of the disease remains uncertain and the treatment disappointing. Aim: 1. To know the epidemiology of cutaneous amyloidosis 2. To evaluate the effect of dimethylsulphoxide on cutaneous amyloidosis. Settings and Design: Data was collected from patients attending the Outpatient Department (OPD) over a period of one year. Material and Methods: Patients were screened on the basis of signs and symptoms and then confirmed histologically. A total of 62 patients who were suspected to be suffering from amyloidosis on the basis of clinical signs and symptoms and 38 patients who were further confirmed histopathologically underwent the treatment. Statistical Analysis Used: Chi-square test was used for testing the significance of proportions. Results: 63.15 percent of the patients had macular amyloidosis and the interscapular area was the most common area involved (52.63%). Pruritus, pigmentation, and papules responded excellently to dimethylsulphoxide after one month of treatment. Conclusions: Cutaneous amyloidosis is a disease found in middle-aged persons, with a female preponderance, and dimethylsulphoxide seems to be an effective therapy. PMID:23189250

  9. Primary diffuse tracheo-bronchial amyloidosis

    PubMed Central

    Antunes, Maria L.; Da Luz, J. M. Vieira

    1969-01-01

    A case of diffuse tracheo-bronchial amyloidosis in a 62-year-old woman thought to have chronic bronchitis is reported. The immunological mechanism in the pathogenesis of this condition is considered and the need for bronchological investigation in chronic bronchial disorders is stressed. Images PMID:5810372

  10. Long-term follow-up after surgery in localized laryngeal amyloidosis.

    PubMed

    Hazenberg, Aldert J C; Hazenberg, Bouke P C; Dikkers, Frederik G

    2016-09-01

    To study effectiveness of surgery and watchful waiting in localized laryngeal amyloidosis, retrospective case series. This retrospective study comprises all consecutive patients with localized laryngeal amyloidosis surgically treated in a tertiary hospital between 1994 and February 2016. Recurrence rate, revision surgery, progression to systemic amyloidosis, and changes in voice were monitored yearly. Eighteen patients were included. Seven women and eleven men had a median age 50 years (range 21-77 years) and median follow-up 6.4 years (2.4-17 years). Amyloid was located in subglottis (5), glottis (8), false vocal folds (8) and other supraglottic areas (5), in more than one laryngeal region (13) and bilaterally (12). Cold steel excision was used at the glottis; CO2 laser excision, sometimes assisted by microdebrider, at other laryngeal areas. Eleven patients needed revision surgery, ten within the first 4 years after surgical treatment. One patient needed his first revision surgery after 11 years. Five patients needed a second revision within 6 years after initial diagnosis. Two patients needed a third revision. Indications for first revision surgery were progression (8) with dysphonia (7), dyspnea (2), dysphagia (1), exclusion of malignancy (1), and aphonia (1). No patient developed systemic amyloidosis during follow-up. Although local progression of amyloid necessitates revision surgery once or twice in the first 4-6 years, progression slows down thereafter. Late progression, however, remains possible. PMID:27156084

  11. Preclinical evaluation of RNAi as a treatment for transthyretin-mediated amyloidosis.

    PubMed

    Butler, James S; Chan, Amy; Costelha, Susete; Fishman, Shannon; Willoughby, Jennifer L S; Borland, Todd D; Milstein, Stuart; Foster, Donald J; Gonçalves, Paula; Chen, Qingmin; Qin, June; Bettencourt, Brian R; Sah, Dinah W; Alvarez, Rene; Rajeev, Kallanthottathil G; Manoharan, Muthiah; Fitzgerald, Kevin; Meyers, Rachel E; Nochur, Saraswathy V; Saraiva, Maria J; Zimmermann, Tracy S

    2016-06-01

    ATTR amyloidosis is a systemic, debilitating and fatal disease caused by transthyretin (TTR) amyloid accumulation. RNA interference (RNAi) is a clinically validated technology that may be a promising approach to the treatment of ATTR amyloidosis. The vast majority of TTR, the soluble precursor of TTR amyloid, is expressed and synthesized in the liver. RNAi technology enables robust hepatic gene silencing, the goal of which would be to reduce systemic levels of TTR and mitigate many of the clinical manifestations of ATTR that arise from hepatic TTR expression. To test this hypothesis, TTR-targeting siRNAs were evaluated in a murine model of hereditary ATTR amyloidosis. RNAi-mediated silencing of hepatic TTR expression inhibited TTR deposition and facilitated regression of existing TTR deposits in pathologically relevant tissues. Further, the extent of deposit regression correlated with the level of RNAi-mediated knockdown. In comparison to the TTR stabilizer, tafamidis, RNAi-mediated TTR knockdown led to greater regression of TTR deposits across a broader range of affected tissues. Together, the data presented herein support the therapeutic hypothesis behind TTR lowering and highlight the potential of RNAi in the treatment of patients afflicted with ATTR amyloidosis. PMID:27033334

  12. Proteomic Analysis of Highly Prevalent Amyloid A Amyloidosis Endemic to Endangered Island Foxes

    PubMed Central

    Gaffney, Patricia M.; Imai, Denise M.; Clifford, Deana L.; Ghassemian, Majid; Sasik, Roman; Chang, Aaron N.; O’Brien, Timothy D.; Coppinger, Judith; Trejo, Margarita; Masliah, Eliezer; Munson, Linda; Sigurdson, Christina

    2014-01-01

    Amyloid A (AA) amyloidosis is a debilitating, often fatal, systemic amyloid disease associated with chronic inflammation and persistently elevated serum amyloid A (SAA). Elevated SAA is necessary but not sufficient to cause disease and the risk factors for AA amyloidosis remain poorly understood. Here we identify an extraordinarily high prevalence of AA amyloidosis (34%) in a genetically isolated population of island foxes (Urocyon littoralis) with concurrent chronic inflammatory diseases. Amyloid deposits were most common in kidney (76%), spleen (58%), oral cavity (45%), and vasculature (44%) and were composed of unbranching, 10 nm in diameter fibrils. Peptide sequencing by mass spectrometry revealed that SAA peptides were dominant in amyloid-laden kidney, together with high levels of apolipoprotein E, apolipoprotein A-IV, fibrinogen-α chain, and complement C3 and C4 (false discovery rate ≤0.05). Reassembled peptide sequences showed island fox SAA as an 111 amino acid protein, most similar to dog and artic fox, with 5 unique amino acid variants among carnivores. SAA peptides extended to the last two C-terminal amino acids in 5 of 9 samples, indicating that near full length SAA was often present in amyloid aggregates. These studies define a remarkably prevalent AA amyloidosis in island foxes with widespread systemic amyloid deposition, a unique SAA sequence, and the co-occurrence of AA with apolipoproteins. PMID:25429466

  13. Preclinical evaluation of RNAi as a treatment for transthyretin-mediated amyloidosis

    PubMed Central

    Butler, James S.; Chan, Amy; Costelha, Susete; Fishman, Shannon; Willoughby, Jennifer L. S.; Borland, Todd D.; Milstein, Stuart; Foster, Donald J.; Gonçalves, Paula; Chen, Qingmin; Qin, June; Bettencourt, Brian R.; Sah, Dinah W.; Alvarez, Rene; Rajeev, Kallanthottathil G.; Manoharan, Muthiah; Fitzgerald, Kevin; Meyers, Rachel E.; Nochur, Saraswathy V.; Saraiva, Maria J.; Zimmermann, Tracy S.

    2016-01-01

    Abstract ATTR amyloidosis is a systemic, debilitating and fatal disease caused by transthyretin (TTR) amyloid accumulation. RNA interference (RNAi) is a clinically validated technology that may be a promising approach to the treatment of ATTR amyloidosis. The vast majority of TTR, the soluble precursor of TTR amyloid, is expressed and synthesized in the liver. RNAi technology enables robust hepatic gene silencing, the goal of which would be to reduce systemic levels of TTR and mitigate many of the clinical manifestations of ATTR that arise from hepatic TTR expression. To test this hypothesis, TTR-targeting siRNAs were evaluated in a murine model of hereditary ATTR amyloidosis. RNAi-mediated silencing of hepatic TTR expression inhibited TTR deposition and facilitated regression of existing TTR deposits in pathologically relevant tissues. Further, the extent of deposit regression correlated with the level of RNAi-mediated knockdown. In comparison to the TTR stabilizer, tafamidis, RNAi-mediated TTR knockdown led to greater regression of TTR deposits across a broader range of affected tissues. Together, the data presented herein support the therapeutic hypothesis behind TTR lowering and highlight the potential of RNAi in the treatment of patients afflicted with ATTR amyloidosis. PMID:27033334

  14. Preclinical evaluation of RNAi as a treatment for transthyretin-mediated amyloidosis.

    PubMed

    Butler, James S; Chan, Amy; Costelha, Susete; Fishman, Shannon; Willoughby, Jennifer L S; Borland, Todd D; Milstein, Stuart; Foster, Donald J; Gonçalves, Paula; Chen, Qingmin; Qin, June; Bettencourt, Brian R; Sah, Dinah W; Alvarez, Rene; Rajeev, Kallanthottathil G; Manoharan, Muthiah; Fitzgerald, Kevin; Meyers, Rachel E; Nochur, Saraswathy V; Saraiva, Maria J; Zimmermann, Tracy S

    2016-06-01

    ATTR amyloidosis is a systemic, debilitating and fatal disease caused by transthyretin (TTR) amyloid accumulation. RNA interference (RNAi) is a clinically validated technology that may be a promising approach to the treatment of ATTR amyloidosis. The vast majority of TTR, the soluble precursor of TTR amyloid, is expressed and synthesized in the liver. RNAi technology enables robust hepatic gene silencing, the goal of which would be to reduce systemic levels of TTR and mitigate many of the clinical manifestations of ATTR that arise from hepatic TTR expression. To test this hypothesis, TTR-targeting siRNAs were evaluated in a murine model of hereditary ATTR amyloidosis. RNAi-mediated silencing of hepatic TTR expression inhibited TTR deposition and facilitated regression of existing TTR deposits in pathologically relevant tissues. Further, the extent of deposit regression correlated with the level of RNAi-mediated knockdown. In comparison to the TTR stabilizer, tafamidis, RNAi-mediated TTR knockdown led to greater regression of TTR deposits across a broader range of affected tissues. Together, the data presented herein support the therapeutic hypothesis behind TTR lowering and highlight the potential of RNAi in the treatment of patients afflicted with ATTR amyloidosis.

  15. Localized amyloidosis masquerading as nasopharyngeal tumor: a review.

    PubMed

    Panda, Naresh K; Saravanan, Karuppiah; Purushotaman, Gilbert Pragache; Gurunathan, Ramesh Kumar; Mahesha, Vankalakunti

    2007-01-01

    Amyloidosis comprises a diverse collection of disease characterized by the presence of amorphous extracellular eosinophilic deposits of unique protein fibrils that gives apple green birefringence under polarized light after staining with Congo red. Head and neck region is the commonest site for localized form of amyloidosis. We report a case of a 43-year-old man with localized amyloidosis of nasopharynx with oropharyngeal extension and its management, along with a review of relevant literatures.

  16. Poor Outcomes in Hepatic Amyloidosis: A Report of 2 Cases

    PubMed Central

    Kertowidjojo, Elizabeth; Zhang, Yue; Patel, Pruthvi

    2016-01-01

    Hepatic amyloidosis is a rare disease entity that results from insoluble amyloid protein deposition in the liver. The disease often presents with vague, nonspecific clinical features. Currently, there is little literature describing treatment outcomes for biopsy-proven hepatic amyloidosis and current treatment guidelines recommend that patients enroll in a clinical trial due to insufficient evidence to suggest an optimal treatment regimen. Here, we present two cases of hepatic amyloidosis at an academic medical center and describe their presentation, treatment, and outcomes. These cases highlight the poor outcomes and difficult management of hepatic amyloidosis. Further understanding and investigation of this rare disease are warranted. PMID:27774327

  17. Gastrointestinal Amyloidosis Presenting with Multiple Episodes of Gastrointestinal Bleeding

    SciTech Connect

    Kim, Sang Hyeon Kang, Eun Ju; Park, Jee Won; Jo, Jung Hyun; Kim, Soo Jin; Cho, Jin Han; Kang, Myong Jin; Park, Byeong Ho

    2009-05-15

    Amyloidosis is characterized by the extracellular deposition of amyloid protein in various organs. Gastrointestinal involvement in amyloidosis is common, but a diagnosis of amyloidosis is often delayed. Severe gastrointestinal hemorrhage in amyloidosis is rare but can be fatal in some cases. We experienced a case of a 49-year-old man who presented with recurrent massive hematochezia. Although embolization was performed eight times for bleeding from different sites of the small intestine, hematochezia did not cease. We report the case, with a review of the literature.

  18. A Rare Case of Ascites due to Peritoneal Amyloidosis

    PubMed Central

    Stofer, Fernanda; Barretto, Maria Fernanda; Gouvea, Ana Luisa; Ribeiro, Mario; Neves, Marcio; Gismondi, Ronaldo Altenburg; Mocarzel, Luís Otavio

    2016-01-01

    Patient: Male, 65 Final Diagnosis: Peritoneal amyloidosis Symptoms: Anasarca • Dyspnea • Orthopnea Medication: — Clinical Procedure: Paracentesis and peritoneal biopsy Specialty: Gastroenterology and Hepatology Objective: Unusual clinical course Background: The clinical manifestations of amyloidosis depend on the type of insoluble protein as well as the location of amyloid deposits in tissues or organs. In the gastrointestinal tract, the small intestine is the most common site of amyloid deposits, whereas peritoneal involvement and ascites are rare. Case Report: We report on a case of ascites due to peritoneal amyloidosis. A 65-year-old patient was admitted to our institution due to anasarca and pulmonary congestion, mimicking heart failure. We started the patient on diuretics and vasodilators. Despite improvement in pulmonary congestion and peripheral edema, his ascites was not reduced. Echocardiogram revealed restrictive cardiomyopathy and a speckle-tracking pattern suggestive of cardiac amyloidosis. Subcutaneous and peritoneal biopsies revealed amyloidosis. Conclusions: Amyloidosis is rare in the peritoneum and is usually asymptomatic. Ascites occurs in only 20% of patients with peritoneal amyloidosis. We searched PubMed using “ascites” and “amyloidosis” and identified only eight case reports of amyloidosis with ascites. Physicians should be particularly careful in heart failure and anasarca cases when ascites is disproportional or not responsive to diuretic treatment. To date, there is no specific treatment for peritoneal amyloidosis. PMID:27353538

  19. Renal AA Amyloidosis in Patients with Type 2 Diabetes Mellitus

    PubMed Central

    Díez, Ramón; Madero, Magdalena; Gamba, Gerardo; Soriano, Juan; Soto, Virgilia

    2014-01-01

    Background Type 2 diabetes mellitus (T2DM) is the leading cause of chronic kidney disease and a major cause of cardiovascular disease (CVD) mortality. Inflammation is closely involved in the pathogenesis of T2DM, and reactive amyloidosis occurs in the presence of chronic inflammation. We hypothesized that patients with T2DM may have a higher prevalence of renal AA amyloidosis (RAAA) and that this could contribute to worse atherosclerosis and CVD. Materials and Methods We analyzed 330 autopsy kidneys from patients with a previous T2DM diagnosis. The kidney tissue was evaluated in order to determine the presence of diabetic nephropathy and RAAA, and systemic vessels were evaluated for the presence of atherosclerosis. Results RAAA was detected in 9% of our study population and was associated with an increased risk for nodular sclerosis [OR (95% CI)] [11 (2.04-59.16)], for chronic ischemic cardiomyopathy [4.59 (2.02-10.42)], for myocardial infarction [3.41 (1.52-7.64)] as well as for aortic [4.75 (1.09-20.69)], coronary [3.22 (1.47-7.04)], and intrarenal atherosclerosis [3.84 (1.46-10.09)]. Conclusions RAAA is prevalent in T2DM and is associated with worse CVD and renal disease, likely because RAAA is a marker of severe chronic inflammation. PMID:25337080

  20. Online registry for mutations in hereditary amyloidosis including nomenclature recommendations.

    PubMed

    Rowczenio, Dorota M; Noor, Islam; Gillmore, Julian D; Lachmann, Helen J; Whelan, Carol; Hawkins, Philip N; Obici, Laura; Westermark, Per; Grateau, Gilles; Wechalekar, Ashutosh D

    2014-09-01

    Hereditary systemic amyloidosis comprises a group of rare monogenic diseases inherited in an autosomal dominant fashion. It is associated with mutations in genes encoding eight different proteins, including transthyretin, apolipoprotein AI, apolipoprotein AII, lysozyme, fibrinogen A α-chain, cystatin C, gelsolin and beta-2-microglobulin. With support from the EU FP6 EURAMY project we have designed an online registry of genes and mutations in hereditary amyloidosis including their associated clinical phenotypes, with a view to having a single free online portal for the collection and distribution of this information. Users can search the registry by either mutation, phenotype or authors who have published or submitted mutations. It provides a submission form for reporting newly identified mutations. We also wanted to introduce nomenclature which complies with recommendations set out by Human Genome Variation Society and HUGO Gene Nomenclature Committee for description of new and known genetic variants. We hope this registry would be a useful and convenient tool for the medical and scientific community. PMID:25044787

  1. Nodular pulmonary amyloidosis associated with asbestos exposure.

    PubMed

    Hiroshima, K; Ohwada, H; Ishibashi, M; Yamamoto, N; Tamiya, N; Yamaguchi, Y

    1996-01-01

    A 71 year old man was admitted for the purpose of diagnosis of a right solitary pulmonary nodule. The size of the nodule was 18 x 18 mm in diameter 2 years ago, but it has become large, 25 x 25 mm in diameter. The nodule was resected by thoracotomy. Microscopically, eosinophilic amorphous, acellular substances were surrounded by inflammatory infiltrates. It stained with Congo red stain and showed green birefringence with polarizing microscopy. Amyloid fibrils were observed electron microscopically. Asbestos bodies were observed in the lung parenchyma around the nodule. This case shows that a nodule in nodular pulmonary amyloidosis can grow gradually and suggests the possibility of asbestos fibers as one of the etiologic factors in nodular pulmonary amyloidosis. PMID:10846552

  2. A Familial Poikiloderma-Like Cutaneous Amyloidosis

    PubMed Central

    Unni, Mahesh; Ankad, Balachandra; Naidu, Varna; Sudakar Rao, KM

    2014-01-01

    Familial poikiloderma-like cutaneous amyloidosis(FPLCA) is a rare, generalized but genetic dyschromic skin disorder characterized by amyloid deposits in dermis due to defective DNA repair secondary to sunlight damage. Clinically, it presents with diffuse brownish pigmentation with hypo-pigmented macules and many brownish scattered lichenoid papules with normal developmental milestones. The condition is autosomal dominant with incomplete penetrance. We are here reporting a rare familial case of FPLCA with a review of the literature PMID:25484425

  3. Transthyretin cardiac amyloidosis: an under-diagnosed cause of heart failure

    PubMed Central

    Molina O, Gabriela; Judge, Daniel; Campbell, Wayne; Chahal, Harjit; Mugmon, Marc

    2014-01-01

    Introduction Cardiac amyloidosis is the most common cause of infiltrative cardiomyopathy and is associated with a poor prognosis. Transthyretin cardiac amyloidosis, particularly the type caused by the mutation that replaces the amino acid valine with the amino acid isoleucine at position 122 (Val122Ile), is most common among African- Americans above 65 years of age. Evidence suggests that this mutation is an important, though under-diagnosed, cause of heart failure in this population. Case presentation A 74-year-old African American male with a diagnosis of non-ischemic cardiomyopathy for several years, presented with gradually worsening dyspnea on exertion and lower extremity edema. There is no known cardiac disease in his family. An echocardiogram was done showing a decrease in ejection fraction to 30% from 45% in the span of a year. An endomyocardial biopsy analysis identified transthyretin amyloid with the Val122Ile mutation, confirming the diagnosis of familial transthyretin cardiomyopathy. Discussion Systemic amyloidosis is a group of diseases caused by the deposition of an abnormally folded, insoluble protein that can accumulate in multiple organs causing progressive and irreversible dysfunction. The mutations that most commonly induce variant transthyretin cardiac amyloidosis are Val122Ile, Val30Met and Thr60Ala. The Val122Ile mutation has been found to be present in 3–4% of the African American/Caribbean population. Conclusions Familial amyloid cardiomyopathy is an uncommonly recognized cause of heart failure in the population, and patients may wait several years before accurate diagnosis, risking additional significant irreversible deterioration. Patients that meet the high-risk profile criteria – male gender, age 65 years and older, heart failure symptoms, symmetric left ventricular (LV) hypertrophy, and moderately depressed LV function – should likely undergo additional testing for cardiac amyloidosis. PMID:25432650

  4. Cardiac amyloidosis: a review and report of a new transthyretin (prealbumin) variant.

    PubMed Central

    Hesse, A; Altland, K; Linke, R P; Almeida, M R; Saraiva, M J; Steinmetz, A; Maisch, B

    1993-01-01

    Cardiac amyloidosis is caused by amyloid deposits derived from different human plasma proteins. It can lead to cardiac conduction disturbances, restrictive cardiomyopathy, and low output heart failure. The heart is variably involved during the development of systemic amyloidosis and seems to be more frequently affected in immunoglobulin (primary) than in reactive (secondary) amyloidosis. Amyloid is common in the elderly. Isolated atrial amyloid, for which a major subunit is the atrial natriuretic peptide, seems to be three times more frequent than senile cardiac amyloid, which is derived from normal prealbumin (transthyretin). Like polyneuropathy, cardiac amyloidosis is a prominent clinical feature of hereditary amyloidosis, namely of the autosomal dominant transthyretin (TTR) type. All 28 cases of TTR amyloidoses reported so far were heterozygotes for a single nucleotide change in the gene for TTR that resulted in amino acid substitutions in the mature protein. A new TTR genetic variant is reported in a German family where the index patient presented at the age of 63 with anginal pain and arrhythmia. Electrocardiography was suggestive of a pseudoinfarction pattern, and echocardiography and cardiac catheterisation showed signs of hypertrophic nonobstructive cardiomyopathy with increased ventricular filling pressures and a prominent "a" wave. Amyloid of the TTR type was identified by immunohistochemistry in the endomyocardial biopsy specimen. Hybrid isoelectric focusing established heterozygosity by showing normal TTR protein and an electrically neutral TTR variant differing from all known TTR variants so far. The patient died in an accident before investigations were complete. Electrophoretic analysis of the plasma from his first degree relatives (son, daughter, brother, and mother) identified the asymptomatic 22 year old son as an apparently heterozygous carrier of the mutant TTR protein. Comparative tryptic peptide mapping and sequencing showed that isoleucine at

  5. High-dose therapy with auto-SCT is feasible in high-risk cardiac amyloidosis.

    PubMed

    Kongtim, P; Qazilbash, M H; Shah, J J; Hamdi, A; Shah, N; Bashir, Q; Wang, M; Champlin, R; Manasanch, E E; Weber, D; Orlowski, R Z; Parmar, S

    2015-05-01

    Cardiac involvement in light-chain amyloidosis (AL) predicts poor prognosis and is associated with higher TRM and morbidity during high-dose therapy and auto-SCT (HDT-ASCT). We studied the outcomes of 30 patients with cardiac amyloidosis undergoing HDT-ASCT at our center between January 1998 and March 2012. The median age of the patients was 53 years (range, 36-74) with a median follow-up of 35 months (range, 0.4-97 months). Twenty-seven patients (90%) had more than one organ involved besides the heart with 37% with cardiac stage ⩾3. Melphalan-based conditioning regimen (140-200 mg/m(2)) was used for HDT-ASCT. One-year TRM is 10%. Three-year OS and EFS from HDT-ASCT was 83% and 56.8%, respectively. Cumulative incidence of relapse at 3 years was 38.5%. Negative factors affecting survival included age >60 years, lack of novel induction therapy and BM plasmacytosis >10%. We conclude that HDT-ASCT is well tolerated in patients with high-risk cardiac amyloidosis and can lead to improved overall outcomes.

  6. Testicular amyloidosis in hamsters experimentally infected with Leishmania donovani.

    PubMed Central

    Gonzalez, J. L.; Gallego, E.; Castaño, M.; Rueda, A.

    1983-01-01

    Thirty hamsters were inoculated intraperitoneally with Leishmania donovani. Testes were examined grossly and histologically by light and electron microscopy. Progressive testicular atrophy developed. Spermatogenic cells of the seminiferous tubules showed vacuolar degeneration and decreased in number leading to a total azoospermia in the final weeks of the pathological process. Lymphoplasmocytic infiltrates with macrophages containing leishmanias appeared in the intertubular space. Amyloid deposits in the intertubular space and tubular basement membrane were identified by optical and ultrastructural methods. It has been suggested that testicular amyloidosis may have a pathogenic mechanism related to a dysfunction of plasma cells and stimulation of the reticuloendothial system, due to the antigenic character of the parasite. Images Fig. 1 Fig. 2 Fig. 3 Fig. 4 PMID:6639870

  7. Spontaneous resolution of multiple nodular pulmonary AA amyloidosis.

    PubMed

    Fukatsu, Hirotoshi; Miyoshi, Haruka; Ishiki, Kuniharu

    2010-01-01

    A 62-year-old man presented with a two-week history of dry cough. A chest computed tomography (CT) showed three nodular masses of soft tissue density without calcification or cavitary formation in the right lung. F-18 fluorodeoxyglucose PET/CT scan revealed high FDG uptake in two out of three pulmonary nodules. Transbronchial lung biopsy specimens consisted of amorphous eosinophilic deposits that were demonstrated to be amyloid because they were positive for Congo Red staining. After oxidation with permanganate solution, the Congo Red staining disappeared, indicating that this amyloid was amyloid A protein-derived type. There was no evidence of any systemic diseases. We diagnosed the patient as having multiple nodular pulmonary AA amyloidosis. The patient was conservatively managed without treatment, and the pulmonary nodules disappeared spontaneously three months later. PMID:21048364

  8. Genetics Home Reference: transthyretin amyloidosis

    MedlinePlus

    ... a loss of sensation in the extremities (peripheral neuropathy). The autonomic nervous system, which controls involuntary body ... peripheral and autonomic nervous systems, resulting in peripheral neuropathy and difficulty controlling bodily functions. Impairments in bodily ...

  9. Prevalence of amyloid deposition in mature healthy chickens in the flock that previously had outbreaks of vaccine-associated amyloidosis

    PubMed Central

    IBI, Kanata; MURAKAMI, Tomoaki; GODA, Wael Mohamed; KOBAYASHI, Naoki; ISHIGURO, Naotaka; YANAI, Tokuma

    2015-01-01

    Avian amyloid A (AA) amyloidosis is commonly observed in adult birds with chronic inflammation, such as that caused by bacterial infection. We previously described vaccine-associated AA amyloidosis in juvenile chickens. In this study, the prevalence of amyloid deposition was measured in mature healthy chickens that survived a previous outbreak of avian AA amyloidosis while they were juveniles. Herein, we analyzed the amyloid deposition in mature chickens and compared the prevalence of amyloid deposition with juvenile chickens obtained in our previous study (Murakami et al., 2013). We found that: 1) amyloid deposition in the liver was absent in mature chickens, while juvenile chickens had a rate of 24%; 2) amyloid deposition in the spleen was observed in 36% of juvenile chickens and in 40% of mature chickens; 3) amyloid deposition in the pectoral muscle of mature chickens (43.75%) was approximately half that of juvenile chickens (88%). These results suggest that additional amyloid deposition in chickens previously exposed to AA amyloidosis may not worsen with age. Further, amyloid deposition in chickens may tend to regress when causative factors, such as vaccinations and/or chronic inflammation, are absent. PMID:25985816

  10. Unusual Combination of Tracheobronchopathia Osteochondroplastica and AA Amyloidosis

    PubMed Central

    Kırbaş, Gökhan; Dağlı, Canan Eren; Tanrıkulu, Abdullah Çetin; Yıldız, Fetin; Bükte, Yaşar; Kıyan, Esen

    2009-01-01

    Tracheobronchopathia osteochondroplastica (TO) is a rare disorder of unknown cause characterized by the presence of multiple submucosal osseous and/or cartilaginous nodules that protrude into the lumen of the trachea and large bronchi. A simultaneous diagnosis of TO and amyloidosis is rarely reported. In this report, a case initially suspected to be asthma bronchiole that could not be treated, was radiologically diagnosed as TO, and also secondary amyloidosis is presented. A 53 years, man patient reported a 3 years history of dyspnea. Pulmonary function tests (PFTs) showed an obstructive pattern. Chest X-rays revealed right middle lobe atelectasis. FOB and CT detected nodular lesions in the trachea and in the anterior and lateral walls of the main bronchi. AA amyloidosis was confirmed by endobronchial biopsy. In the abdominal fat pad biopsy, amyloidosis was not detected. Asthma bronchiole was excluded by PFTs. This case illustrates that it is possible for TO and amyloidosis to masquerade as asthma. TO and amyloidosis should be suspected in patients of older ages with asthma and especially with poorly treated asthmatic patients. Although nodular lesions in the anterior and lateral tracheobronchial walls are typical for TO, a biopsy should be obtained to exclude amyloidosis. PMID:19881980

  11. Peptide p5 binds both heparinase-sensitive glycosaminoglycans and fibrils in patient-derived AL amyloid extracts.

    PubMed

    Martin, Emily B; Williams, Angela; Heidel, Eric; Macy, Sallie; Kennel, Stephen J; Wall, Jonathan S

    2013-06-21

    In previously published work, we have described heparin-binding synthetic peptides that preferentially recognize amyloid deposits in a mouse model of reactive systemic (AA) amyloidosis and can be imaged by using positron and single photon emission tomographic imaging. We wanted to extend these findings to the most common form of visceral amyloidosis, namely light chain (AL); however, there are no robust experimental animal models of AL amyloidosis. To further define the binding of the lead peptide, p5, to AL amyloid, we characterized the reactivity in vitro of p5 with in situ and patient-derived AL amyloid extracts which contain both hypersulfated heparan sulfate proteoglycans as well as amyloid fibrils. Histochemical staining demonstrated that the peptide specifically localized with tissue-associated AL amyloid deposits. Although we anticipated that p5 would undergo electrostatic interactions with the amyloid-associated glycosaminoglycans expressing heparin-like side chains, no significant correlation between peptide binding and glycosaminoglycan content within amyloid extracts was observed. In contrast, following heparinase I treatment, although overall binding was reduced, a positive correlation between peptide binding and amyloid fibril content became evident. This interaction was further confirmed using synthetic light chain fibrils that contain no carbohydrates. These data suggest that p5 can bind to both the sulfated glycosaminoglycans and protein fibril components of AL amyloid. Understanding these complex electrostatic interactions will aid in the optimization of synthetic peptides for use as amyloid imaging agents and potentially as therapeutics for the treatment of amyloid diseases.

  12. Primary amyloidosis with spontaneous splenic rupture, cholestasis, and liver failure treated with emergency liver transplantation.

    PubMed

    Sandberg-Gertzén, H; Ericzon, B G; Blomberg, B

    1998-11-01

    A 61-yr-old man with cholestatic jaundice soon after presentation had an emergency operation because of spontaneous rupture of the spleen. This was found to be caused by primary systemic amyloidosis. After the splenectomy, the patient deteriorated with liver failure and was successfully treated with liver transplantation. Osteopenic fractures of the thoracic columna developed after transplantation. Except for this, the patient is well 18 months after transplantation.

  13. Amyloid in the cardiovascular system: a review

    PubMed Central

    Kholová, I; Niessen, H W M

    2005-01-01

    The cardiovascular system is a common target of amyloidosis. This review presents the current clinical and diagnostic approach to amyloidosis, with the emphasis on cardiovascular involvement. It summarises recent nomenclature, classification, and pathogenesis of amyloidosis. In addition, non-invasive possibilities are discussed, together with endomyocardial biopsies in the diagnosis of cardiac amyloidosis. Finally, recent advances in treatment and prognostic implications are presented. PMID:15677530

  14. Hereditary apolipoprotein AI-associated renal amyloidosis: A diagnostic challenge.

    PubMed

    Samillán-Sosa, Kelly Del Rocío; Sención-Martínez, Gloria; Lopes-Martín, Vanessa; Martínez-González, Miguel Angel; Solé, Manel; Arostegui, Jose Luis; Mesa, Jose; García-Díaz, Juan de Dios; Rodríguez-Puyol, Diego; Martínez-Miguel, Patricia

    2015-01-01

    Hereditary renal amyloidosis is an autosomal dominant condition with considerable overlap with other amyloidosis types. Differential diagnosis is complicated, but is relevant for prognosis and treatment. We describe a patient with nephrotic syndrome and progressive renal failure, who had a mother with renal amiloidosis. Renal biopsy revealed amyloid deposits in glomerular space, with absence of light chains and protein AA. We suspected amyloidosis with fibrinogen A alpha chain deposits, which is the most frequent cause of hereditary amyloidosis in Europe, with a glomerular preferential affectation. However, the genetic study showed a novel mutation in apolipoprotein AI. On reviewing the biopsy of the patient's mother similar glomerular deposits were found, but there were significant deposits in the renal medulla as well, which is typical in APO AI amyloidosis. The diagnosis was confirmed by immunohistochemistry. Apo AI amyloidosis is characterized by slowly progressive renal disease and end-stage renal disease occurs aproximately 3 to 15 years from initial diagnosis. Renal transplantation offers an acceptable graft survival and in these patients with hepatorenal involvement simultaneous liver and kidney transplantation could be considered.

  15. ADMA Levels Correlate with Proteinuria, Secondary Amyloidosis, and Endothelial Dysfunction

    PubMed Central

    Yilmaz, Mahmut Ilker; Sonmez, Alper; Saglam, Mutlu; Qureshi, Abdul R.; Carrero, Juan Jesus; Caglar, Kayser; Eyileten, Tayfun; Cakir, Erdinc; Oguz, Yusuf; Vural, Abdulgaffar; Yenicesu, Mujdat; Lindholm, Bengt; Stenvinkel, Peter; Axelsson, Jonas

    2008-01-01

    Asymmetric dimethyl-arginine (ADMA), a residue of the proteolysis of arginine-methylated proteins, is a potent inhibitor of nitric oxide synthesis. The increased protein turnover that accompanies proteinuric secondary amyloidosis may increase circulating levels of ADMA, and this may contribute to endothelial dysfunction. We performed a cross-sectional study of 121 nondiabetic proteinuric patients with normal GFR (including 39 patients with nephrotic-range proteinuria and secondary amyloidosis) and 50 age-, sex-, and BMI–matched healthy controls. The proteinuric patients had higher levels of serum ADMA, symmetric dimethyl-arginine (SDMA), high-sensitivity C-reactive protein (hsCRP), and insulin resistance (homeostasis model assessment index) than controls. Compared with controls, brachial artery flow-mediated dilatation (FMD), serum L-Arginine, and the L-Arginine/ADMA ratio were significantly lower among proteinuric patients, suggesting greater endothelial dysfunction. When patients with secondary amyloidosis were compared with patients with glomerulonephritis who had similar levels of proteinuria, those with amyloidosis had higher ADMA and SDMA levels and lower L-Arginine/ADMA ratios and FMD measurements (P < 0.001 for all). Finally, even after adjusting for confounders, ADMA level correlated with both proteinuria and the presence of secondary amyloidosis, and was an independent predictor of FMD. We propose that ADMA synthesis may be increased in chronic kidney disease, especially in secondary amyloidosis, and this may explain part of the mechanism by which proteinuria increases cardiovascular morbidity and mortality. PMID:18199801

  16. Multiple myeloma-associated skin light chain amyloidosis: A case of misdiagnosis

    PubMed Central

    XIAO, LI; LIN, FENGXIA; XIAO, RONG; HU, CHUN; DENG, MINGYANG; LI, DAIQIANG; SHE, XIAOLING; LIU, FUYOU; SUN, LIN

    2016-01-01

    The present study reports the case of a 42-year-old male with multiple myeloma (MM)-associated skin light chain amyloidosis who presented with skin purpura as the initial symptom, which was misdiagnosis as Henoch-Schönlein purpura nephritis prior to admission to the Second Xiangya Hospital (Changsha, Hunan, China). The patient presented with purpura, papules petechiae and spontaneous ecchymosis, which was located scattered around the neck, chest and limbs, accompanied by a small amount of bleeding in the conjunctival and oral mucosa, and a swollen tongue. Upon laboratory examination, the serum immunological change showed increased serum immunoglobulin G and λ light chain levels, and a urine Bence Jones protein level of >1 g/24 h. This was accompanied with an abnormal result for immunofixation electrophoresis, and positive staining with Congo red showing apple-green birefringence in skin biopsy specimens. Thus, the patient was diagnosed with MM-associated skin amyloidosis with the initial symptom of skin purpura. Following treatment with chemotherapy consisting of prednisone and bortezomib, the skin lesions markedly improved. The present study indicates that the presentation of skin purpura in systemic amyloidosis associated with MM may be an important aid in the diagnosis and direct treatment of this disease in the clinic. PMID:27284363

  17. Evolving landscape in the management of transthyretin amyloidosis

    PubMed Central

    Hawkins, Philip N.; Ando, Yukio; Dispenzeri, Angela; Gonzalez-Duarte, Alejandra; Adams, David; Suhr, Ole B.

    2015-01-01

    Transthyretin (TTR) amyloidosis (ATTR amyloidosis) is a multisystemic, multigenotypic disease resulting from deposition of insoluble ATTR amyloid fibrils in various organs and tissues. Although considered rare, the prevalence of this serious disease is likely underestimated because symptoms can be non-specific and diagnosis largely relies on amyloid detection in tissue biopsies. Treatment is guided by which tissues/organs are involved, although therapeutic options are limited for patients with late-stage disease. Indeed, enthusiasm for liver transplantation for familial ATTR amyloidosis with polyneuropathy was dampened by poor outcomes among patients with significant neurological deficits or cardiac involvement. Hence, there remains an unmet medical need for new therapies. The TTR stabilizers tafamidis and diflunisal slow disease progression in some patients with ATTR amyloidosis with polyneuropathy, and the postulated synergistic effect of doxycycline and tauroursodeoxycholic acid on dissolution of amyloid is under investigation. Another therapeutic approach is to reduce production of the amyloidogenic protein, TTR. Plasma TTR concentration can be significantly reduced with ISIS-TTRRx, an investigational antisense oligonucleotide-based drug, or with patisiran and revusiran, which are investigational RNA interference-based therapeutics that target the liver. The evolving treatment landscape for ATTR amyloidosis brings hope for further improvements in clinical outcomes for patients with this debilitating disease. PMID:26611723

  18. Evolving landscape in the management of transthyretin amyloidosis.

    PubMed

    Hawkins, Philip N; Ando, Yukio; Dispenzeri, Angela; Gonzalez-Duarte, Alejandra; Adams, David; Suhr, Ole B

    2015-01-01

    Transthyretin (TTR) amyloidosis (ATTR amyloidosis) is a multisystemic, multigenotypic disease resulting from deposition of insoluble ATTR amyloid fibrils in various organs and tissues. Although considered rare, the prevalence of this serious disease is likely underestimated because symptoms can be non-specific and diagnosis largely relies on amyloid detection in tissue biopsies. Treatment is guided by which tissues/organs are involved, although therapeutic options are limited for patients with late-stage disease. Indeed, enthusiasm for liver transplantation for familial ATTR amyloidosis with polyneuropathy was dampened by poor outcomes among patients with significant neurological deficits or cardiac involvement. Hence, there remains an unmet medical need for new therapies. The TTR stabilizers tafamidis and diflunisal slow disease progression in some patients with ATTR amyloidosis with polyneuropathy, and the postulated synergistic effect of doxycycline and tauroursodeoxycholic acid on dissolution of amyloid is under investigation. Another therapeutic approach is to reduce production of the amyloidogenic protein, TTR. Plasma TTR concentration can be significantly reduced with ISIS-TTR(Rx), an investigational antisense oligonucleotide-based drug, or with patisiran and revusiran, which are investigational RNA interference-based therapeutics that target the liver. The evolving treatment landscape for ATTR amyloidosis brings hope for further improvements in clinical outcomes for patients with this debilitating disease. PMID:26611723

  19. Long term outcomes of cardiac transplant for immunoglobulin light chain amyloidosis: The Mayo Clinic experience

    PubMed Central

    Grogan, Martha; Gertz, Morie; McCurdy, Arleigh; Roeker, Lindsey; Kyle, Robert; Kushwaha, Sudhir; Daly, Richard; Dearani, Joseph; Rodeheffer, Richard; Frantz, Robert; Lacy, Martha; Hayman, Suzanne; McGregor, Christopher; Edwards, Brooks; Dispenzieri, Angela

    2016-01-01

    AIM: To determine the outcome of orthotopic heart transplantation (OHT) in immunoglobulin light chain (AL) amyloidosis. METHODS: The medical records of patients with AL who underwent orthotopic heart transplantation at the Mayo Clinic in Rochester Minnesota from 1992 to 2011 were reviewed. Patients met at least one of the following at: New York Heart Association class IV heart failure, ventricular thickness > 15 mm, ejection fraction < 40%. Selection guidelines for heart transplant included age < 60 years, absence of multiple myeloma and significant extra-cardiac organ involvement. Baseline characteristics including age, gender, organ involvement, and New York Heart Association functional class were recorded. Laboratory data, waiting time until heart transplant, and type of treatment of the underlying plasma cell disorder were recorded. Survival from the time of OHT was calculated using Kaplan-Meier survival curves. Survival of patients undergoing OHT for AL was compared to that of non-amyloid patients undergoing OHT during the same time period. RESULTS: Twenty-three patients (median age 53 years) with AL received OHT. There were no deaths in the immediate perioperative period. Twenty patients have died post OHT. For the entire cohort, the median overall survival was 3.5 years (95%CI: 1.2, 8.2 years). The 1-year survival post OHT was 77%, the 2-year survival 65%, and the 5-year survival 43%. The 5-year survival for non-amyloid patients undergoing OHT during the same era was 85%. Progressive amyloidosis contributed to death in twelve patients. Of those without evidence of progressive amyloidosis, the cause of death included complications of autologous hematopoietic stem cell transplantation for 3 patients, post-transplant lymphoproliferative disorder for 2 patients; and for the remaining one death was related to each of the following causes: acute rejection; cardiac vasculopathy; metastatic melanoma; myelodysplastic syndrome; and unknown. Eight patients had

  20. UNUSUAL PRESENTATION OF GENERALIZED MACULAR AMYLOIDOSIS IN A YOUNG ADULT

    PubMed Central

    Kudur, Mohan H; B, Sathish Pai; H, Sripathi; Prabhu, Smitha

    2008-01-01

    Macular amyloidosis is a common problem seen dermatology out-patient department. Generalized macular amyloidosis presenting with a poikilodermatous appearance is rare. In our case, an 18-year-old male presented with generalized hypopigmented macules with a poikilodermatous appearance of 10-year duration. His developmental milestones were normal with negative family history of similar complaints. Histopathology of hyperpigmented lesions revealed hyperkeratosis and acanthosis of epidermis and hypopigmented lesion showing only hyperkeratosis. Both lesions were showing the deposition of amorphous, hazy material in the tips of papillary dermis with perivascular inflammatory infiltrate. Congo red staining of the amorphous material was positive for amyloid. PMID:19882037

  1. [Hepatic amyloidosis as cause of severe intrahepatic cholestasis].

    PubMed

    Gavilán, J C; Bermúdez, F J; Márquez, A; Sánchez-Carrillo, J J; González-Santos, P

    2003-01-01

    The liver is frequently involved by amyloidosis, but hyperbilirubinemia and liver failure are uncommon features. A mild elevation of the serum alkaline phosphatase value and, less frequently, hepatomegaly are the most common findings. Usually the patients have no symptoms related with the liver involvement; the clinical manifestation and the long term prognosis depends on the renal and cardiac disease. We report an unusual clinical presentation of primary amyloidosis in a previously asymptomatic 65 years old woman who was admitted to the hospital because of ictericia and ascitis mimicking a drug induced acute hepatic failure.

  2. 'Abd al-Rahman al-Sufi's 3-Step Magnitude System

    NASA Astrophysics Data System (ADS)

    Hafez, Ihsan; Stephenson, F. Richard; Orchiston, Wayne

    'Abd al-Rahmān al-ūfī's Book of the Fixed Stars dates from around AD 964 and is one of the most important medieval Arabic treatises on astronomy. In this paper we begin with a very brief introduction to the Book of the Fixed Stars. This book contains an extensive star catalogue that lists star coordinates and magnitude estimates for all of the Ptolemaic stars. However, in his book al-hūfī utilized three distinct intermediate magnitude values whereas Ptolemy only mentioned two. We believe that al-hūfī used what we have termed a '3-step intermediate magnitude system,' which is more accurate than Ptolemy's 2-step intermediate system. In this paper we examine in detail the accuracy of this unique 3-step system in comparison with Ptolemy's and modern magnitude values.

  3. Phenotypic, transcriptomic, and genomic features of clonal plasma cells in light-chain amyloidosis.

    PubMed

    Paiva, Bruno; Martinez-Lopez, Joaquin; Corchete, Luis A; Sanchez-Vega, Beatriz; Rapado, Inmaculada; Puig, Noemi; Barrio, Santiago; Sanchez, Maria-Luz; Alignani, Diego; Lasa, Marta; García de Coca, Alfonso; Pardal, Emilia; Oriol, Alberto; Garcia, Maria-Esther Gonzalez; Escalante, Fernando; González-López, Tomás J; Palomera, Luis; Alonso, José; Prosper, Felipe; Orfao, Alberto; Vidriales, Maria-Belen; Mateos, María-Victoria; Lahuerta, Juan-Jose; Gutierrez, Norma C; San Miguel, Jesús F

    2016-06-16

    Immunoglobulin light-chain amyloidosis (AL) and multiple myeloma (MM) are 2 distinct monoclonal gammopathies that involve the same cellular compartment: clonal plasma cells (PCs). Despite the fact that knowledge about MM PC biology has significantly increased in the last decade, the same does not apply for AL. Here, we used an integrative phenotypic, molecular, and genomic approach to study clonal PCs from 24 newly diagnosed patients with AL. Through principal-component-analysis, we demonstrated highly overlapping phenotypic profiles between AL and both monoclonal gammopathy of undetermined significance and MM PCs. However, in contrast to MM, highly purified fluorescence-activated cell-sorted clonal PCs from AL (n = 9) showed almost normal transcriptome, with only 38 deregulated genes vs normal PCs; these included a few tumor-suppressor (CDH1, RCAN) and proapoptotic (GLIPR1, FAS) genes. Notwithstanding, clonal PCs in AL (n = 11) were genomically unstable, with a median of 9 copy number alterations (CNAs) per case, many of such CNAs being similar to those found in MM. Whole-exome sequencing (WES) performed in 5 AL patients revealed a median of 15 nonrecurrent mutations per case. Altogether, our results show that in the absence of a unifying mutation by WES, clonal PCs in AL display phenotypic and CNA profiles similar to MM, but their transcriptome is remarkably similar to that of normal PCs. PMID:27069257

  4. Burden of cytogenetically abnormal plasma cells in light chain amyloidosis and their prognostic relevance.

    PubMed

    Kim, Seon Young; Im, Kyongok; Park, Si Nae; Kim, Jung-Ah; Yoon, Sung-Soo; Lee, Dong Soon

    2016-05-01

    We performed cytoplasmic fluorescence in situ hybridization assays of light chain amyloidosis (AL). In total, 234 patients were enrolled: 28 patients with AL, 24 with monoclonal gammopathy of undetermined significance (MGUS), and 182 with multiple myeloma (MM). Chromosomal abnormalities were detected in 13 of 22 (59%) AL patients without MM. All 13 patients demonstrated IGH rearrangement, and t(11;14)/IGH-CCND1 was most frequent (32%). Chromosome gain was not observed in AL patients without MM. These findings were dissimilar to findings in MGUS patients, in whom trisomy 9 was the most frequent abnormality. Of 6 AL patients with MM, 5 (83%) patients had cytogenetic abnormalities: 1q gain (4/6, 67%), gains of chromosome 9 (3/6, 50%), IGH rearrangement and RB1 (13q) deletions (2/6 each, 33%). The percentage of clonal plasma cells among total plasma cells was variable (median, 75%; range, 16-100%) for AL patients without MM, which was lower than the results for MM patients (median 100%). The overall survival of AL patients without MM was not significantly different according to the presence of cytogenetic abnormalities (P=0.510). In summary, among Korean AL patients, IGH rearrangement was the most frequent cytogenetic abnormality and cytogenetic aberration patterns differ compared with MGUS and MM patients. PMID:27015231

  5. [Otolaryngological complaints in tongue amyloidosis: a case report].

    PubMed

    Pons Rocher, F; Guallart Doménech, F; Mompó Romero, L; Artazkozl del Toro, J J; Serrano Badía, E; Dalmau Galofré, J; Faubel Serra, M

    1994-01-01

    We present a case of Amyloidosis of the oral cavity associated to multiple mieloma, with otolaryngological symptom. Review of structural characterization of the disease, its pathogenesis and clinical disorders when displayed in thyroid, oral cavity and upper respiratory tract. PMID:7864303

  6. Primary hepatic amyloidosis: A case report and review of literature

    PubMed Central

    Sonthalia, Nikhil; Jain, Samit; Pawar, Sunil; Zanwar, Vinay; Surude, Ravindra; Rathi, Praveen M

    2016-01-01

    We describe a case of 42-year-old female presenting with abdominal pain associated with loss of weight and fever for 8 mo. On evaluation she had gross hepatomegaly with raised alkaline phosphatase and raised GGT levels with normal transaminases and bilirubin. On imaging she had diffuse enlargement of liver with heterogeneous contrast uptake in liver. Her viral marker and autoimmune markers were negative. Liver biopsy depicted massive deposition of amyloid in peri-sinusoidal spaces which revealed apple green birefringence on polarizing microscopy after Congo red staining. Cardiac and renal evaluation was unremarkable. Abdominal fat pad and rectum biopsy was negative for amyloid deposit. There was no evidence of primary amyloidosis as bone marrow examination was normal. Serum and urine immunofixation electrophoresis were normal. Immunoperoxidase staining for serum amyloid associated protein for secondary amyloidosis was negative from liver biopsy. We present this rare case of primary hepatic amyloidosis and review the literature regarding varied presentations of hepatic involvement in amyloidosis. PMID:26962400

  7. Evaluation of 99mTc-MAMA-chrysamine G as an in vivo probe for amyloidosis.

    PubMed

    Dezutter, N A; Landman, W J; Jager, P L; de Groot, T J; Dupont, P J; Tooten, P C; Zekarias, B; Gruys, E; Verbruggen, A M

    2001-09-01

    To date, systemic amyloidosis is diagnosed histologically using Congo red staining or in vivo using iodine-123 labelled serum amyloid P component (123I-SAP) scintigraphy. We developed 99mTc-MAMA-CG, a 99mTc-labelled derivative of the lipophilic Congo red analogue chrysamine G (CG), as a possible alternative to 123I-SAP. In vivo 99mTc-MAMA-CG scintigraphy, performed in chickens with spontaneous joint amyloidosis, resulted as soon as 10 min after injection in scintigraphic images showing uptake of activity in amyloid-loaded organs (liver, joints). One of these chickens was studied also with 123I-SAP resulting in scintigraphic images revealing 123I-SAP binding to amyloid deposits in the liver. However, up to 11 h after injection no radioactivity was visible in the amyloid positive joints. In vitro autoradiography, performed on sections of chicken joints with Enterococcus faecalis induced amyloid arthropathy (chjAA), demonstrated the failure of 99mTc-MAMA-CG to bind significantly to amyloid deposits in the presence of 10 microM Congo red The specificity of 99mTc-MAMA-CG localisation was also established by the absence of 99mTc-MAMA-CG binding in non-amyloidotic organs in vitro and in vivo. 99mTc-MAMA-CG did not show any sign of acute toxicity. These findings establish the usefulness of 99mTc-MAMA-CG as a non-invasive in vivo diagnostic probe in chickens with amyloid arthropathy and suggest that it may also be applicable to human amyloidosis.

  8. Phase competition in ternary Ti-Ni-Al system

    NASA Astrophysics Data System (ADS)

    Wierzba, Bartek

    2016-07-01

    In this paper the reactive diffusion in Ti-Ni-Al system is discussed at 1173 K. The calculation method based on the binary approach is presented. The key kinetic parameter is Wagner integral diffusion coefficient. The experimental and simulation results of reactive diffusion between pure Ti and β-NiAl are compared at 1173 K after 100 h.

  9. Successful Treatment of Amyloid Light-chain Amyloidosis in a Charcot-Marie-Tooth Disease Patient with Lenalidomide, Cyclophosphamide, and Dexamethasone.

    PubMed

    Kikukawa, Yoshitaka; Hata, Hiroyuki; Ueda, Mitsuharu; Yamashita, Taro; Nasu, Singo; Ide, Kazuhiko; Ueno, Shikiko; Ando, Yukio; Mitsuya, Hiroaki; Okuno, Yutaka

    2016-01-01

    A 70-year-old woman with Charcot-Marie-Tooth disease (CMT) suffered from nephrotic syndrome and a renal biopsy revealed non-AA amyloid depositions that contained immunoglobulin light chain λ. Her serum λ free LC was elevated to 80.8 mg/L and she was diagnosed with primary amyloid light-chain (AL) amyloidosis. She was subsequently treated with lenalidomide, cyclophosphamide, and dexamethasone (RCD). After 14 cycles of RCD, she achieved complete remission. Her serum albumin levels gradually normalized to 3.1 g/dL. No exacerbation of neurologic symptoms related to CMT was observed. Thus, RCD may be a well-tolerated and effective regimen for treating AL amyloidosis in patients with CMT disease. PMID:27629972

  10. Interdiffusion in the Mg-Al system and Intrinsic Diffusion in (Al3Mg2) Phase

    SciTech Connect

    Brennan, Sarah; Bermudez, Katrina; Kulkarni, Nagraj S; Sohn, Yong Ho

    2011-01-01

    Increasing use and development of lightweight Mg-alloys have led to the desire for more fundamental research in and understanding of Mg-based systems. As a strengthening component, Al is one of the most important and common alloying elements for Mg-alloys. In this study, solid-to-solid diffusion couple techniques were employed to examine the interdiffusion between pure Mg and Al. Diffusion anneals were carried out at 300 , 350 , and 400 C for 720, 360, and 240 hours, respectively. Optical and scanning electron microscopies (SEM) were employed to observe the formation of the intermetallics -Al12Mg17 and -Al3Mg2, but not -phase. Concentration profiles were determined using X-ray energy dispersive spectroscopy (XEDS). The growth constants and activation energies were determined for each intermetallic phase.

  11. Apolipoprotein AI mutation Arg-60 causes autosomal dominant amyloidosis.

    PubMed Central

    Soutar, A K; Hawkins, P N; Vigushin, D M; Tennent, G A; Booth, S E; Hutton, T; Nguyen, O; Totty, N F; Feest, T G; Hsuan, J J

    1992-01-01

    A mutation in the gene for apolipoprotein AI (apoAI) was identified in an English family with autosomal dominant non-neuropathic systemic amyloidosis. The plasma of all affected individuals contained a variant apoAI with one additional charge, as well as normal apoAI. The propositus was heterozygous; the coding region of his apoAI gene contained both the normal sequence and a single-base substitution changing the codon for residue 60 of the mature protein from CTG (leucine) to CGG (arginine). Allele-specific oligonucleotide hybridization showed that the other affected individuals were also heterozygotes and that there was concordance of the mutant allele with the presence of variant plasma apoAI. Amyloid fibrils isolated from the spleen of the propositus consisted of proteins that ran as a doublet with an apparent mass of approximately 10 kDa in SDS/PAGE and a trace band at 28 kDa. Electrospray mass spectrometry of the purified 10-kDa material revealed components with mass corresponding to the N-terminal 88, 92, 93, and 94 residues of apoAI each with substitution of arginine for leucine. These observations were confirmed by direct protein sequencing and laser desorption time-of-flight mass analysis. No material with the normal apoAI sequence was detected. The trace band at 28 kDa yielded the N-terminal sequence of mature apoAI, indicating that intact or minimally degraded apoAI was also present in the fibril preparation. Discovery of this mutation and the detailed characterization of the apoAI fragments that form the amyloid fibrils open additional avenues for investigation of amyloidogenesis. Images PMID:1502149

  12. The Advanced Light Source (ALS) Radiation Safety System. Revised

    SciTech Connect

    Ritchie, A.L.; Oldfather, D.E.; Lindner, A.F.

    1993-08-01

    The Advanced Light Source (ALS) at the Lawrence Berkeley Laboratory (LBL) is a 1.5 Gev synchrotron light source facility consisting of a 120 kev electron gun, 50 Mev linear accelerator, 1.5 Gev booster synchrotron, 200 meter circumference electron storage ring, and many photon beamline transport systems for research. Figure 1. ALS floor plan. Pairs of neutron and gamma radiation monitors are shown as dots numbered from 1 to 12. The Radiation Safety System for the ALS has been designed and built with a primary goal of providing protection against inadvertent personnel exposure to gamma and neutron radiation and, secondarily, to enhance the electrical safety of select magnet power supplies.

  13. Peptide p5 binds both heparinase-sensitive glycosaminoglycans and fibrils in patient-derived AL amyloid extracts

    SciTech Connect

    Martin, Emily B.; Williams, Angela; Heidel, Eric; Macy, Sallie; Kennel, Stephen J.; Wall, Jonathan S.

    2013-06-21

    Highlights: •Polybasic peptide p5 binds human light chain amyloid extracts. •The binding of p5 with amyloid involves both glycosaminoglycans and fibrils. •Heparinase treatment led to a correlation between p5 binding and fibril content. •p5 binding to AL amyloid requires electrostatic interactions. -- Abstract: In previously published work, we have described heparin-binding synthetic peptides that preferentially recognize amyloid deposits in a mouse model of reactive systemic (AA) amyloidosis and can be imaged by using positron and single photon emission tomographic imaging. We wanted to extend these findings to the most common form of visceral amyloidosis, namely light chain (AL); however, there are no robust experimental animal models of AL amyloidosis. To further define the binding of the lead peptide, p5, to AL amyloid, we characterized the reactivity in vitro of p5 with in situ and patient-derived AL amyloid extracts which contain both hypersulfated heparan sulfate proteoglycans as well as amyloid fibrils. Histochemical staining demonstrated that the peptide specifically localized with tissue-associated AL amyloid deposits. Although we anticipated that p5 would undergo electrostatic interactions with the amyloid-associated glycosaminoglycans expressing heparin-like side chains, no significant correlation between peptide binding and glycosaminoglycan content within amyloid extracts was observed. In contrast, following heparinase I treatment, although overall binding was reduced, a positive correlation between peptide binding and amyloid fibril content became evident. This interaction was further confirmed using synthetic light chain fibrils that contain no carbohydrates. These data suggest that p5 can bind to both the sulfated glycosaminoglycans and protein fibril components of AL amyloid. Understanding these complex electrostatic interactions will aid in the optimization of synthetic peptides for use as amyloid imaging agents and potentially as

  14. Microstructure of the Al-La-Ni-Fe system

    SciTech Connect

    Vasil’ev, A. L.; Ivanova, A. G.; Bakhteeva, N. D.; Kolobylina, N. N.; Orekhov, A. S.; Presnyakov, M. Yu.; Todorova, E. V.

    2015-01-15

    The microstructure of alloys based on the Al-La-Ni-Fe system, which are characterized by a unique ability to form metal glasses and nanoscale composites in a wide range of compositions, has been investigated. Al{sub 85}Ni{sub 7}Fe{sub 4}La{sub 4} and Al{sub 85}Ni{sub 9}Fe{sub 2}La{sub 4} alloys have been analyzed by electron microscopy (including high-resolution scanning transmission electron microscopy), energy-dispersive X-ray microanalysis, electron diffraction (ED), and X-ray diffraction (XRD). It is found that, along with fcc Al and Al{sub 4}La (Al{sub 11}La{sub 3}) particles, these alloys contain a ternary phase Al{sub 3}Ni{sub 1−x}Fe{sub x} (sp. gr. Pnma) isostructural to the Al{sub 3}Ni phase and a quaternary phase Al{sub 8}Fe{sub 2−x}Ni{sub x}La isostructural to the Al{sub 8}Fe{sub 2}Eu phase (sp. gr. Pbam). The unit-cell parameters of the Al{sub 3}Ni{sub 1−x}Fe{sub x} and Al{sub 8}Fe{sub 2−x}Ni{sub x}La compounds, determined by ED and refined by XRD, are a = 0.664(1) nm, b = 0.734(1) nm, and c = 0.490(1) nm for Al{sub 3}Ni{sub 1−x}Fe{sub x} and a = 1.258(3) nm, b = 1.448(3) nm, and c = 0.405(8) nm for Al{sub 8}Fe{sub 2−x}Ni{sub x}La. In both cases Ni and Fe atoms are statistically arranged, and no ordering is found. Al{sub 8}Fe{sub 2−x}Ni{sub x}La particles contain inclusions in the form of Al{sub 3}Fe δ layers.

  15. Chronic eyelid swelling as an initial manifestation of myeloma-associated amyloidosis.

    PubMed

    Chee, Elaine; Kim, Yoon-Duck; Lee, Jung Hye; Woo, Kyung In

    2013-01-01

    Orbital amyloidosis is uncommon and difficult to diagnose due to their variable clinical presentations. The authors report a case of a patient who presents with chronic eyelid swelling as an initial manifestation of myeloma-associated amyloidosis. This patient was also found to have retrobulbar infiltration with no visual impairment. The authors also describe the first documentation of the atypical necrotic appearance of amyloidosis in the involved eyelid tissues. Myeloma-associated amyloidosis can present as chronic, nonspecific periorbital swelling, hence a biopsy of the affected tissues is important in preventing a delay in the correct diagnosis.

  16. Macular posterior pigmentary incontinence: its relation to macular amyloidosis and notalgia paresthetica.

    PubMed

    Westermark, P; Ridderström, E; Vahlquist, A

    1996-07-01

    Patients with clinical features of dorsal macular amyloidosis but without subepidermal amyloid deposits were followed for 2-11 years. The clinical appearance was fairly stable during this period of time, with little tendency of healing. Only 2 of the patients developed typical macular amyloidosis during the follow-up. It is concluded that a condition strongly resembling macular amyloidosis but without amyloid is an entity, and the designation "macular posterior pigmentary incontinence" is proposed. The relationship between macular posterior pigmentary incontinence and the two conditions macular amyloidosis and notalgia paresthetica is discussed. PMID:8869690

  17. Molecular basis of a novel renal amyloidosis due to N184K gelsolin variant.

    PubMed

    Bonì, Francesco; Milani, Mario; Porcari, Riccardo; Barbiroli, Alberto; Ricagno, Stefano; de Rosa, Matteo

    2016-09-16

    Mutations in gelsolin are responsible for a systemic amyloidosis first described in 1969. Until recently, the disease was associated with two substitutions of the same residue, leading to the loss of the calcium binding site. Novel interest arose in 2014 when the N184K variant of the protein was identified as the etiological agent of a novel kidney-localized amyloidosis. Here we provide a first rationale for N184K pathogenicity. We show that the mutation induces a destabilization of gelsolin second domain, without compromising its calcium binding capacity. X-ray data combined with molecular dynamics simulations demonstrates that the primary source of the destabilization is a loss of connectivity in proximity of the metal. Such rearrangement of the H-bond network does not have a major impact on the overall fold of the domain, nevertheless, it increases the flexibility of a stretch of the protein, which is consequently processed by furin protease. Overall our data suggest that the N184K variant is subjected to the same aberrant proteolytic events responsible for the formation of amyloidogenic fragments in the previously characterized mutants. At the same time our data suggest that a broader number of mutations, unrelated to the metal binding site, can lead to a pathogenic phenotype.

  18. Molecular basis of a novel renal amyloidosis due to N184K gelsolin variant

    PubMed Central

    Bonì, Francesco; Milani, Mario; Porcari, Riccardo; Barbiroli, Alberto; Ricagno, Stefano; de Rosa, Matteo

    2016-01-01

    Mutations in gelsolin are responsible for a systemic amyloidosis first described in 1969. Until recently, the disease was associated with two substitutions of the same residue, leading to the loss of the calcium binding site. Novel interest arose in 2014 when the N184K variant of the protein was identified as the etiological agent of a novel kidney-localized amyloidosis. Here we provide a first rationale for N184K pathogenicity. We show that the mutation induces a destabilization of gelsolin second domain, without compromising its calcium binding capacity. X-ray data combined with molecular dynamics simulations demonstrates that the primary source of the destabilization is a loss of connectivity in proximity of the metal. Such rearrangement of the H-bond network does not have a major impact on the overall fold of the domain, nevertheless, it increases the flexibility of a stretch of the protein, which is consequently processed by furin protease. Overall our data suggest that the N184K variant is subjected to the same aberrant proteolytic events responsible for the formation of amyloidogenic fragments in the previously characterized mutants. At the same time our data suggest that a broader number of mutations, unrelated to the metal binding site, can lead to a pathogenic phenotype. PMID:27633054

  19. Molecular basis of a novel renal amyloidosis due to N184K gelsolin variant.

    PubMed

    Bonì, Francesco; Milani, Mario; Porcari, Riccardo; Barbiroli, Alberto; Ricagno, Stefano; de Rosa, Matteo

    2016-01-01

    Mutations in gelsolin are responsible for a systemic amyloidosis first described in 1969. Until recently, the disease was associated with two substitutions of the same residue, leading to the loss of the calcium binding site. Novel interest arose in 2014 when the N184K variant of the protein was identified as the etiological agent of a novel kidney-localized amyloidosis. Here we provide a first rationale for N184K pathogenicity. We show that the mutation induces a destabilization of gelsolin second domain, without compromising its calcium binding capacity. X-ray data combined with molecular dynamics simulations demonstrates that the primary source of the destabilization is a loss of connectivity in proximity of the metal. Such rearrangement of the H-bond network does not have a major impact on the overall fold of the domain, nevertheless, it increases the flexibility of a stretch of the protein, which is consequently processed by furin protease. Overall our data suggest that the N184K variant is subjected to the same aberrant proteolytic events responsible for the formation of amyloidogenic fragments in the previously characterized mutants. At the same time our data suggest that a broader number of mutations, unrelated to the metal binding site, can lead to a pathogenic phenotype. PMID:27633054

  20. Phase correlations in the CuAlSe2-CuAlTe2 system

    NASA Astrophysics Data System (ADS)

    Korzun, B. V.; Fadzeyeva, A. A.; Bente, K.; Schmitz, W.; Kommichau, G.

    2005-07-01

    Alloys in the CuAlSe2-CuAlTe2 system were synthesized in BN-crucibles in silica tubes under vacuum to obtain the corresponding phase equilibria. X-ray powder diffraction and thermal analytic data of the T-x phase diagram revealed a complete solid solutions series in the subsolidus region. Within the CuAlSe2xTe2(1-x) system the refined lattice parameters a and c approximately obey the Vegard rule and also the cell volume and the heat of fusion confirm linear correlations with the composition of the mixed crystals. The anion position parameter calculated after S. C. Abrahams & J. L. Bernstein (uAB) and J. E Jaffe & A. Zunger (uJZ) is greater than 0.25 and reveals a linear dependence on composition. The liquidus part of the CuAlSe2xTe2(1-x) system with x < 0.35 exhibits vertical section behaviour with a ternary peritectic followed up by a ternary monotectic whereas the region with x > 0.35 shows quasibinary equilibria.

  1. Familial Amyloidosis Cutis Dyschromica in Three Siblings: Report from Indonesia

    PubMed Central

    Hermawan, Melyawati; Rihatmadja, Rahadi; Sirait, Sondang Pandjaitan

    2014-01-01

    Amyloidosis cutis dyschromica (ACD) is an extremely rare type of primary cutaneous amyloidosis. To date there are fewer than 40 published cases worldwide; some were reported affecting several family members. Its resemblance to other common pigmentation disorders makes it rarely recognized at first sight. Our patient, the 12-year-old firstborn son of non-consanguineous parents presented with generalized mottled pigmentation starting from lower extremities. His siblings suffered from similar condition. The clue for diagnosis is the amyloid deposition in the papillary dermis. The etiology of ACD is still unknown, but genetic factors and ultraviolet radiation are implicated. It is proposed that disturbance of keratinocyte repair following ultraviolet radiation results in amyloid deposition. The treatment remains a challenge. Oral acitretin treatment, thought to repair keratinization defect, gave a slight improvement in our case. Our is the first case of ACD reported in Indonesia. PMID:25386328

  2. Reaction diffusion in the NiCrAl and CoCrAl systems

    NASA Technical Reports Server (NTRS)

    Levine, S. R.

    1978-01-01

    The paper assesses the effect of overlay coating and substrate composition on the kinetics of coating depletion by interdiffusion. This is accomplished by examining the constitution, kinetics and activation energies for a series of diffusion couples primarily of the NiCrAl/Ni-10Cr or CoCrAl/Ni-10Cr type annealed at temperatures in the range 1000-1205 C for times up to 500 hr. A general procedure is developed for analyzing diffusion in multicomponent multiphase systems. It is shown that by introducing the concept of beta-source strength, which can be determined from appropriate phase diagrams, the Wagner solution for consumption of a second phase in a semiinfinite couple is successfully applied to the analysis of MCrAl couples. Thus, correlation of beta-recession rate constants with couple composition, total and diffusional activation energies, and interdiffusion coefficients are determined.

  3. Buccal mucosa graft urethroplasty in a case of urethral amyloidosis presenting with long anterior urethral stricture

    PubMed Central

    Kurbatov, Dmitry; Stojanovic, Borko; Dubskiy, Sergey; Lepetukhin, Alex; Djordjevic, Miroslav L.

    2015-01-01

    Urethral amyloidosis is a rare condition, but clinically relevant because it can mimic urothelial carcinoma. We report a case of localized urethral amyloidosis presenting with a long anterior urethral stricture. We used extensive grafts of buccal mucosa for standard augmentation urethroplasty, with a successful outcome at the 2-year follow-up. PMID:26600896

  4. Phase relations in the U-Mo-Al ternary system

    NASA Astrophysics Data System (ADS)

    Noël, H.; Tougait, O.; Dubois, S.

    2009-06-01

    The phase relations in the U-Mo-Al system of quenched samples annealed at 800 °C for 2 weeks and at 400 °C for 2 months have been established using X-ray powder diffraction, scanning electron microscopy and energy dispersive spectroscopic analysis performed at room temperature. Two ternary Al-rich phases, UMo 2-xAl 20+x and U 6Mo 4+xAl 43-x are found stable at 800 °C and 400 °C. They show significant homogeneity ranges resulting from Mo/Al substitution mechanism on various mixed crystallographic sites, as evidenced by single-crystal structure refinements. Substitution of up to 25 at.% of Al by Mo atoms is also observed for UAl 2 (cubic MgCu 2-type) giving a quite large extension (UAl 2-xMo x, 0 < x < 0.5) into the ternary system. Larger substitution (0.6 < x < 0.7 at T = 800 °C) stabilizes another ternary Laves phase, UAl 2-xMo x with the hexagonal MgZn 2-type. There is no detectable solubility of Mo in UAl 4, and it is of the order of 1 at.% in UAl 3. The interaction layers between the γU-Mo alloys and the Al matrix in nuclear fuel plates can be successively estimated as composed of the two- and three-phase fields equilibrium indicated on the assessment of the phase relations drawn for samples heat-treated at 400 °C.

  5. Behçet's disease associated with amyloidosis in Turkey and in the world.

    PubMed Central

    Dilşen, N; Koniçe, M; Aral, O; Erbengi, T; Uysal, V; Koçak, N; Ozdogan, E

    1988-01-01

    The association of amyloidosis with Behçet's disease has infrequently been reported in published works. Twenty four such cases have been observed in the world, of which 12 are from Turkey, including eight of ours. In all our eight cases renal biopsy showed amyloidosis of type AA. Behçet's disease of male preponderance, long duration, complete type, multiple organ involvement, and positive skin pathergy test were the main characteristics of all 24 cases of Behçet's disease with amyloidosis. We conclude that amyloidosis associated with Behçet's disease is a secondary AA amyloidosis occurring as an intrinsic manifestation of Behçet's disease. Images PMID:3281606

  6. [18F]-NaF PET/CT imaging in cardiac amyloidosis.

    PubMed

    Van Der Gucht, Axel; Galat, Arnault; Rosso, Jean; Guellich, Aziz; Garot, Jérôme; Bodez, Diane; Plante-Bordeneuve, Violaine; Hittinger, Luc; Dubois-Randé, Jean-Luc; Evangelista, Eva; Sasanelli, Myriam; Chalaye, Julia; Meignan, Michel; Itti, Emmanuel; Damy, Thibaud

    2016-08-01

    Cardiac amyloidosis (CA) is recognized as a common cause of restrictive cardiomyopathy and heart failure due to the deposition of insoluble proteins in the myocardial interstitium. We emphasize the role of [18F]-sodium fluoride (NaF) PET/CT as a potential noninvasive tool to identify and differentiate the transthyretin-related cardiac amyloidosis from the light-chain cardiac amyloidosis. We report cases of a 73-year-old man and a 75-year-old woman followed in our center for congestive heart failure with marked alteration of the left ventricular ejection fraction due to familial transthyretin Val122Ile cardiac amyloidosis and light-chain cardiac amyloidosis, respectively, confirmed on endomyocardial biopsy.

  7. Enthalpies of mixing of liquid systems for lead free soldering: Al-Cu-Sn system.

    PubMed

    Flandorfer, Hans; Rechchach, Meryem; Elmahfoudi, A; Bencze, László; Popovič, Arkadij; Ipser, Herbert

    2011-11-01

    The present work refers to high-temperature drop calorimetric measurements on liquid Al-Cu, Al-Sn, and Al-Cu-Sn alloys. The binary systems have been investigated at 973 K, up to 40 at.% Cu in case of Al-Cu, and over the entire concentrational range in case of Al-Sn. Measurements in the ternary Al-Cu-Sn system were performed along the following cross-sections: x(Al)/x(Cu) = 1:1, x(Al)/x(Sn) = 1:1, x(Cu)/x(Sn) = 7:3, x(Cu)/x(Sn) = 1:1, and x(Cu)/x(Sn) = 3:7 at 1273 K. Experimental data were used to find ternary interaction parameters by applying the Redlich-Kister-Muggianu model for substitutional solutions, and a full set of parameters describing the concentration dependence of the enthalpy of mixing was derived. From these, the isoenthalpy curves were constructed for 1273 K. The ternary system shows an exothermic enthalpy minimum of approx. -18,000 J/mol in the Al-Cu binary and a maximum of approx. 4000 J/mol in the Al-Sn binary system. The Al-Cu-Sn system is characterized by considerable repulsive ternary interactions as shown by the positive ternary interaction parameters.

  8. Interdiffusion and intrinsic diffusion in the NiAl /delta/ phase of the Al-Ni system

    NASA Technical Reports Server (NTRS)

    Shankar, S.; Seigle, L. L.

    1978-01-01

    Interdiffusion coefficients at 950 to 1150 C and the ratio of intrinsic diffusion coefficients at 1100 C were measured as functions of composition in the NiAl (delta) phase of the Al-Ni system, using a vapor-solid technique. Diffusivity values were also obtained for the Ni3Al (epsilon) and Ni (Al) solid solution (zeta) phases from 950 to 1150 C. The interdiffusion coefficient in NiAl (delta) varies several orders of magnitude over the delta phase field with a deep minimum in the diffusivity-composition curve at 48 to 49 at% Al. The ratio of intrinsic diffusion coefficients DNi/DAl, in the delta phase also varies with composition from a value of 3 to 3.5 below 50 at% Al to 0.1 or less above 50 at% Al.

  9. Proteomics and mass spectrometry in the diagnosis of renal amyloidosis

    PubMed Central

    Picken, Maria M.

    2015-01-01

    The amyloidoses are a ‘group’ of disorders, all of which are associated with deposits that display similar staining and ultrastructural features and are toxic to tissues. Many proteins—currently 31 protein types and many more variants—have been shown to undergo such transformations. Among the various currently known amyloidoses, there are marked differences with regard to their pathogenesis and incidence, while the associated clinical picture is frequently overlapping. However, the therapies that are currently available are amyloid-type specific. The diagnosis of amyloidosis thus involves two steps: (i) a generic diagnosis, followed by (ii) an amyloid type-specific diagnosis or ‘amyloid typing’. Immunofluorescence in frozen sections or immunohistochemistry (IHC) in paraffin sections has traditionally been used in the typing of amyloid. However, IHC of amyloid differs significantly from IHC in other areas of surgical pathology; both caution and experience are necessary for its interpretation. The rationale for the application of proteomic methods to amyloid typing lies in the relative abundance of amyloid proteins in tissue where, frequently, it is the ‘dominant’ protein. Proteomic techniques include the following steps: sample preparation, protein extraction and digestion into peptide fragments, followed by their subsequent separation and measurement by mass spectrometry (MS) and protein identification by informatics. The advantages as well as the limitations of both methods—immunohistochemistry and MS-based proteomics—are discussed. The current recommendations for the application of proteomics in renal amyloidosis are summarized. PMID:26613021

  10. Risk factors for venous thromboembolism in immunoglobulin light chain amyloidosis

    PubMed Central

    Bever, Katherine M.; Masha, Luke I.; Sun, Fangui; Stern, Lauren; Havasi, Andrea; Berk, John L.; Sanchorawala, Vaishali; Seldin, David C.; Sloan, J. Mark

    2016-01-01

    Patients with immunoglobulin light chain amyloidosis are at risk for both thrombotic and bleeding complications. While the hemostatic defects have been extensively studied, less is known about thrombotic complications in this disease. This retrospective study examined the frequency of venous thromboembolism in 929 patients with immunoglobulin light chain amyloidosis presenting to a single referral center, correlated risk of venous thromboembolism with clinical and laboratory factors, and examined complications of anticoagulation in this population. Sixty-five patients (7%) were documented as having at least one venous thromboembolic event. Eighty percent of these patients had events within one year prior to or following diagnosis. Lower serum albumin was associated with increased risk of VTE, with a hazard ratio of 4.30 (CI 1.60–11.55; P=0.0038) for serum albumin less than 3 g/dL compared to serum albumin greater than 4 g/dL. Severe bleeding complications were observed in 5 out of 57 patients with venous thromboembolism undergoing treatment with anticoagulation. Prospective investigation should be undertaken to better risk stratify these patients and to determine the optimal strategies for prophylaxis against and management of venous thromboembolism. PMID:26452981

  11. Klystron 'efficiency loop' for the ALS storage ring RF system

    SciTech Connect

    Kwiatkowski, Slawomir; Julian, Jim; Baptiste, Kenneth

    2002-05-20

    The recent energy crisis in California has led us to investigate the high power RF systems at the Advanced Light Source (ALS) in order to decrease the energy consumption and power costs. We found the Storage Ring Klystron Power Amplifier system operating as designed but with significant power waste. A simple proportional-integrator (PI) analog loop, which controls the klystron collector beam current, as a function of the output RF power, has been designed and installed. The design considerations, besides efficiency improvement, were to interface to the existing system without major expense. They were to also avoid the klystron cathode power supply filter's resonance in the loop's dynamics, and prevent a conflict with the existing Cavity RF Amplitude Loop dynamics. This efficiency loop will allow us to save up to 700 MW-hours of electrical energy per year and increase the lifetime of the klystron.

  12. Nomenclature of amyloid and amyloidosis. WHO-IUIS Nomenclature Sub-Committee.

    PubMed Central

    1993-01-01

    This classification of amyloid and amyloidosis is based on the amyloid fibril proteins, followed by a designation of the fibril protein precursor. Additional information includes the protein type or variant (where applicable) and the clinical diagnosis. PMID:8440029

  13. Metastability in the MgAl2O4-Al2O3 System

    DOE PAGESBeta

    Wilkerson, Kelley R.; Smith, Jeffrey D.; Hemrick, James G.

    2014-07-22

    Aluminum oxide must take a spinel form ( γ-Al2O3) at elevated temperatures in order for extensive solid solution to form between MgAl2O4 and α-Al2O3. The solvus line between MgAl2O4 and Al2O3 has been defined at 79.6 wt% Al2O3 at 1500°C, 83.0 wt% Al2O3 at 1600°C, and 86.5 wt% Al2O3 at 1700°C. A metastable region has been defined at temperatures up to 1700°C which could have significant implications for material processing and properties. Additionally, initial processing could have major implications on final chemistry. The spinel solid solution region has been extended to form an infinite solid solution with Al2O3 at elevatedmore » temperatures. A minimum in melting at 1975°C and a chemistry of 96 wt% Al2O3 rather than a eutectic is present, resulting in no eutectic crystal formation during solidification.« less

  14. Very long term studies of the seeding of beta-amyloidosis in primates.

    PubMed

    Ridley, R M; Baker, H F; Windle, C P; Cummings, R M

    2006-09-01

    Cerebral beta-amyloidosis was found in 16/18 marmosets aged <10 yrs and 8/9 marmosets aged >10 yrs, injected intracerebrally with human or marmoset brain homogenate containing beta-amyloid 1-8 years previously. It was found in only 2/12 marmosets aged <10 yrs and 1/15 marmosets aged >10 yrs, injected with synthetic Abeta-peptides, CSF, or brain tissue which did not contain beta-amyloid. Cerebral beta-amyloidosis was found in 0/11 uninjected marmosets aged <10 yrs and in 5/29 uninjected marmosets aged >10 yrs. The beta-amyloidosis comprised small and large vessel angiopathy and some plaques throughout cortex and was qualitatively similar in injected marmosets and, when present, in uninjected marmosets. Of those injected marmosets which were positive, the amount of beta-amyloidosis was unrelated to age or incubation times but the 3 injected marmosets without beta-amyloidosis had incubation times of <3.5 years. We conclude that beta-amyloid, or associated factors, can initiate or accelerate the process of cerebral amyloidosis in primates.

  15. Mechanisms of Islet Amyloidosis Toxicity in Type 2 Diabetes

    PubMed Central

    Abedini, Andisheh; Schmidt, Ann Marie

    2014-01-01

    Amyloid formation by the neuropancreatic hormone, islet amyloid polypeptide (IAPP or amylin), one of the most amyloidogenic sequences known, leads to islet amyloidosis in type 2 diabetes and to islet transplant failure. Under normal conditions, IAPP plays a role in the maintenance of energy homeostasis by regulating several metabolic parameters, such as satiety, blood glucose levels, adiposity and body weight. The mechanisms of IAPP amyloid formation, the nature of IAPP toxic species and the cellular pathways that lead to pancreatic β-cell toxicity are not well characterized. Several mechanisms of toxicity, including receptor and non-receptor-mediated events, have been proposed. Analogs of IAPP have been approved for the treatment of diabetes and are under investigation for the treatment of obesity. PMID:23337872

  16. Measured Activities of Al and Ni in gamma-(Ni) and gamma'-(Ni)3Al in the Ni-Al-Pt System

    NASA Technical Reports Server (NTRS)

    Copland, Evan

    2007-01-01

    Adding Pt to Ni-Al coatings is critical to achieving the required oxidation protection of Ni-based superalloys, but the nature of the Pt effect remains unresolved. This research provides a fundamental part of the answer by measuring the influence of Pt on the activities of Al and Ni in gamma-(Ni), gamma prime-(Ni)3Al and liquid in the Ni-Al-Pt system. Measurements have been made at 25 compositions in the Ni-rich corner over the temperature range, T = 1400-1750 K, by the vapor pressure technique with a multiple effusion-cell mass spectrometer (multi-cell KEMS). These measurements clearly show adding Pt (for X(sub Pt) less than 0.25) decreases a(Al) while increasing a(Ni). This solution behavior supports the idea that Pt increases Al transport to an alloy / Al2O3 interface and also limits the interaction between the coating and substrate alloys in the gamma-(Ni) + gamma prime-(Ni)3Al region. This presentation will review the progress of this study.

  17. Cryptanalysis and improvement of Yan et al.'s biometric-based authentication scheme for telecare medicine information systems.

    PubMed

    Mishra, Dheerendra; Mukhopadhyay, Sourav; Chaturvedi, Ankita; Kumari, Saru; Khan, Muhammad Khurram

    2014-06-01

    Remote user authentication is desirable for a Telecare Medicine Information System (TMIS) for the safety, security and integrity of transmitted data over the public channel. In 2013, Tan presented a biometric based remote user authentication scheme and claimed that his scheme is secure. Recently, Yan et al. demonstrated some drawbacks in Tan's scheme and proposed an improved scheme to erase the drawbacks of Tan's scheme. We analyze Yan et al.'s scheme and identify that their scheme is vulnerable to off-line password guessing attack, and does not protect anonymity. Moreover, in their scheme, login and password change phases are inefficient to identify the correctness of input where inefficiency in password change phase can cause denial of service attack. Further, we design an improved scheme for TMIS with the aim to eliminate the drawbacks of Yan et al.'s scheme. PMID:24880932

  18. Combustion synthesis of ceramic-metal composite materials - The TiC-Al2O3-Al system

    NASA Technical Reports Server (NTRS)

    Feng, H. J.; Moore, John J.; Wirth, D. G.

    1992-01-01

    Combustion synthesis was applied for producing ceramic-metal composites with reduced levels of porosity, by allowing an excess amount of liquid metal, generated by the exothermic reaction during synthesis, to infiltrate the pores. It is shown that this method, when applied to TiC-Al2O3 system, led to a decreased level of porosity in the resulting TiC-Al2O3-Al product, as compared with that of TiC-Al2O3 system. This in situ procedure is more efficient than the two-stage conventional processes (i.e., sintering followed by liquid metal infiltration), although there are limitations with respect to total penetration of the liquid metal and maintaining a stable propagation of the combustion reaction.

  19. Renal leukocyte chemotactic factor 2 (LECT2) amyloidosis in First Nations people in Northern British Columbia, Canada: a report of 4 cases.

    PubMed

    Hutton, Holly L; DeMarco, Mari L; Magil, Alex B; Taylor, Paul

    2014-11-01

    Leukocyte chemotactic factor 2 (LECT2) amyloidosis is a recently identified type of amyloidosis that may represent an underdiagnosed cause of chronic kidney disease. LECT2 amyloidosis typically is reported as being renal limited and, in the United States, more prevalent in Hispanic patients. We add to the epidemiologic data of this condition by describing 4 First Nations people from Northern British Columbia, Canada, who presented with slowly progressive chronic kidney disease that was found to be due to LECT2 amyloidosis.

  20. Lattice crossover and phase transitions in NdAlO3-GdAlO3 system

    NASA Astrophysics Data System (ADS)

    Vasylechko, L.; Shmanko, H.; Ohon, N.; Prots, Yu.; Hoffmann, S.; Ubizskii, S.

    2013-02-01

    Phase and structural behaviour in the (1-x)NdAlO3-xGdAlO3 system in a whole concentration range has been studied by means of in situ high-resolution X-ray synchrotron powder diffraction technique and differential thermal analysis. Two kinds of solid solutions Nd1-xGdxAlO3 have been found at room temperature: one with rhombohedral (x<0.15) and one with orthorhombic (x≥0.20) symmetry. A morphotropic phase transition occurs at x≈0.15, where the co-existence of both phases was observed. Peculiarity of the orthorhombic solid solution is the lattice parameter crossover at the compositions with x=0.33, 0.49 and 0.62. First-order structural transition Pbnm↔R3¯с has been detected both from in situ powder diffraction and thermal analysis data. Continuous phase transformation R3¯с↔Pm3¯m above 2140 K has been predicted for Nd-rich sample Nd0.85Gd0.15AlO3 from the extrapolation of high-temperature behaviour of the lattice parameter ratio of the rhombohedral phase. Based on the experimental data, the phase diagram of the pseudo-binary system NdAlO3-GdAlO3 has been constructed.

  1. Interdiffusion and Intrinsic Diffusion in the Mg-Al System

    SciTech Connect

    Brennan, Sarah; Bermudez, Katrina; Sohn, Yong Ho; Kulkarni, Nagraj S

    2012-01-01

    Solid-to-solid diffusion couples were assembled and annealed to examine the diffusion between pure Mg (99.96%) and Al (99.999%). Diffusion anneals were carried out at 300 , 350 , and 400 C for 720, 360, and 240 hours, respectively. Optical and scanning electron microscopes were utilized to identify the formation of the intermetallic phases, -Al12Mg17 and -Al3Mg2 and absence of the -phase in the diffusion couples. Thicknesses of the -Al12Mg17 and -Al3Mg2 phases were measured and the parabolic growth constants were calculated to determine the activation energies for the growth, 165 and 86 KJ/mole, respectively. Concentration profiles were determined with electron microprobe analysis using pure elemental standards. Composition-dependent interdiffusion coefficients in Mg-solid solution, -Al12Mg17 and - Al3Mg2 and Al-solid solutions were calculated based on the Boltzmann-Matano analysis. Average effective interdiffusion coefficients for each phase were also calculated, and the magnitude was the highest for the -Al3Mg2 phase, followed by -Al12Mg17, Al-solid solution and Mg-solid solution. Intrinsic diffusion coefficients based on Huemann s analysis (e.g., marker plane) were determined for the ~38 at.% Mg in the -Al3Mg2 phase. Activation energies and the pre-exponential factors for the inter- and intrinsic diffusion coefficients were calculated for the temperature range examined. The -Al3Mg2 phase was found to have the lowest activation energies for growth and interdiffusion among all four phases studied. At the marker location in the -Al3Mg2 phase, the intrinsic diffusion of Al was found to be faster than that of Mg. Extrapolations of the impurity diffusion coefficients in the terminal solid solutions were made and compared to the available self- and impurity diffusion data from literature. Thermodynamic factor, tracer diffusion coefficients and atomic mobilities at the marker plane composition were approximated using available literature values of Mg activity in the -Al

  2. Advanced launch system (ALS) actuation and power systems impact operability and cost

    SciTech Connect

    Sundberg, G.R. . Lewis Research Center)

    1990-09-01

    To obtain the advanced launch system (ALS) primary goals of reduced costs ($300/lb earth to LEO) and improved operability, there must be significant reductions in the launch operability, there must be significant reductions in the launch operations and servicing requirements relative to current vehicle designs and practices. One of the primary methods for achieving these goals is by using electrical actuation integrated with a single vehicle electrical power system and controls for all actuation and avionics requirements. This paper reviews the ALS and its associated advanced development program to demonstrate maturation of those technologies that will help meet the overall operability and cost goals. The electric power and actuation systems are highlighted as a specific technology ready not only to meet the ALS goals (cryogenic fuel valves and thrust vector controls with peak power demands to 75 hp), but also those of other launch vehicles, military and civilian aircraft, lunar/Martian vehicles and a multitude of commercial applications.

  3. Advanced Launch System (ALS): Electrical actuation and power systems improve operability and cost picture

    NASA Technical Reports Server (NTRS)

    Sundberg, Gale R.

    1990-01-01

    To obtain the Advanced Launch System (ALS) primary goals of reduced costs and improved operability, there must be significant reductions in the launch operations and servicing requirements relative to current vehicle designs and practices. One of the primary methods for achieving these goals is by using vehicle electrical power system and controls for all actuation and avionics requirements. A brief status review of the ALS and its associated Advanced Development Program is presented to demonstrate maturation of those technologies that will help meet the overall operability and cost goals. The electric power and actuation systems are highlighted as a specific technology ready not only to meet the stringent ALS goals (cryogenic field valves and thrust vector controls with peak power demands to 75 hp), but also those of other launch vehicles, military and civilian aircraft, lunar/Martian vehicles, and a multitude of commercial applications.

  4. Advanced launch system (ALS) - Electrical actuation and power systems improve operability and cost picture

    NASA Technical Reports Server (NTRS)

    Sundberg, Gale R.

    1990-01-01

    To obtain the Advanced Launch System (ALS) primary goals of reduced costs and improved operability, there must be significant reductions in the launch operations and servicing requirements relative to current vehicle designs and practices. One of the primary methods for achieving these goals is by using vehicle electrrical power system and controls for all aviation and avionics requirements. A brief status review of the ALS and its associated Advanced Development Program is presented to demonstrate maturation of those technologies that will help meet the overall operability and cost goals. The electric power and actuation systems are highlighted as a sdpecific technology ready not only to meet the stringent ALS goals (cryogenic field valves and thrust vector controls with peak power demands to 75 hp), but also those of other launch vehicles, military ans civilian aircraft, lunar/Martian vehicles, and a multitude of comercial applications.

  5. Advanced Launch System (ALS) actuation and power systems impact operability and cost

    NASA Technical Reports Server (NTRS)

    Sundberg, Gale R.

    1990-01-01

    To obtain the Advanced Launch System (ALS) primary goals of reduced costs and improved operability, there must be significant reductions in the launch operations and servicing requirements relative to current vehicle designs and practices. One of the primary methods for achieving these goals is by using vehicle electrical power system and controls for all actuation and avionics requirements. A brief status review of the ALS and its associated Advanced Development Program is presented to demonstrate maturation of those technologies that will help meet the overall operability and cost goals. The electric power and actuation systems are highlighted as a specific technology ready not only to meet the stringent ALS goals (cryogenic field valves and thrust vector controls with peak power demands to 75 hp), but also those of other launch vehicles, military and civilian aircraft, lunar/Martian vehicles, and a multitude of commercial applications.

  6. Induction Therapy with Bortezomib Followed by Bortezomib-High Dose Melphalan and Stem Cell Transplantation for Light Chain Amyloidosis: Results of a Prospective Clinical Trial.

    PubMed

    Sanchorawala, Vaishali; Brauneis, Dina; Shelton, Anthony C; Lo, Stephen; Sun, Fangui; Sloan, J Mark; Quillen, Karen; Seldin, David C

    2015-08-01

    The depth of hematologic response has been shown to correlate with survival and organ responses for patients with light chain (AL) amyloidosis. We conducted a prospective trial of 2 cycles of induction with bortezomib and dexamethasone on a twice a week schedule followed by conditioning with bortezomib and high-dose melphalan (HDM) and autologous stem cell transplantation (SCT). The objectives were hematologic responses, tolerability, and survival. Thirty-five patients were enrolled from 2010 to 2013. Of these, 30 proceeded with SCT, whereas 5 did not because of clinical deterioration during induction (n = 3) or complications after stem cell collection (n = 2). Two patients developed features of an autologous graft-versus-host disease-like syndrome post-SCT, which responded to steroids; no other unusual complications were seen. Treatment-related mortality occurred in 8.5% (3/35). Hematologic responses were achieved by 100% of the 27 assessable patients (63% complete response, 37% very good partial response [VGPR]) who completed the planned treatment. By intention-to-treat, hematologic responses occurred in 77% of patients (49% complete response, 29% VGPR). With a median follow-up of 36 months, the median overall survival and progression-free survival were not reached. In conclusion, incorporating bortezomib into induction and conditioning yielded a high rate of hematologic responses after HDM/SCT in patients with AL amyloidosis.

  7. Curcumin: A multi-target disease-modifying agent for late-stage transthyretin amyloidosis

    PubMed Central

    Ferreira, Nelson; Gonçalves, Nádia P.; Saraiva, Maria J.; Almeida, Maria R.

    2016-01-01

    Transthyretin amyloidoses encompass a variety of acquired and hereditary diseases triggered by systemic extracellular accumulation of toxic transthyretin aggregates and fibrils, particularly in the peripheral nervous system. Since transthyretin amyloidoses are typically complex progressive disorders, therapeutic approaches aiming multiple molecular targets simultaneously, might improve therapy efficacy and treatment outcome. In this study, we evaluate the protective effect of physiologically achievable doses of curcumin on the cytotoxicity induced by transthyretin oligomers in vitro by showing reduction of caspase-3 activity and the levels of endoplasmic reticulum-resident chaperone binding immunoglobulin protein. When given to an aged Familial Amyloidotic Polyneuropathy mouse model, curcumin not only reduced transthyretin aggregates deposition and toxicity in both gastrointestinal tract and dorsal root ganglia but also remodeled congophilic amyloid material in tissues. In addition, curcumin enhanced internalization, intracellular transport and degradation of transthyretin oligomers by primary macrophages from aged Familial Amyloidotic Polyneuropathy transgenic mice, suggesting an impaired activation of naïve phagocytic cells exposed to transthyretin toxic intermediate species. Overall, our results clearly support curcumin or optimized derivatives as promising multi-target disease-modifying agent for late-stage transthyretin amyloidosis. PMID:27197872

  8. Restrictive cardiomyopathy in inherited ATTR amyloidosis (TTR-Ser23Asn) in a patient of German-Italian extraction

    PubMed Central

    Mueller, Iris I; Gawaz, Meinrad; Linke, Reinhold P; Zuern, Christine; Steiner, Dagmar; Altland, Klaus; Von Beckerath, Nicolas; Weig, Hans-Joerg

    2010-01-01

    Amyloidosis occurs when certain soluble proteins are transformed into amyloid fibrils in the extracellular space. Most common are the light-chain amyloidoses; less common is the AA-amyloidosis, which follows chronic inflammatory diseases, and the amyloidoses of transthyretin (TTR) origin. We report on a women of Italian-German origin with the mutation TTR (Ser23Asn). Whole body scintigraphy using TC99m-DPD showed end stage hereditary amyloidosis caused by ATTR with predominant tracer retention in the myocardium. Myocardial biopsies revealed the presence of amyloid by Congo red staining. Further immunohistochemical analysis showed ATTR amyloidosis. DNA sequencing revealed a point mutation of the transthyretin gene leading to a single amino acid substitution. The only effective treatment in patients with manifest cardiac ATTR amyloidosis is combined heart and liver transplantation. Our patient was placed on a list for this procedure, but unfortunately she died during the standby procedure due to urosepsis. PMID:22400056

  9. Restrictive cardiomyopathy in inherited ATTR amyloidosis (TTR-Ser23Asn) in a patient of German-Italian extraction.

    PubMed

    Mueller, Iris I; Gawaz, Meinrad; Linke, Reinhold P; Zuern, Christine; Steiner, Dagmar; Altland, Klaus; Von Beckerath, Nicolas; Weig, Hans-Joerg

    2010-01-01

    Amyloidosis occurs when certain soluble proteins are transformed into amyloid fibrils in the extracellular space. Most common are the light-chain amyloidoses; less common is the AA-amyloidosis, which follows chronic inflammatory diseases, and the amyloidoses of transthyretin (TTR) origin. We report on a women of Italian-German origin with the mutation TTR (Ser23Asn). Whole body scintigraphy using TC99m-DPD showed end stage hereditary amyloidosis caused by ATTR with predominant tracer retention in the myocardium. Myocardial biopsies revealed the presence of amyloid by Congo red staining. Further immunohistochemical analysis showed ATTR amyloidosis. DNA sequencing revealed a point mutation of the transthyretin gene leading to a single amino acid substitution. The only effective treatment in patients with manifest cardiac ATTR amyloidosis is combined heart and liver transplantation. Our patient was placed on a list for this procedure, but unfortunately she died during the standby procedure due to urosepsis.

  10. Impaired angiogenesis in a transgenic mouse model of cerebral amyloidosis.

    PubMed

    Paris, Daniel; Patel, Nikunj; DelleDonne, Anthony; Quadros, Amita; Smeed, Robert; Mullan, Michael

    2004-08-01

    Abeta peptides are naturally occurring peptides, which are thought to play a key role in the pathophysiology of Alzheimer's disease (AD). In AD cases, levels of soluble and insoluble Abeta peptides increase in the brain as well as in the cerebrovasculature, a phenomenon that does not occur in extra-cranial vessels. There are frequently anomalies in the cerebrovasculature in AD, and despite increases in several pro-angiogenic factors in AD brain, evidence for increased vascularity is lacking; in fact there is evidence to the contrary. It has also been recently shown that Abeta peptides may have profound anti-angiogenic effects in vitro and in vivo. We therefore investigated whether there is evidence for altered angiogenesis in the vasculature in a transgenic mouse model of Abeta amyloidosis (Tg APPsw line 2576). In vitro, the formation of capillary-like structures on a reconstituted extracellular matrix by endothelial cells isolated from Tg APPsw is impaired. Ex vivo, the sprouting of new capillaries from arterial explants (over expressing Abeta) isolated from 9-month-old Tg APPsw is reduced compared to arterial explants isolated from control littermates. In addition, Tg APPsw mice show a reduction in vascular density in the cortex and hippocampus compared to control littermates. Altogether, our data suggest that the over expression of APPsw in the vasculature may oppose angiogenesis.

  11. Crystalline Al1 - x Ti x phases in the hydrogen cycled NaAlH4 + 0.02TiCl3 system

    NASA Astrophysics Data System (ADS)

    Pitt, M. P.; Vullum, P. E.; Sørby, M. H.; Emerich, H.; Paskevicius, M.; Buckley, C. E.; Gray, E. MacA.; Walmsley, J. C.; Holmestad, R.; Hauback, B. C.

    2013-03-01

    The hydrogen (H) cycled planetary milled (PM) NaAlH4 + 0.02TiCl3 system has been studied by high resolution synchrotron X-ray diffraction and transmission electron microscopy during the first 10 H cycles. After the first H absorption, we observe the formation of four nanoscopic crystalline (c-) Ti-containing phases embedded on the NaAlH4 surface, i.e. Al2Ti, Al3Ti, Al82Ti18 and Al89Ti11, with 100% of the originally added Ti atoms accounted for. Al2Ti and Al3Ti are observed morphologically as a mechanical couple on the NaAlH4 surface, with a moderately strained interface. Electron diffraction shows that the Al82Ti18 phase retains some ordering from the L12 structure type, with the observation of forbidden (100) ordering reflections in the fcc Al82Ti18 lattice. After 2 H cycles the NaAlH4 + 0.02TiCl3 system displays only two crystalline Ti-containing phases, Al3Ti and Al89Ti11. After 10 H cycles, the Al89Ti11 is completely converted to Al85Ti15. Al89Ti11, Al85Ti15 and Al3Ti do not display any ordering reflections, and they are modeled in the A1 structure type. Quantitative phase analysis indicates that the Al3Ti proportion continues to increase with further H cycles. The formation of Ti-poor Al1 - x Ti x (x < 0.25) phases in later H cycles is detrimental to hydrogenation kinetics, compared to the starting Ti-richer near-surface Al2Ti/NaAlH4 interface present during the first absorption of hydrogen.

  12. Transthyretin Cardiac Amyloidosis: Pathogenesis, Treatments, and Emerging Role in Heart Failure with Preserved Ejection Fraction

    PubMed Central

    Ton, Van-Khue; Mukherjee, Monica; Judge, Daniel P

    2014-01-01

    Transthyretin (TTR) amyloidosis causes heart failure from cardiac deposition of TTR amyloid fibrils, the by-product of TTR homotetramer disassembly. Wild-type (WT) TTR deposition leads to senile amyloidosis, predominantly manifesting with cardiomyopathy. Missense mutations in the TTR gene result in familial TTR amyloidosis. Certain mutations are more likely to affect the heart, while others cause more neurologic involvement. Extracellular fibril deposition triggers intracellular stress response, upregulation of the inflammatory cascades, apoptosis, and organ dysfunction. Recent studies suggest that TTR cardiac amyloid may be a significant contributor to the pathogenesis of heart failure with preserved ejection fraction (HFpEF). Summarized in this review are the molecular pathways underlying the cellular toxicity of TTR amyloid fibrils and the emerging therapies aimed at TTR tetramer stabilization, abrogation of TTR synthesis in the liver, or inhibition of amyloidogenesis. PMID:25628512

  13. Secondary amyloidosis in autoinflammatory diseases and the role of inflammation in renal damage

    PubMed Central

    Scarpioni, Roberto; Ricardi, Marco; Albertazzi, Vittorio

    2016-01-01

    The release of proinflammatory cytokines during inflammation represents an attempt to respond to injury, but it may produce detrimental effects. The inflammasome is a large, multiprotein complex that drives proinflammatory cytokine production in response to infection and tissue injury; the best-characterized inflammasome is the nod-like receptor protein-3 (NLRP3). Once activated, inflammasome leads to the active form of caspase-1, the enzyme required for the maturation of interleukin-1beta. Additional mechanisms bringing to renal inflammatory, systemic diseases and fibrotic processes were recently reported, via the activation of the inflammasome that consists of NLRP3, apoptosis associated speck-like protein and caspase-1. Several manuscripts seem to identify NLRP3 inflammasome as a possible therapeutic target in the treatment of progressive chronic kidney disease. Serum amyloid A (SAA), as acute-phase protein with also proinflammatory properties, has been shown to induce the secretion of cathepsin B and inflammasome components from human macrophages. SAA is a well recognised potent activator of the NLRP3. Here we will address our description on the involvement of the kidney in autoinflammatory diseases driven mainly by secondary, or reactive, AA amyloidosis with a particular attention on novel therapeutic approach which has to be addressed in suppressing underlying inflammatory disease and reducing the SAA concentration. PMID:26788465

  14. Germinal centre protein HGAL promotes lymphoid hyperplasia and amyloidosis via BCR-mediated Syk activation

    PubMed Central

    Romero-Camarero, Isabel; Jiang, Xiaoyu; Natkunam, Yasodha; Lu, Xiaoqing; Vicente-Dueñas, Carolina; Gonzalez-Herrero, Ines; Flores, Teresa; Garcia, Juan Luis; McNamara, George; Kunder, Christian; Zhao, Shuchun; Segura, Victor; Fontan, Lorena; Martínez-Climent, Jose A.; García-Criado, Francisco Javier; Theis, Jason D.; Dogan, Ahmet; Campos-Sánchez, Elena; Green, Michael R.; Alizadeh, Ash A.; Cobaleda, Cesar; Sánchez-García, Isidro; Lossos, Izidore S.

    2012-01-01

    The human germinal centre associated lymphoma (HGAL) gene is specifically expressed in germinal centre B-lymphocytes and germinal centre-derived B-cell lymphomas, but its function is largely unknown. Here we demonstrate that HGAL directly binds Syk in B-cells, increases its kinase activity upon B-cell receptor stimulation and leads to enhanced activation of Syk downstream effectors. To further investigate these findings in vivo, HGAL transgenic mice were generated. Starting from 12 months of age these mice developed polyclonal B-cell lymphoid hyperplasia, hypergammaglobulinemia and systemic reactive AA amyloidosis, leading to shortened survival. The lymphoid hyperplasia in the HGAL transgenic mice are likely attributable to enhanced B-cell receptor signalling as shown by increased Syk phosphorylation, ex vivo B-cell proliferation and increased RhoA activation. Overall, our study shows for the first time that the germinal centre protein HGAL regulates B-cell receptor signalling in B-lymphocytes which, without appropriate control, may lead to B-cell lymphoproliferation. PMID:23299888

  15. Abnormal FISH in patients with immunoglobulin light chain amyloidosis is a risk factor for cardiac involvement and for death

    PubMed Central

    Warsame, R; Kumar, S K; Gertz, M A; Lacy, M Q; Buadi, F K; Hayman, S R; Leung, N; Dingli, D; Lust, J A; Ketterling, R P; Lin, Y; Russell, S; Hwa, L; Kapoor, P; Go, R S; Zeldenrust, S R; Kyle, R A; Rajkumar, S V; Dispenzieri, A

    2015-01-01

    Importance of interphase fluorescent in situ hybridization (FISH) with cytoplasmic staining of immunoglobulin FISH (cIg-FISH) on bone marrow is not well understood in light chain amyloidosis (AL). This is in contrast with multiple myeloma where prognostic and treatment related decisions are dependent on cytogenetic testing. This retrospective study reviewed 401 AL patients with cIg-FISH testing performed at our institution between 2004 and 2012. Eighty-one percent of patients had an abnormal cIg-FISH. Common abnormalities involved translocations of chromosome 14q32 (52%), specifically: t(11;14) (43%), t(14;16) (3%) and t(4;14) (2%). Other common abnormalities include monosomy 13/deletion 13q (30%), trisomies 9 (20%), 15 (14%), 11 (10%) and 3 (10%). Median overall survival for this cohort of patients is 3.5 years. When plasma cell burden was greater than 10% trisomies predicted for worse survival (44 vs 19 months), and when it was ⩽10% t(11;14) predicted for worse survival (53 months vs not reached). Abnormal cIg-FISH was significantly associated with advanced cardiac involvement, and remained a prognostic factor on multivariate analysis. This large AL cohort demonstrates that abnormal FISH at diagnosis is prognostic for survival and advanced cardiac disease. Particularly, trisomies and t(11;14) affect survival when degree of plasma cell burden is considered. PMID:25933374

  16. Transthyretin Ile 122: Gene frequency and risk of cardiac amyloidosis in African Americans

    SciTech Connect

    Buxbaum, J. |; Pastore, R.; Yaghoubian, R.

    1994-09-01

    Point mutations in the protein transthyretin (TTR) cause familial amyloidosis involving mainly the heart and peripheral nerves. To determine the TTR Ile 122 gene frequency in African Americans, the allele encoding TTR Ile 122 was identified by PCR-Primer Introduced Restriction Analysis of TTR exon 4 and FokI digestion on 1659 control DNA samples from 1659 African Americans: 64 were heterozygous and one homozygous (allele frequency .02). To determine the risk to gene carriers of developing cardiac amyloidosis, immunohistochemical and genetic studies were performed on amyloid-containing samples identified among 52,370 autopsy samples examined over 32 years. In autopsy samples from patients over age 60, isolated cardiac amyloidosis was more common in African Americans (1.6%) than in White Americans (.40%), suggesting a genetic risk factor. All 29 samples from African Americans were immunohistochemically TTR-positive, and 5 were heterozygous for the TTR Ile 122 allele (17%), as compared with 5/108 heterozygotes (4.6%) among age-matched (over age 60) African American controls without amyloidosis from the same autopsy study (one-tailed P value < .03). Among the 29 amyloid-positive samples were 16 with 1+ or 2+ deposition and 13 with 3+ or 4+ deposition. All 5 TTR Ile 122 heterozygotes had 3+ or 4+ deposition. None of 15 samples from White Americans with cardiac amyloidosis was TTR Ile 122-positive. Extrapolating from the 5/29 samples from which we were able to obtain genetic data, we estimate that 9/54 samples containing cardiac amyloid were TTR Ile 122 heterozygotes. We conclude that heterozygosity for TTR Ile 122 is associated with a greater frequency (relative risk 5.8) and greater severity of cardiac amyloid deposition than in non-carriers of the TTR Ile 122 gene. Of over 40 TTR point mutations associated with amyloidosis described to date, TTR Ile 122 appears to be the most common worldwide and to have the lowest penetrance in causing disease.

  17. Cerebral amyloidosis associated with cognitive decline in autosomal dominant Alzheimer disease

    PubMed Central

    Wang, Fen; Gordon, Brian A.; Ryman, Davis C.; Ma, Shengmei; Xiong, Chengjie; Hassenstab, Jason; Goate, Alison; Fagan, Anne M.; Cairns, Nigel J.; Marcus, Daniel S.; McDade, Eric; Ringman, John M.; Graff-Radford, Neill R.; Ghetti, Bernardino; Farlow, Martin R.; Sperling, Reisa; Salloway, Steve; Schofield, Peter R.; Masters, Colin L.; Martins, Ralph N.; Rossor, Martin N.; Jucker, Mathias; Danek, Adrian; Förster, Stefan; Lane, Christopher A.S.; Morris, John C.; Bateman, Randall J.

    2015-01-01

    Objective: To investigate the associations of cerebral amyloidosis with concurrent cognitive performance and with longitudinal cognitive decline in asymptomatic and symptomatic stages of autosomal dominant Alzheimer disease (ADAD). Methods: Two hundred sixty-three participants enrolled in the Dominantly Inherited Alzheimer Network observational study underwent neuropsychological evaluation as well as PET scans with Pittsburgh compound B. One hundred twenty-one participants completed at least 1 follow-up neuropsychological evaluation. Four composite cognitive measures representing global cognition, episodic memory, language, and working memory were generated using z scores from a battery of 13 standard neuropsychological tests. General linear mixed-effects models were used to investigate the relationship between baseline cerebral amyloidosis and baseline cognitive performance and whether baseline cerebral amyloidosis predicts cognitive change over time (mean follow-up 2.32 years ± 0.92, range 0.89–4.19) after controlling for estimated years from expected symptom onset, APOE ε4 allelic status, and education. Results: In asymptomatic mutation carriers, amyloid burden was not associated with baseline cognitive functioning but was significantly predictive of longitudinal decline in episodic memory. In symptomatic mutation carriers, cerebral amyloidosis was correlated with worse baseline performance in multiple cognitive composites and predicted greater decline over time in global cognition, working memory, and Mini-Mental State Examination. Conclusions: Cerebral amyloidosis predicts longitudinal episodic memory decline in presymptomatic ADAD and multidomain cognitive decline in symptomatic ADAD. These findings imply that amyloidosis in the brain is an indicator of early cognitive decline and provides a useful outcome measure for early assessment and prevention treatment trials. PMID:26245925

  18. Mixed systemic amyloidosis in a patient receiving long term haemodialysis.

    PubMed Central

    Fernandez-Alonso, J; Rios-Camacho, C; Valenzuela-Castaño, A; Hernanz-Mediano, W

    1994-01-01

    A 64 year old woman had been receiving haemodialysis for 11 years. She had a history of chronic renal failure, caused by probable chronic pyelonephritis, and dialysis arthropathy. She died of acute pulmonary oedema and haemorrhage. At necropsy, histological, immunohistochemical, and ultrastructural studies showed widespread visceral deposits of beta 2-microglobulin (beta 2-M) and AA amyloid. Images PMID:8063944

  19. Immunoparesis status in immunoglobulin light chain amyloidosis at diagnosis affects response and survival by regimen type

    PubMed Central

    Muchtar, Eli; Dispenzieri, Angela; Kumar, Shaji K.; Dingli, David; Lacy, Martha Q.; Buadi, Francis K.; Hayman, Suzanne R.; Kapoor, Prashant; Leung, Nelson; Chakraborty, Rajshekhar; Russell, Stephen; Lust, John A.; Lin, Yi; Go, Ronald S.; Zeldenrust, Steven; Kyle, Robert A.; Rajkumar, S. Vincent; Gertz, Morie A.

    2016-01-01

    Clinical tools to guide in the appropriate treatment selection in immunoglobulin light chain (AL) amyloidosis are not well developed. We evaluated the response and outcome for various regimens at first-line treatment (n=681) and first progression (n=240) stratified by the immunoparesis status at diagnosis. Immunoparesis was assessed by the average relative difference of the uninvolved immunoglobulins, classifying patients into a negative average relative difference (i.e. significant immunoparesis) or a positive average relative difference (no/modest immunoparesis). Treatment was categorized as autologous stem cell transplant and four non-transplant regimens (melphalan-based; bortezomib-based, immunomodulatory drug-based and dexamethasone alone). Patients with significant immunoparesis who underwent stem cell transplant had a significantly lower rate of very good partial response or better response (58%), progression-free survival (median 30 months) and overall survival (108 months), compared to those without significant immunoparesis (80%, 127 months, median not reached, respectively; P<0.001 for all comparisons). Among the non-transplant regimens, melphalan resulted in an unfavorable progression-free survival (11 vs. 27 months; P<0.001) and overall survival (30 vs. 74 months; P=0.001) in patients with significant immunoparesis compared to those without significant immunoparesis. In contrast, no significant difference in outcomes between the immunoparesis groups was seen for those treated with bortezomib or immunomodulatory drugs. At first progression, immunoparesis status did not impact response or survival of any regimen. Melphalan at first-line provided poorer outcomes for patients with significant immunoparesis, while bortezomib or immunomodulatory drugs were more likely to overcome the adverse prognosis associated with significant immunoparesis. PMID:27479823

  20. Vapor Pressures in the Al(I)+Al2O3(s) System: Reconsidering Al2O3(s) Condensation

    NASA Technical Reports Server (NTRS)

    Copland, Evan

    2005-01-01

    The vaporization behavior of the A1-O system has been studied on numerous occasions but significant uncertainties remain. The origin of this uncertainty must be understood before A1-O vaporization behavior can be accurately determined. The condensation of A12O3 and clogging of the effusion orifice is a difficult problem for the Knudsen effusion technique that influences the measured vaporization behavior but has only received limited attention. This study reconsiders this behavior in detail. A new theory for A12O3 condensation is proposed together with procedures that will improve the measured thermodynamic properties of A1-O vaporization.

  1. Clasmatodendrosis and β-amyloidosis in aging hippocampus.

    PubMed

    Mercatelli, Raffaella; Lana, Daniele; Bucciantini, Monica; Giovannini, Maria Grazia; Cerbai, Francesca; Quercioli, Franco; Zecchi-Orlandini, Sandra; Delfino, Giovanni; Wenk, Gary L; Nosi, Daniele

    2016-04-01

    Alterations of the tightly interwoven neuron/astrocyte interactions are frequent traits of aging, but also favor neurodegenerative diseases, such as Alzheimer disease (AD). These alterations reflect impairments of the innate responses to inflammation-related processes, such as β-amyloid (Aβ) burdening. Multidisciplinary studies, spanning from the tissue to the molecular level, are needed to assess how neuron/astrocyte interactions are influenced by aging. Our study addressed this requirement by joining fluorescence-lifetime imaging microscopy/phasor multiphoton analysis with confocal microscopy, implemented with a novel method to separate spectrally overlapped immunofluorescence and Aβ autofluorescence. By comparing data from young control rats, chronically inflamed rats, and old rats, we identified age-specific alterations of neuron/astrocyte interactions in the hippocampus. We found a correlation between Aβ aggregation (+300 and +800% of aggregated Aβ peptide in chronically inflamed and oldvs.control rats, respectively) and fragmentation (clasmatodendrosis) of astrocyte projections (APJs) (+250 and +1300% of APJ fragments in chronically inflamed and oldvs.control rats, respectively). Clasmatodendrosis, in aged rats, associates with impairment of astrocyte-mediated Aβ clearance (-45% of Aβ deposits on APJs, and +33% of Aβ deposits on neurons in oldvs.chronically inflamed rats). Furthermore, APJ fragments colocalize with Aβ deposits and are involved in novel Aβ-mediated adhesions between neurons. These data define the effects of Aβ deposition on astrocyte/neuron interactions as a key topic in AD biology.-Mercatelli, R., Lana, D., Bucciantini, M., Giovannini, M. G., Cerbai, F., Quercioli, F., Zecchi-Orlandini, S., Delfino, G., Wenk, G. L., Nos, D. Clasmatodendrosis and β-amyloidosis in aging hippocampus. PMID:26722005

  2. Primary Amyloidosis of Celiac/Para-Pancreatic Lymph Nodes Diagnosed by Endosonography-Guided Fine Needle Aspiration: A Case Report.

    PubMed

    Akbar, Nuralhuda; Kubbara, Aahd; Nawras, Ali

    2015-01-01

    Introduction. Primary amyloidosis is a disorder resulting from the deposition of fibrillary protein in extracellular tissue. Diagnosis of primary amyloidosis in the celiac/para-pancreatic lymph nodes via endoscopic ultrasound-guided fine needle aspiration has not been reported in the literature. In this article, we report our first observation. Our patient is a 64-year-old Caucasian man who was referred to our institution from an outlying hospital for recurrent abdominal pain. Radiological imaging revealed an enlarged abdominal lymph node that was already biopsied under computed tomography needle guidance but diagnosis was not achieved on pathological examination. At our institution, endoscopic ultrasound-guided fine needle aspiration showed enlarged para-celiac/pancreatic lymph nodes. Endosonography-guided fine needle aspiration revealed the diagnosis of primary amyloidosis. The patient tolerated the procedure well with follow-up as an outpatient. Conclusions. Lymph node involvement in amyloidosis is not uncommon. However, the involvement of the pancreatic/celiac lymph nodes by amyloidosis is obscure in this case. This case shows a rare presentation of amyloidosis diagnosed for the first time by the technique of endosonography-guided fine needle aspiration. In the future, this might serve as an establishment to standardize diagnosing abdominal lymph node amyloidosis, once suspected, by endosonography-guided fine needle aspiration. PMID:26904706

  3. Phase equilibria in the Ni-Al-Ti system at 1173 k

    NASA Astrophysics Data System (ADS)

    Nash, P.; Liang, W. W.

    1985-03-01

    The phase equilibria at 1173 K have been determined in the Ni-AI-Ti system for Al contents less than 50 at. pct. The extent of the H (Ni2AlTi) phase field has been established as well as the extent of solubility in the binary compounds γ (Ni3Al), ν(Ni3Ti), β2(NiTi), NiTi2, and ζ(AlTi3). Substantial differences were found between the phase equilibria determined in this study and previous studies, in part due to the large solubility of Al in NiTi2.

  4. Renal AH Amyloidosis Associated With a Truncated Immunoglobulin Heavy Chain Undetectable by Immunostaining.

    PubMed

    Manabe, Shun; Hatano, Michiyasu; Yazaki, Masahide; Nitta, Kosaku; Nagata, Michio

    2015-12-01

    AH amyloidosis is a rare type of amyloidosis caused by deposition of monoclonal immunoglobulin heavy chain. The key diagnostic feature is positive immunostaining for a single class of immunoglobulin heavy chain. We report a case of AH amyloidosis with immunoglobulin G (IgG) λ monoclonal gammopathy that was diagnosed by liquid chromatography-tandem mass spectrometry (LC-MS/MS) after immunostaining of renal tissue for immunoglobulin heavy chain gave negative results. The molecular weight of the purified renal amyloid protein was ∼11kDa, which was determined by LC-MS/MS analysis to correspond to an amino acid sequence comprising the variable region and a truncated portion of the constant region of IgG heavy chain. The exact same truncated heavy chain was detected by LC-MS/MS of a protein isolated from the patient's serum, suggesting that the truncated serum protein was the precursor of the amyloid protein. Because antibodies to immunoglobulin heavy chain recognize the Fc portion, the large deletion in the constant region could explain the negative results upon immunostaining. Direct protein detection by LC-MS/MS is a powerful aid to diagnose renal AH amyloidosis, particularly when the findings of immunoglobulin staining are inconsistent with the background monoclonal gammopathy.

  5. Benefit of doxycycline treatment on articular disability caused by dialysis related amyloidosis.

    PubMed

    Montagna, Giovanni; Cazzulani, Benedetta; Obici, Laura; Uggetti, Carla; Giorgetti, Sofia; Porcari, Riccardo; Ruggiero, Rubina; Mangione, P Patrizia; Brambilla, Moreno; Lucchetti, Jacopo; Guiso, Giovanna; Gobbi, Marco; Merlini, Giampaolo; Salmona, Mario; Stoppini, Monica; Villa, Giuseppe; Bellotti, Vittorio

    2013-09-01

    Abstract Doxycycline inhibits amyloid formation in vitro and its therapeutic efficacy is under evaluation in clinical trials for different protein conformational diseases, including prion diseases, Alzheimer's disease and transthyretin amyloidosis. In patients on chronic hemodialysis, a persistently high concentration of β2-microglobulin causes a form of amyloidosis (dialysis-related amyloidosis, DRA) localized in bones and ligaments. Since doxycycline inhibits β2-microglobulin fibrillogenesis in vitro and accumulates in bones, DRA represents an ideal form of amyloidosis where doxycycline may reach a therapeutic concentration at the site of amyloid deposition. Three patients on long-term dialysis with severe articular impairment and uncontrollable pain due to DRA were treated with 100 mg of doxycycline daily. Pharmacokinetics and safety of treatment were conducted. Plasmatic levels of the drug reached a plateau after one week (1.1-2.3 µg/ml). Treatment was well tolerated in two patients for a year, while one was suspended after 5 months due to mild esophagitis. Treatment was associated with a significant reduction in articular pain and with a significant and measurable improvement in passive and active movements in all cases, despite the persistence of unchanged amyloid deposits measured by magnetic resonance imaging. PMID:23734692

  6. Intestinal pseudo-obstruction due to amyloidosis of the colon in association with an intestinal plasmacytoma.

    PubMed Central

    Nicholl, D.; Jones, T.

    1991-01-01

    A case of large bowel pseudo-obstruction due to colonic amyloidosis associated with an intestinal plasmacytoma is described. The association of an intestinal plasmacytoma with massive local amyloid deposition has not to our knowledge been previously reported. Images Figure 1 Figure 2 PMID:1800969

  7. Sensitivity of technetium-99m-pyrophosphate scintigraphy in diagnosing cardiac amyloidosis

    SciTech Connect

    Falk, R.H.; Lee, V.W.; Rubinow, A.; Hood, W.B. Jr.; Cohen, A.S.

    1983-03-01

    To determine the value of technetium-99m-pyrophosphate myocardial scintigraphy in the diagnosis of amyloid heart disease this procedure was prospectively performed in 20 consecutive patients with biopsy-proven primary amyloidosis. Eleven patients had echocardiographic abnormalities compatible with amyloid cardiomyopathy, 9 of whom had congestive heart failure. Diffuse myocardial pyrophosphate uptake was of equal or greater intensity than that of the ribs in 9 of the 11 patients with echocardiograms suggestive of amyloidosis, but in only 2 of the 9 with normal echocardiograms, despite abnormal electrocardiograms (p less than 0.01). Increased wall thickness measured by M-mode echocardiography correlated with myocardial pyrophosphate uptake (r . 0.68, p less than 0.01). None of 10 control patients with nonamyloid, nonischemic heart disease had a strongly positive myocardial pyrophosphate uptake. Thus, myocardial technetium-99m-pyrophosphate scanning is a sensitive and specific test for the diagnosis of cardiac amyloidosis in patients with congestive heart failure of obscure origin. It does not appear to be of value for the early detection of cardiac involvement in patients with known primary amyloidosis without echocardiographic abnormalities.

  8. Irish (Donegal) amyloidosis is associated with the transthyretinALA60 (Appalachian) variant.

    PubMed

    Staunton, H; Davis, M B; Guiloff, R J; Nakazato, M; Miyazato, N; Harding, A E

    1991-12-01

    A cluster of cases of late onset amyloidosis, presenting chiefly with peripheral neuropathy and cardiac failure, has been described in Donegal, north-west Ireland. Molecular genetic and protein analyses show that two new patients from the same region have a mutation in the transthyretin gene previously reported in a family with an Irish ancestor from the Appalachian region of the United States.

  9. Analysis of the modifying effects of SAA1, SAA2 and TNF-alpha gene polymorphisms on development of amyloidosis in FMF patients.

    PubMed

    Yilmaz, Engin; Balci, Banu; Kutlay, Sim; Ozen, Seza; Ertürk, Sensuvar; Oner, Ayse; Beşbaş, Nesrin; Bakkaloğlu, Ayşin

    2003-01-01

    The aim of this study was to examine whether polymorphisms at serum amyloid A (SAA) and tumor necrosis factor-alpha (TNF-alpha) genes are associated with development of amyloidosis in familial Mediterranean fever (FMF) patients. Seventy-three FMF patients with amyloidosis and 100 other FMF patients without amyloidosis of known genotypes and 100 healthy control subjects were analyzed. There was a significant difference in the frequency of alpha/alpha genotype at the SAA1 locus between FMF patients with amyloidosis and controls and FMF patients without amyloidosis. The frequencies of the alpha/alpha genotype and alpha alleles at SAA1 locus were significantly higher in the FMF patients with amyloidosis. The frequencies of the alpha allele at SAA1 locus in FMF patients with amyloidosis, without amyloidosis and controls were 85.6%, 49.5% and 42.5%, respectively. We demonstrated that alpha/alpha genotype at SAA1 gene might have modifying effects on the development of amyloidosis. Determination of genotypes at SAA1 locus can play a key role in conferring genetic susceptibility and patient's prognosis to renal amyloidosis.

  10. Clinical, biopsy, and mass spectrometry characteristics of renal apolipoprotein A-IV amyloidosis.

    PubMed

    Dasari, Surendra; Amin, Md Shahrier; Kurtin, Paul J; Vrana, Julie A; Theis, Jason D; Grogg, Karen L; Alexander, Mariam P; Nasr, Samih H; Fervenza, Fernando C; Leung, Nelson; Sethi, Sanjeev

    2016-09-01

    Apolipoprotein A-IV associated amyloidosis (AApoAIV amyloidosis) is a rare cause of amyloidosis with only a single reported case. Here we describe the clinical, biopsy, and mass spectrometry characteristics of 11 cases of renal AApoAIV amyloidosis encompassing 9 men and 2 women with a mean age at diagnosis of 63.5 years. Progressive chronic kidney disease (mean serum creatinine 2.9 mg/dl) was the most common cause for biopsy with proteinuria absent or minimal in all except one. Hematological and serological evaluation was negative in 9 patients, while 2 had a monoclonal gammopathy. The renal biopsy findings were striking and showed large amounts of eosinophilic Congo-red positive amyloid deposits restricted to the renal medulla with sparing of the renal cortex. In 6 cases, peritubular amyloid was noted in addition to the interstitial involvement. Immunofluorescence studies were negative for immunoglobulins. Electron microscopy showed nonbranching fibrils measuring 7 to 10 nm in diameter. Laser microdissection of the amyloid deposits followed by mass spectrometry showed large spectra number (a semiquantitative measure of abundance) for AApoAIV protein ranging from 49 to 169 (average 85), serum amyloid protein (average 19), and apolipoprotein E (average 48). Importantly, no peptides were detected for any other forms of known amyloidogenic precursor proteins. Thus, renal AApoAIV amyloidosis typically presents with progressive chronic kidney disease and histologically exhibits extensive medullary involvement with sparing of the cortex. The diagnosis is best established by mass spectrometry. Hence, a high degree of suspicion and examination of the renal medulla is required to make the diagnosis. PMID:27262366

  11. Renal amyloidosis followed more than 5 years: report of 12 cases.

    PubMed

    Kaaroud, H; Boubaker, K; Béji, S; Abderrahim, E; Moussa, F Ben; Turki, S; Goucha, R; Hedri, H; El Younsi, F; Kheder, A; Maiz, H Ben

    2004-01-01

    Renal involvement with amyloidosis is common but causes patient survival to be poor, rarely reaching 5 years. In this study, we retrospectively reviewed clinical and biological characteristics as well as treatments and outcomes of patients with renal amyloidosis followed for more than 5 years. Between 1975 and 2003, 485 patients were diagnosed with renal amyloidosis including only 12 patients who were followed more than 5 years. The six men and six women of mean age 42.4 years (range 18 to 66 years) displayed renal signs of lower limb edema in all cases; hypertension in four cases, proteinuria on urinalysis in all cases with microscopic hematuria in five cases. Biological tests showed nephrotic syndrome in 11 patients, normal renal function in nine patients, and renal failure in three patients whose mean creatinine was 481.6 micromol/L (range 294 to 726). The amyloidosis was AA type in 11 cases and non-AA in one case. An etiologic survey revealed spondylarthropathy in one patient, pulmonary tuberculosis in two patients, chronic bronchitis in three patients, hepatic hydatic cyst in one patient, Mediterranean familial fever in two patients, Crohn's disease in one patient, Hodgkin's lymphoma in one patient, and multiple myeloma in one patient. Specific treatment was initiated with colchicine in seven patients. At a 110-month mean follow-up (range 53 to 153 months), remission of nephrotic syndrome was observed in four cases, progression to chronic renal failure in two patients, and to end-stage renal failure in five cases (range 53 to 196 months), with stabilization of renal function in seven patients. In conclusion, primary amyloid disease should be optimally suppressed in patients with renal involvement. The role of this treatment in remission of renal amyloidosis is not well established. This efficacy of the treatment has been demonstrated in some patients with improved survival.

  12. Formation enthalpies of Al-Fe-Zr-Nd system calculated by using geometric and Miedema's models

    NASA Astrophysics Data System (ADS)

    Zhang, Lei; Wang, Rongcheng; Tao, Xiaoma; Guo, Hui; Chen, Hongmei; Ouyang, Yifang

    2015-04-01

    Formation enthalpy is important for the phase stability and amorphous forming ability of alloys. The formation enthalpies of Fe17RE2 (RE=Ce, Pr, Nd, Gd and Er) obtained by Miedema's theory are in good agreement with those of the experiments. The dependence of formation enthalpy on concentration of Al for intermetallic (AlxFe1-x)17Nd2 have been calculated by Miedema's theory and the geometric model. The solid solubility of Al in (AlxFe1-x)17Nd2 is coincident with the concentration dependence of formation enthalpy. The mixing enthalpies of liquid alloys and formation enthalpies of alloys for Al-Fe-Zr-Nd system have been predicted. The calculated mixing enthalpy indicates that the adding of Fe or Nd decreases monotonously the magnitude of enthalpy. The formation enthalpies of Al-Fe-Zr-Nd system indicate that the shape of the enthalpy contour map changes when the content of Al is less than 50.0 at% and then it remains unchanged except the decrease of magnitude. The formation enthalpy of Al-Fe-Zr-Nd increases with the increase of Fe and/or Nd content. The negative formation enthalpy indicates that Al-Fe-Zr-Nd system has higher amorphous forming ability and wide amorphous forming range. The certain contents of Zr and/or Al are beneficial for the formation of Al-Fe-Zr-Nd intermetallics.

  13. Prevalence and organ distribution of leukocyte chemotactic factor 2 amyloidosis (ALECT2) among decedents in New Mexico

    PubMed Central

    Larsen, Christopher P.; Beggs, Marjorie L.; Wilson, Jon D.; Lathrop, Sarah L.

    2016-01-01

    Abstract Leukocyte chemotactic factor 2 (LECT2) amyloidosis is one of the most recently described types of amyloidosis. Since its description, it has been found to be one the most common types of amyloidosis in large series of amyloid cases involving the kidney and liver in the United States, where it primarily affects patients of Hispanic ethnicity. We sought to investigate the prevalence of this disease among Hispanic adult decedents who had an autopsy performed at the New Mexico Office of the Medical Investigator and determine the organ distribution of amyloid deposition. LECT2 amyloid deposits were identified within the kidney in 3.1% of Hispanic decedents. It was consistently deposited in the liver, spleen, adrenals, and lungs but did not involve the myocardium or brain. LECT2 amyloidosis is likely not rare among Hispanics in the Southwest United States and could represent an important but under-recognized etiology of chronic kidney disease in this population. PMID:26912093

  14. Reversible pathologic and cognitive phenotypes in an inducible model of Alzheimer-amyloidosis

    PubMed Central

    Melnikova, Tatiana; Fromholt, Susan; Kim, HyunSu; Lee, Deidre; Xu, Guilian; Price, Ashleigh; Moore, Brenda D.; Golde, Todd E.; Felsenstein, Kevin M.; Savonenko, Alena; Borchelt, David R.

    2013-01-01

    Transgenic mice that express mutant amyloid precursor protein (APPsi) using tet-Off vector systems provide an alternative model for assessing short- and long-term effects of Aβ-targeting therapies on phenotypes related to the deposition of Alzheimer-type amyloid. Here we use such a model, termed APPsi:tTA, to determine what phenotypes persist in mice with high amyloid burden after new production of APP/Aβ has been suppressed. We find that 12-13 month old APPsi:tTA mice are impaired in cognitive tasks that assess short- and long-term memories. Acutely suppressing new APPsi/Aβ production produced highly significant improvements in performance short-term spatial memory tasks; which upon continued suppression translated to superior performance in more demanding tasks that assess long-term spatial memory and working memory. Deficits in episodic-like memory and cognitive flexibility, however, were more persistent. Arresting mutant APPsi production caused a rapid decline in the brain levels of soluble APP ectodomains, full-length APP, and APP C-terminal fragments. As expected, amyloid deposits persisted after new APP/Aβ production was inhibited whereas, unexpectedly, we detected persistent pools of solubilizable, relatively mobile, Aβ42. Additionally, we observed persistent levels of Aβ immunoreactive entities that were of a size consistent with SDS-resistant oligomeric assemblies. Thus, in this model with significant amyloid pathology, a rapid amelioration of cognitive deficits was observed despite persistent levels of oligomeric Aβ assemblies and low, but detectable solubilizable Aβ42 peptides. These findings implicate complex relationships between accumulating Aβ and activities of APP, soluble APP ectodomains, and/or APP CTFs in mediating cognitive deficits in this model of amyloidosis. PMID:23447589

  15. Reversible pathologic and cognitive phenotypes in an inducible model of Alzheimer-amyloidosis.

    PubMed

    Melnikova, Tatiana; Fromholt, Susan; Kim, HyunSu; Lee, Deidre; Xu, Guilian; Price, Ashleigh; Moore, Brenda D; Golde, Todd E; Felsenstein, Kevin M; Savonenko, Alena; Borchelt, David R

    2013-02-27

    Transgenic mice that express mutant amyloid precursor protein (APPsi) using tet-Off vector systems provide an alternative model for assessing short- and long-term effects of Aβ-targeting therapies on phenotypes related to the deposition of Alzheimer-type amyloid. Here we use such a model, termed APPsi:tTA, to determine what phenotypes persist in mice with high amyloid burden after new production of APP/Aβ has been suppressed. We find that 12- to 13-month-old APPsi:tTA mice are impaired in cognitive tasks that assess short- and long-term memories. Acutely suppressing new APPsi/Aβ production produced highly significant improvements in performing short-term spatial memory tasks, which upon continued suppression translated to superior performance in more demanding tasks that assess long-term spatial memory and working memory. Deficits in episodic-like memory and cognitive flexibility, however, were more persistent. Arresting mutant APPsi production caused a rapid decline in the brain levels of soluble APP ectodomains, full-length APP, and APP C-terminal fragments. As expected, amyloid deposits persisted after new APP/Aβ production was inhibited, whereas, unexpectedly, we detected persistent pools of solubilizable, relatively mobile, Aβ42. Additionally, we observed persistent levels of Aβ-immunoreactive entities that were of a size consistent with SDS-resistant oligomeric assemblies. Thus, in this model with significant amyloid pathology, a rapid amelioration of cognitive deficits was observed despite persistent levels of oligomeric Aβ assemblies and low, but detectable solubilizable Aβ42 peptides. These findings implicate complex relationships between accumulating Aβ and activities of APP, soluble APP ectodomains, and/or APP C-terminal fragments in mediating cognitive deficits in this model of amyloidosis.

  16. Heterogeneous Distribution of 26Al at the Birth of the Solar System

    NASA Astrophysics Data System (ADS)

    Makide, Kentaro; Nagashima, Kazuhide; Krot, Alexander N.; Huss, Gary R.; Ciesla, Fred J.; Hellebrand, Eric; Gaidos, Eric; Yang, Le

    2011-06-01

    It is believed that 26Al, a short-lived (t 1/2 = 0.73 Ma) and now extinct radionuclide, was uniformly distributed in the nascent solar system (SS) with the initial 26Al/27Al ratio of ~5.2 × 10-5, suggesting an external, stellar origin rather than local, solar source. However, the stellar source of 26Al and the manner in which it was injected into the SS remain controversial: the 26Al could have been produced by an asymptotic giant branch star, a supernova, or a Wolf-Rayet star and injected either into the protosolar molecular cloud, protosolar cloud core, or protoplanetary disk. Corundum (Al2O3) is predicted to be the first condensate from a cooling gas of solar composition. Here we show that micron-sized corundum condensates from 16O-rich (Δ17O ~ -25‰) gas of solar composition recorded heterogeneous distribution of 26Al at the birth of the SS: the inferred initial 26Al/27Al ratio ranges from ~6.5×10-5 to <2×10-6 52% of corundum grains measured are 26Al-poor. Abundant 26Al-poor, 16O-rich refractory objects include grossite- and hibonite-rich calcium-aluminum-rich inclusions (CAIs) in CH (high metal abundance and high iron concentration) chondrites, platy hibonite crystals in CM (Mighei-like) chondrites, and CAIs with fractionation and unidentified nuclear effects CAIs chondrites. Considering the apparently early and short duration (<0.3 Ma) of condensation of refractory 16O-rich solids in the SS, we infer that 26Al was injected into the collapsing protosolar molecular cloud and later homogenized in the protoplanetary disk. The apparent lack of correlation between 26Al abundance and O-isotope composition of corundum grains constrains the stellar source of 26Al in the SS.

  17. Comparative evaluation of p5+14 with SAP and peptide p5 by dual-energy SPECT imaging of mice with AA amyloidosis

    PubMed Central

    Martin, Emily B.; Williams, Angela; Richey, Tina; Stuckey, Alan; Heidel, R. Eric; Kennel, Stephen J.; Wall, Jonathan S.

    2016-01-01

    Amyloidosis is a protein-misfolding disorder characterized by the extracellular deposition of amyloid, a complex matrix composed of protein fibrils, hyper-sulphated glycosaminoglycans and serum amyloid P component (SAP). Accumulation of amyloid in visceral organs results in the destruction of tissue architecture leading to organ dysfunction and failure. Early differential diagnosis and disease monitoring are critical for improving patient outcomes; thus, whole body amyloid imaging would be beneficial in this regard. Non-invasive molecular imaging of systemic amyloid is performed in Europe by using iodine-123-labelled SAP; however, this tracer is not available in the US. Therefore, we evaluated synthetic, poly-basic peptides, designated p5 and p5+14, as alternative radiotracers for detecting systemic amyloidosis. Herein, we perform a comparative effectiveness evaluation of radiolabelled peptide p5+14 with p5 and SAP, in amyloid-laden mice, using dual-energy SPECT imaging and tissue biodistribution measurements. All three radiotracers selectively bound amyloid in vivo; however, p5+14 was significantly more effective as compared to p5 in certain organs. Moreover, SAP bound principally to hepatosplenic amyloid, whereas p5+14 was broadly distributed in numerous amyloid-laden anatomic sites, including the spleen, liver, pancreas, intestines and heart. These data support clinical validation of p5+14 as an amyloid radiotracer for patients in the US. PMID:26936002

  18. Comparative evaluation of p5+14 with SAP and peptide p5 by dual-energy SPECT imaging of mice with AA amyloidosis.

    PubMed

    Martin, Emily B; Williams, Angela; Richey, Tina; Stuckey, Alan; Heidel, R Eric; Kennel, Stephen J; Wall, Jonathan S

    2016-03-03

    Amyloidosis is a protein-misfolding disorder characterized by the extracellular deposition of amyloid, a complex matrix composed of protein fibrils, hyper-sulphated glycosaminoglycans and serum amyloid P component (SAP). Accumulation of amyloid in visceral organs results in the destruction of tissue architecture leading to organ dysfunction and failure. Early differential diagnosis and disease monitoring are critical for improving patient outcomes; thus, whole body amyloid imaging would be beneficial in this regard. Non-invasive molecular imaging of systemic amyloid is performed in Europe by using iodine-123-labelled SAP; however, this tracer is not available in the US. Therefore, we evaluated synthetic, poly-basic peptides, designated p5 and p5+14, as alternative radiotracers for detecting systemic amyloidosis. Herein, we perform a comparative effectiveness evaluation of radiolabelled peptide p5+14 with p5 and SAP, in amyloid-laden mice, using dual-energy SPECT imaging and tissue biodistribution measurements. All three radiotracers selectively bound amyloid in vivo; however, p5+14 was significantly more effective as compared to p5 in certain organs. Moreover, SAP bound principally to hepatosplenic amyloid, whereas p5+14 was broadly distributed in numerous amyloid-laden anatomic sites, including the spleen, liver, pancreas, intestines and heart. These data support clinical validation of p5+14 as an amyloid radiotracer for patients in the US.

  19. Obesity-induced chronic inflammation in high fat diet challenged C57BL/6J mice is associated with acceleration of age-dependent renal amyloidosis.

    PubMed

    van der Heijden, Roel A; Bijzet, Johan; Meijers, Wouter C; Yakala, Gopala K; Kleemann, Robert; Nguyen, Tri Q; de Boer, Rudolf A; Schalkwijk, Casper G; Hazenberg, Bouke P C; Tietge, Uwe J F; Heeringa, Peter

    2015-11-13

    Obesity-induced inflammation presumably accelerates the development of chronic kidney diseases. However, little is known about the sequence of these inflammatory events and their contribution to renal pathology. We investigated the effects of obesity on the evolution of age-dependent renal complications in mice in conjunction with the development of renal and systemic low-grade inflammation (LGI). C57BL/6J mice susceptible to develop age-dependent sclerotic pathologies with amyloid features in the kidney, were fed low (10% lard) or high-fat diets (45% lard) for 24, 40 and 52 weeks. HFD-feeding induced overt adiposity, altered lipid and insulin homeostasis, increased systemic LGI and adipokine release. HFD-feeding also caused renal upregulation of pro-inflammatory genes, infiltrating macrophages, collagen I protein, increased urinary albumin and NGAL levels. HFD-feeding severely aggravated age-dependent structural changes in the kidney. Remarkably, enhanced amyloid deposition rather than sclerosis was observed. The degree of amyloidosis correlated significantly with body weight. Amyloid deposits stained positive for serum amyloid A (SAA) whose plasma levels were chronically elevated in HFD mice. Our data indicate obesity-induced chronic inflammation as a risk factor for the acceleration of age-dependent renal amyloidosis and functional impairment in mice, and suggest that obesity-enhanced chronic secretion of SAA may be the driving factor behind this process.

  20. Power Systems for Future Missions: Appendices A-L

    NASA Technical Reports Server (NTRS)

    Gill, S. P.; Frye, P. E.; Littman, Franklin D.; Meisl, C. J.

    1994-01-01

    Selection of power system technology for space applications is typically based on mass, readiness of a particular technology to meet specific mission requirements, and life cycle costs (LCC). The LCC is typically used as a discriminator between competing technologies for a single mission application. All other future applications for a given technology are usually ignored. As a result, development cost of a technology becomes a dominant factor in the LCC comparison. Therefore, it is common for technologies such as DIPS and LMR-CBC to be potentially applicable to a wide range of missions and still lose out in the initial LCC comparison due to high development costs. This collection of appendices (A through L) contains the following power systems technology plans: CBC DIPS Technology Roadmap; PEM PFC Technology Roadmap; NAS Battery Technology Roadmap; PV/RFC Power System Technology Roadmap; PV/NAS Battery Technology Roadmap; Thermionic Reactor Power System Technology Roadmap; SP-100 Power System Technology Roadmap; Dynamic SP-100 Power System Technology Roadmap; Near-Term Solar Dynamic Power System Technology Roadmap; Advanced Solar Dynamic Power System Technology Roadmap; Advanced Stirling Cycle Dynamic Isotope Power System Technology Roadmap; and the ESPPRS (Evolutionary Space Power and Propulsion Requirements System) User's Guide.

  1. Phase diagram of the Al-Er-Mo ternary system at 873 K

    NASA Astrophysics Data System (ADS)

    Pan, Yanfang; Yang, Wenchao; Tang, Chenghuang; Lan, Yanni; Zhan, Yong Zhong

    2015-11-01

    The phase relationship in the Al-Er-Mo ternary system at 873 K has been investigated based on the equilibrated method mainly by means of X-ray powder diffraction and scanning electron microscopy. The existence of 10 binary compounds and two ternary compounds has been confirmed. The results present that the isothermal section at 873 K is governed by 15 single-phase regions, 29 two-phase regions and 15 three-phase regions. By using the phase-disappearing method, Al8Mo3 has a narrow homogeneity range (from 72 to 73 at% Al), while the homogeneity range of AlMo3 is from 21% to 28.5% at% Al. Also, the maximum solubility of Al in Mo is about 16 at%.

  2. Increasing fatal AA amyloidosis in hunting falcons and how to identify the risk: a report from the United Arab Emirates.

    PubMed

    Hampel, Mirjam Ricarda; Kinne, Joerg; Wernery, Ulrich; Pospischil, Andreas; Kellermann, Josef; Linke, Reinhold Paul

    2009-01-01

    In hunting falcons, a fatal syndrome of wasting, weight loss, green mutes and, finally, sudden death of emaciated birds has been observed in the United Arab Emirates (UAE). Histological examination using Congo red has revealed amyloid in most organs, in particular in the liver, spleen, kidney, and adrenal glands. Moreover, a retrospective study revealed amyloidosis in 100 cases among a total of 623 necropsied falcons between August 1995 and March 2004 in Dubai/UAE (16%; varying from 8 to 30% in different raptor bird species). The amyloid was immunohistochemically classified as amyloid A (AA), which was confirmed by Western blot analysis and N-terminal amino acid sequence analysis, suggesting it to be secondary to a chronic inflammatory process. Retrospective analysis has indicated a significantly increased prevalence of bumble foot and visceral gout among falcons with amyloidosis. In addition, a significant increase of amyloidosis from 5.6% of necropsied falcons with amyloidosis in 1995 to 40.0% in 2004 has been noticed. Finally, a semi-quantitative serum test for falcon serum amyloid A (f-SAA) has been developed. Among 38 falcons with fatal AA amyloidosis, f-SAA was increased pathologically in 36, whereas f-SAA was elevated in only one of 15 apparently disease-free falcons (p < 0.001). This significant result indicates that a normal f-SAA will indicate a minimal or even absent risk of succumbing to AA amyloidosis.

  3. Solid state phase equilibria and intermetallic compounds of the Al-Cr-Ho system

    SciTech Connect

    Pang, Mingjun; Zhan, Yongzhong; Du, Yong

    2013-02-15

    The solid state phase equilibria of the Al-Cr-Ho ternary system at 500 Degree-Sign C were experimentally investigated. The phase relations at 500 Degree-Sign C are governed by 14 three-phase regions, 29 two-phase regions and 15 single-phase regions. The existences of 10 binary compounds and 2 ternary phases have been confirmed. Al{sub 11}Cr{sub 2}, Al{sub 11}Cr{sub 4} and Al{sub 17}Ho{sub 2} were not found at 500 Degree-Sign C. Crystal structures of Al{sub 9}Cr{sub 4} and Al{sub 8}Cr{sub 4}Ho were determined by the Rietveld X-ray powder data refinement. Al{sub 9}Cr{sub 4} was found to exhibit cubic structure with space group I4-bar 3m (no. 217) and lattice parameters a=0.9107(5) nm. Al{sub 8}Cr{sub 4}Ho crystallizes in ThMn{sub 12} structure type with space group I4/mmm (no. 139) and lattice parameters a=0.8909(4) nm, c=0.5120(5) nm. It is concluded that the obtained Al{sub 4}Cr phase in this work should be {mu}-Al{sub 4}Cr by comparing with XRD pattern of the hexagonal {mu}-Al{sub 4}Mn compound. - Graphical abstract: The solid state phase equilibria of the Al-Cr-Ho ternary system at 500 Degree-Sign C. Highlights: Black-Right-Pointing-Pointer Al-Cr-Ho system has been investigated. Black-Right-Pointing-Pointer Al{sub 9}Cr{sub 4} has cubic structure with space group I4-bar 3m. Black-Right-Pointing-Pointer Al{sub 8}Cr{sub 4}Ho crystallizes in ThMn{sub 12} type with space group I4/mmm. Black-Right-Pointing-Pointer Al{sub 4}Cr phase is {mu}-type at 500 Degree-Sign C.

  4. {sup 26}Al IN THE EARLY SOLAR SYSTEM: NOT SO UNUSUAL AFTER ALL

    SciTech Connect

    Jura, M.; Xu, S.; Young, E. D. E-mail: sxu@astro.ucla.edu

    2013-10-01

    Recently acquired evidence shows that extrasolar asteroids exhibit over a factor of 100 variation in the iron to aluminum abundance ratio. This large range likely is a consequence of igneous differentiation that resulted from heating produced by radioactive decay of {sup 26}Al with an abundance comparable to that in the solar system's protoplanetary disk at birth. If so, the conventional view that our solar system began with an unusually high amount of {sup 26}Al should be discarded.

  5. Component Activity Measurements in the Ti-Al-O System by Knudsen Cell Mass Spectrometry

    NASA Technical Reports Server (NTRS)

    Copland, Evan; Jacobson, Nathan S.

    2003-01-01

    Titanium-aluminides (containing (alpha)2-Ti3Al and gamma-TiAl intermetallic phases) have received continued research focus due to their potential as low-density materials for structural applications at intermediate temperatures. However their application above about 850C is hindered by poor oxidation resistance, characterized by the formation of a non-protective TiO2+Al2O3 scale and an oxygen-enriched subsurface zone. Consistent with this are measured titanium and aluminum activities in "oxygen-free" titanium-aluminides, which indicate Al2O3 is only stable for aluminum concentrations greater then 54 atom percent at 1373 K. However, the inability to form a protective Al2O3 scale is in apparent conflict with phase diagram studies, as experimental isothermal sections of the Ti-Al-O system show gamma-TiAl + alpha2-Ti3Al structures are in equilibrium only with Al2O3. The apparent resolution to this conflict lies in the inclusion of oxygen effects in the thermodynamic measurements

  6. [Osteo-articular amyloidosis caused by dialysis. Clinical and radiologic aspects].

    PubMed

    Baldrati, L; Brunetti, L; Rocchi, A; Bonsanto, R; Docci, D; Turci, F

    1990-10-01

    Twenty-nine patients who had received chronic hemodialysis for more than 5 years provided the material for the present study. In 12 of them (41%) there were radiological findings of dialysis related amyloidosis, mainly destructive spondyloarthropathy of the cervical spine (n = 11) and geodes of the shoulder (n = 5). When compared with negative patients, these patients were significantly older (p less than 0.001 and had been dialyzed for longer periods of time (p less than 0.01). Moreover, in such patients there was an higher incidence of carpal tunnel syndrome (p less than 0.025) and shoulder pain (p less than 0.001). Our results confirm that osteoarticular amyloidosis is a frequent long-term complication of chronic hemodialysis and underline the correlation between clinical and radiological findings.

  7. Is 18F-flutemetamol PET/CT able to reveal cardiac amyloidosis?

    PubMed

    Lhommel, Renaud; Sempoux, Christine; Ivanoiu, Adrian; Michaux, Lucienne; Gerber, Bernhard

    2014-08-01

    We report here the case of a 56-year-old man with well-documented cardiac light chain amyloidosis successfully imaged with F-flutemetamol PET/CT in 2011. A matched pair healthy volunteer was imaged in the same conditions, and no specific heart uptake could be detected. The present report suggests the potential benefit of this new pharmaceutical in this indication, as it has been recently reported for C-Pittsburgh compound B, but with the main advantage to benefit from the more practical half-life of F. Further prospective trials would, however, be required to define the potential impact of F-flutemetamol PET/CT in cardiac amyloidosis.

  8. Phase diagram of the Co-Al-W system. structure and phase transformations near the Co3(Al, W) intermetallic composition range

    NASA Astrophysics Data System (ADS)

    Kazantseva, N. V.; Demakov, S. L.; Yurovskikh, A. S.; Stepanova, N. N.; Vinogradova, N. I.; Davydov, D. I.; Lepikhin, S. V.

    2016-07-01

    Low-temperature portion of the polythermal section for the Co-Al-W system in the vicinity of the Co3(Al, W) intermetallic composition has been studied experimentally using electron microscopy and hightemperature X-ray diffraction analysis. Low-temperature structural phase transformations and temperature ranges of the existence of phases have been determined. The morphology of Co3(Al, W) intermetallic particles was studied as a function of the tungsten content in alloys.

  9. Light chain amyloidosis of the urinary bladder. A site restricted deposition of an externally produced immunoglobulin

    PubMed Central

    Livneh, A; Shtrasburg, S; Martin, B; Baniel, J; Gal, R; Pras, M

    2001-01-01

    Aims—To identify the amyloid protein in a patient with amyloidosis localised to the urinary bladder, and to see whether subtyping of the protein by sequence analysis increases the understanding of the selection of the urinary bladder as the site of amyloid deposition. Methods—A patient with gross haematuria and a congophilic mass in his urinary bladder was evaluated further. Characterisation of the amyloid protein was performed using conventional histological and immunohistochemical methods. Determination of the N-terminal amino acid sequence of the amyloid protein was performed using protein sequencers. Results—The patient's history, physical examination, and laboratory evaluation excluded the involvement of other organs, justifying a diagnosis of amyloidosis localised to the urinary bladder. Histological and immunological studies showed that the amyloid protein deposited in the urinary bladder of the patient was probably of the amyloid light chain type. No plasma cells or lymphocytes were seen in sections of the urinary bladder and lower ureter adjacent to the amyloid deposits. Molecular analysis showed the sequence NFMLTQPHSISGSPG, which assigned the amyloid protein to either the VλI or the VλVI immunoglobulin (Ig) light chain families. Conclusions—The findings suggest that the amyloid protein in this patient originated outside the urinary bladder. The heterogeneity of the Ig proteins in known cases of amyloidosis of the lower urinary tract suggests that the amino acid residues, which determine the Vλ subtyping, have no major role in restricting the deposited protein to the urinary bladder. Key Words: primary amyloidosis • urinary bladder • λ light chain • amino acid sequence PMID:11729210

  10. Ar + induced interfacial mixing and phase formation in the Al/Cr system

    NASA Astrophysics Data System (ADS)

    Kim, H. K.; Kim, S. O.; Song, J. H.; Kim, K. W.; Woo, J. J.; Whang, C. N.; Smith, R. J.

    1991-07-01

    Evaporated Al/Cr bilayer thin films were irradiated by 80 keV Ar + at doses in the range from 1 × 10 15 to 2 × 10 16 Ar +/cm 2 at room temperature in order to investigate the Ar + induced interfacial mixing behavior and the phase formation and transition by Ar + bombardment. Ion bombardment induces intermixing across the Al/Cr interface and mixing variance increases with increasing ion dose. Cascade and thermal spike models are found to be not adequate for the ion beam mixing mechanism at room temperature in this system. The Al 13Cr 2 phase is formed as an initial phase by ion beam mixing and then transforms into the Al 11Cr 2 or Al 4Cr phases at subsequent ion bombardment. This result is discussed in terms of the enhanced atomic mobility and the thermodynamical driving force by introducing the concept of an effective heat of formation.

  11. A Tongue Lesion as a Sign of a Systemic Disease

    PubMed Central

    Liakou, Chrysoula I.; Koh, Joan; Tsimpidakis, Antonios; Rios, Katrina; Paskalis, Charalabos; Pipilis, Athanasios; Kantianis, Dimitrios; Georgiadis, Thomas

    2016-01-01

    Amyloidosis is the extracellular fibril deposition of a variety of proteins, many of which circulate as plasma ingredients. It is a disease difficult to identify due to its nonspecific symptoms and manifestations. Amyloidosis of the tongue, either isolated or part of the systemic disease, is rare and its features resemble those of a tumor. We report the case of a patient with amyloidosis who presented with a tongue lesion, weakness, nonspecific arthritis, and dyspnea on exertion that resulted in multiorgan system failure. PMID:27092182

  12. Gene expression profile in hereditary transthyretin amyloidosis: differences in targeted and source organs

    PubMed Central

    Olsson, Malin; Nyström, Hanna; Ericzon, Bo Göran; de Tayrac, Marie; Genin, Emmanuelle; Planté-Bordeneuve, Violaine; Suhr, Ole B

    2014-01-01

    Introduction Hereditary transthyretin amyloidosis (ATTR) is a genetic disease caused by a point mutation in the TTR gene that causes the liver to produce an unstable TTR protein. The most effective treatment has been liver transplantation in order to replace the variant TTR producing liver with one that produces only wild-type TTR. ATTR amyloidosis patients’ livers are reused for liver sick patients, i.e. the Domino procedure. However, recent findings have demonstrated that ATTR amyloidosis can develop in the recipients within 7–8 years. The aim of this study was to elucidate how the genetic profile of the liver is affected by the disease, and how amyloid deposits affect target tissue. Methods Gene expression analysis was used to unravel the genetic profiles of Swedish ATTR V30M patients and controls. Biopsies from adipose tissue and liver were examined. Results and Conclusions ATTR amyloid patients’ gene expression profile of the main source organ, the liver, differed markedly from that of the controls, whereas the target organs’ gene expression profiles were not markedly altered in the ATTR amyloid patients compared to those of the controls. An impaired ER/protein folding pathway might suggest ER overload due to mutated TTR protein. PMID:24601850

  13. Hereditary cerebral hemorrhage with amyloidosis in patients of Dutch origin is related to Alzheimer disease

    SciTech Connect

    van Duinen, S.G.; Castano, E.M.; Prelli, F.; Bots, G.T.A.B.; Luyendijk, W.; Frangione, B.

    1987-08-01

    Hereditary cerebral hemorrhage with amyloidosis in Dutch patients is an autosomal dominant form of vascular amyloidosis restricted to the leptomeninges and cerebral cortex. Clinically the disease is characterized by cerebral hemorrhages leading to an early death. Immunohistochemical studies of five patients revealed that the vascular amyloid deposits reacted intensely with an antiserum raised against a synthetic peptide homologous to the Alzheimer disease-related ..beta..-protein. Silver stain-positive, senile plaque-like structures were also labeled by the antiserum, yet these lesions lacked the dense amyloid cores present in typical plaques of Alzheimer disease. No neurofibrillary tangles were present. Amyloid fibrils were purified from the leptomeningeal vessels of one patient who clinically had no signs of dementia. The protein had a molecular weight of approx. 4000 and its partial amino acid sequence to position 21 showed homology to the ..beta..-protein of Alzheimer disease and Down syndrome. These results suggest that hereditary cerebral hemorrhage with amyloidosis of Dutch origin is pathogenetically related to Alzheimer disease and support the concept that the initial amyloid deposition in this disorder occurs in the vessel walls before damaging the brain parenchyma. Thus, deposition of ..beta..-protein in brain tissue seems to be related to a spectrum of diseases involving vascular syndromes, progressive dementia, or both.

  14. Tubulointerstitial nephritis is a dominant feature of hereditary apolipoprotein A-I amyloidosis.

    PubMed

    Gregorini, Gina; Izzi, Claudia; Ravani, Pietro; Obici, Laura; Dallera, Nadia; Del Barba, Andrea; Negrinelli, Alessandro; Tardanico, Regina; Nardi, Matilde; Biasi, Luciano; Scalvini, Tiziano; Merlini, Giampaolo; Scolari, Francesco

    2015-06-01

    Apolipoprotein A-I is the main protein of high-density lipoprotein particles, and is encoded by the APOA1 gene. Several APOA1 mutations have been found, either affecting the lecithin:cholesterol acyltransferase activity, determining familial HDL deficiency, or resulting in amyloid formation with prevalent deposits in the kidney and liver. Evaluation of familial tubulointerstitial nephritis in patients with the Leu75Pro APOA-I amyloidosis mutation resulted in the identification of 253 carriers belonging to 50 families from Brescia, Italy. A total of 219 mutation carriers underwent clinical, laboratory, and instrumental tests. Of these, 62% had renal, hepatic, and testicular disease; 38% were asymptomatic. The disease showed an age-dependent penetrance. Tubulointerstitial nephritis was diagnosed in 49% of the carriers, 13% of whom progressed to kidney failure requiring dialysis. Hepatic involvement with elevation of cholestasis indices was diagnosed in 30% of the carriers, 38% of whom developed portal hypertension. Impaired spermatogenesis and hypogonadism was found in 68% of male carriers. The cholesterol levels were lower than normal in 80% of the mutation carriers. Thus, tubulointerstitial nephritis was highly prevalent in this large series of patients with Leu75Pro apoA-I amyloidosis. Persistent elevation of alkaline phosphatase, reduced HDL cholesterol plasma levels, and hypogonadism in men are key diagnostic features of this form of amyloidosis.

  15. Reactive eccrine syringofibroadenomatosis secondary to primary cutaneous amyloidosis: a novel association.

    PubMed

    Saggini, Andrea; Mully, Thaddeus

    2014-04-01

    We report the unprecedented case of reactive eccrine syringofibroadenoma (ESFA) secondary to primary cutaneous amyloidosis. A 62-year-old woman of Asian ethnicity presented with a pruritic rash on the back of long-standing duration. Physical examination revealed diffuse hyperpigmentation localized to the interscapular region; there were a multitude of hyperpigmented macules merged in a rippled pattern intermixed with scattered papules and cobblestone-like areas. A punch biopsy from a papule was taken. Histopathological examination revealed a network of epithelial strands and cords hanging from the epidermis and harboring foci of ductal differentiation. Eosinophilic collections of amorphous material were found between the epithelial strands, obscuring the superficial dermis. The microscopic picture was consistent with primary cutaneous amyloidosis associated with reactive ESFA. Results of histochemical and immunohistochemical staining confirmed the diagnosis. We speculate that pathogenetic mechanisms intrinsic to primary cutaneous amyloidosis, in addition to unknown genetic factors, resulted in clinical changes of lichen amyloidosus associated with an abnormal hyperplastic epithelial response with histopathological features of ESFA rather than the common epidermal change of acanthosis and hyperkeratosis. PMID:24325801

  16. Early accretion of protoplanets inferred from a reduced inner solar system 26Al inventory

    NASA Astrophysics Data System (ADS)

    Schiller, Martin; Connelly, James N.; Glad, Aslaug C.; Mikouchi, Takashi; Bizzarro, Martin

    2015-06-01

    The mechanisms and timescales of accretion of 10-1000 km sized planetesimals, the building blocks of planets, are not yet well understood. With planetesimal melting predominantly driven by the decay of the short-lived radionuclide 26Al (26Al→26Mg; t1/2 = 0.73 Ma), its initial abundance determines the permissible timeframe of planetesimal-scale melting and its subsequent cooling history. Currently, precise knowledge about the initial 26Al abundance [(26Al/27Al)0] exists only for the oldest known solids, calcium aluminum-rich inclusions (CAIs) - the so-called canonical value. We have determined the 26Al/27Al of three angrite meteorites, D'Orbigny, Sahara 99555 and NWA 1670, at their time of crystallization, which corresponds to (3.98 ± 0.15) ×10-7, (3.64 ± 0.18) ×10-7, and (5.92 ± 0.59) ×10-7, respectively. Combined with a newly determined absolute U-corrected Pb-Pb age for NWA 1670 of 4564.39 ± 0.24 Ma and published U-corrected Pb-Pb ages for the other two angrites, this allows us to calculate an initial (26Al/27Al)0 of (1.33-0.18+0.21) ×10-5 for the angrite parent body (APB) precursor material at the time of CAI formation, a value four times lower than the accepted canonical value of 5.25 ×10-5. Based on their similar 54Cr/52Cr ratios, most inner solar system materials likely accreted from material containing a similar 26Al/27Al ratio as the APB precursor at the time of CAI formation. To satisfy the abundant evidence for widespread planetesimal differentiation, the subcanonical 26Al budget requires that differentiated planetesimals, and hence protoplanets, accreted rapidly within 0.25 ± 0.15 Ma of the formation of canonical CAIs.

  17. Systems integration and demonstration of advanced reusable structure for ALS

    NASA Technical Reports Server (NTRS)

    Gibbins, Martin N.

    1991-01-01

    The objective was to investigate the potential of advanced material to achieve life cycle cost (LCC) benefits for reusable structure on the advanced launch system. Three structural elements were investigated - all components of an Advanced Launch System reusable propulsion/avionics module. Leading aeroshell configurations included sandwich structure using titanium, graphite/polyimide (Gr/PI), or high-temperature aluminum (HTA) face sheets. Thrust structure truss concepts used titanium, graphite/epoxy, or silicon carbide/aluminum struts. Leading aft bulkhead concepts employed graphite epoxy and aluminum. The technical effort focused on the aeroshell because the greatest benefits were expected there. Thermal analyses show the structural temperature profiles during operation. Finite element analyses show stresses during splash-down. Weight statements and manufacturing cost estimates were prepared for calculation of LCC for each design. The Gr/PI aeroshell showed the lowest potential LCC, but the HTA aeroshell was judged to be lower risk. A technology development plan was prepared to validate the applicable structural technology.

  18. Postperovskite phase equilibria in the MgSiO3-Al2O3 system.

    PubMed

    Tsuchiya, Jun; Tsuchiya, Taku

    2008-12-01

    We investigate high-P,T phase equilibria of the MgSiO(3)-Al(2)O(3) system by means of the density functional ab initio computation methods with multiconfiguration sampling. Being different from earlier studies based on the static substitution properties with no consideration of Rh(2)O(3)(II) phase, present calculations demonstrate that (i) dissolving Al(2)O(3) tends to decrease the postperovskite transition pressure of MgSiO(3) but the effect is not significant ( approximately -0.2 GPa/mol% Al(2)O(3)); (ii) Al(2)O(3) produces the narrow perovskite+postperovskite coexisting P,T area (approximately 1 GPa) for the pyrolitic concentration (x(Al2O3) approximately 6 mol%), which is sufficiently responsible to the deep-mantle D'' seismic discontinuity; (iii) the transition would be smeared (approximately 4 GPa) for the basaltic Al-rich composition (x(Al2O3) approximately 20 mol%), which is still seismically visible unless iron has significant effects; and last (iv) the perovskite structure spontaneously changes to the Rh(2)O(3)(II) with increasing the Al concentration involving small displacements of the Mg-site cations.

  19. Solid Solution Effects on the MgAl2O4 System

    SciTech Connect

    O'Hara, Kelley; Smith, Jeffrey D; Hemrick, James Gordon

    2009-01-01

    Phase relations between the binaries MgAl2O4-ZnAl2O4 and MgAl2O4-MgGa2O4 were studied. Stoichiometric MgAl2O4 spinel can be formed in the laboratory through a coprecipitation method. Complete solid solution formation in the MgAl2O4-MgGa2O4 system was confirmed through X-ray diffraction (XRD) analysis. XRD analysis of the MgAl2O4-ZnAl2O4 system did not confirm solid solution due to the similar lattice parameters of the two end points, however, previous studies have shown that complete solid solution does form. Thermal conductivity data is pending and will be included in the presentation. Based on previous experimentation and open literature, it is suspected that thermal conductivity will be decreased with the addition of solid solution. With increased amounts of disruption to the lattice from solid solution it is also theorized that the temperature at which the mean free path still impacts thermal conductivity could be increased.

  20. Direct Metal Deposition by Laser in TiNi-Al System for Graded Structure Fabrication

    NASA Astrophysics Data System (ADS)

    Shishkovsky, I.; Kakovkina, N.; Missemer, F.

    2016-07-01

    Intermetallic phase formation was studied in powdered TiNi-Al system under layerwise laser cladding with the aim of forming a gradient of properties due to a change in the concentration relation of Al in the NiTi powder mixture from one layer to another. The relationship between the laser cladding parameters and the intermetallic phase structures in consecutively cladded layers were determined. The structure of intermetallic compounds formed by laser synthesis was studied by optical microscopy, measurement of microhardness, SEM with EDX analysis. Microhardness doubling from 500 HV to 1000 HV was achieved due to nitinol matrix enrichment by Al, which is promising for aerospace applications.

  1. Vacuum system for the LBL advanced light source (ALS)

    SciTech Connect

    Kennedy, K.

    1988-05-01

    A 1.5 to 1.9 GeV synchrotron light source is being built at LBL. The vacuum system is designed to permit all synchrotron photons on the median plane to escape the electron channel and go into an antechamber through a 10 mm high slot. This slot offers effective RF isolation between the electron duct and the antechamber. All unused synchrotron photons within a few mrad of the median plane will be stopped by 96 nearly horizontal absorbers located in the antechamber. The gas, generated by the photons hitting the absorbers, will be directed down to reactive titanium surfaces. Twelve 10 meter long vessels constitute the vacuum chambers for all the lattice magnets. Each chamber will be machined from two thick plates of 5083-H321 aluminum and welded at the perimeter. The nominal wall thickness of the vacuum chamber is 40 mm, which makes it possible to machine a flange into the chamber without the use of welding. 5 refs., 5 figs.

  2. ALS control system IP I/O module upgrade

    SciTech Connect

    Weber, Jonah M.; Chin, Michael

    2004-04-23

    The Control System for the Advanced Light Source uses in-house designed IndustryPack (IP) I/O Modules in compact PCI (cPCI) chassis to control instrumentation. Each module consists of digital I/O ports and 16-bit analog I/O interfaced to instrumentation via a cPCI rear I/O card. During the past few years of installed operation, several factors have prompted investigation into the design of a new IP I/O Module. The ADC channels have significant offset drift over periods of days of initial installed operation. An in-situ calibration procedure was developed to address this problem, but it lacks speed and is inconvenient to perform. Digital I/O port limitations have led to increasing amounts of wasted I/O. Fast orbit feedback requires faster ADC sampling and better filtering than the current IP module offers. This paper discusses the issues related to the current IP I/O Module and the design of a new Double-size IP I/O Module.

  3. Molecular dynamics simulation of surface segregation, diffusion and reaction phenomena in equiatomic Ni-Al systems

    NASA Astrophysics Data System (ADS)

    Evteev, A. V.; Levchenko, E. V.; Belova, I. V.; Murch, G. E.

    2012-12-01

    The molecular dynamics method is used to provide fundamental insights into surface segregation, bulk diffusion and alloying reaction phenomena in equiatomic Ni-Al systems. This knowledge can serve as a guide for the search and development of economic routes for controlling microstructure and properties of the intermetallic compound NiAl. This paper gives an overview of recent molecular dynamics simulations in the area along with other theoretical calculations and experimental measurements.

  4. Speciation in the AlCl3/SO2Cl2 catholyte system.

    SciTech Connect

    Boyle, Timothy J.; Ingersoll, David T.; Tallant, David Robert; Andrews, Nicholas L.; Rodriguez, Mark Andrew; Alam, Todd Michael

    2005-02-01

    The fundamental chemical behavior of the AlCl{sub 3}/SO{sub 2}Cl{sub 2} catholyte system was investigated using {sup 27}Al NMR spectroscopy, Raman spectroscopy, and single-crystal X-ray diffraction. Three major Al-containing species were found to be present in this catholyte system, where the ratio of each was dependent upon aging time, concentration, and/or storage temperature. The first species was identified as [Cl{sub 2}Al({mu}-Cl)]{sub 2} in equilibrium with AlCl{sub 3}. The second species results from the decomposition of SO{sub 2}Cl{sub 2} which forms Cl{sub 2}(g) and SO{sub 2}(g). The SO{sub 2}(g) is readily consumed in the presence of AlCl{sub 3} to form the crystallographically characterized species [Cl{sub 2}Al({mu}-O{sub 2}SCl)]{sub 2} (1). For 1, each Al is tetrahedrally (T{sub d}) bound by two terminal Cl and two {mu}-O ligands whereas, the S is three-coordinated by two {mu}-O ligands and one terminal Cl. The third molecular species also has T{sub d}-coordinated Al metal centers but with increased oxygen coordination. Over time it was noted that a precipitate formed from the catholyte solutions. Raman spectroscopic studies show that this gel or precipitate has a component that was consistent with thionyl chloride. We have proposed a polymerization scheme that accounts for the precipitate formation. Further NMR studies indicate that the precipitate is in equilibrium with the solution.

  5. HETEROGENEOUS DISTRIBUTION OF {sup 26}Al AT THE BIRTH OF THE SOLAR SYSTEM

    SciTech Connect

    Makide, Kentaro; Nagashima, Kazuhide; Krot, Alexander N.; Huss, Gary R.; Ciesla, Fred J.; Yang, Le; Hellebrand, Eric; Gaidos, Eric

    2011-06-01

    It is believed that {sup 26}Al, a short-lived (t{sub 1/2} = 0.73 Ma) and now extinct radionuclide, was uniformly distributed in the nascent solar system (SS) with the initial {sup 26}Al/{sup 27}Al ratio of {approx}5.2 x 10{sup -5}, suggesting an external, stellar origin rather than local, solar source. However, the stellar source of {sup 26}Al and the manner in which it was injected into the SS remain controversial: the {sup 26}Al could have been produced by an asymptotic giant branch star, a supernova, or a Wolf-Rayet star and injected either into the protosolar molecular cloud, protosolar cloud core, or protoplanetary disk. Corundum (Al{sub 2}O{sub 3}) is predicted to be the first condensate from a cooling gas of solar composition. Here we show that micron-sized corundum condensates from {sup 16}O-rich ({Delta}{sup 17}O {approx} -25 per mille ) gas of solar composition recorded heterogeneous distribution of {sup 26}Al at the birth of the SS: the inferred initial {sup 26}Al/{sup 27}Al ratio ranges from {approx}6.5x10{sup -5} to <2x10{sup -6}; 52% of corundum grains measured are {sup 26}Al-poor. Abundant {sup 26}Al-poor, {sup 16}O-rich refractory objects include grossite- and hibonite-rich calcium-aluminum-rich inclusions (CAIs) in CH (high metal abundance and high iron concentration) chondrites, platy hibonite crystals in CM (Mighei-like) chondrites, and CAIs with fractionation and unidentified nuclear effects CAIs chondrites. Considering the apparently early and short duration (<0.3 Ma) of condensation of refractory {sup 16}O-rich solids in the SS, we infer that {sup 26}Al was injected into the collapsing protosolar molecular cloud and later homogenized in the protoplanetary disk. The apparent lack of correlation between {sup 26}Al abundance and O-isotope composition of corundum grains constrains the stellar source of {sup 26}Al in the SS.

  6. Timing and extent of Mg and Al isotopic homogenization in the early inner Solar System

    NASA Astrophysics Data System (ADS)

    Mishra, Ritesh Kumar; Chaussidon, Marc

    2014-03-01

    The first million years of the Solar System is a key period when the first solids were formed from the nebula gas. The chronology of the different processes at the origin of these solids is still largely unknown and relies strongly on the assumption made of homogeneous distribution for short-lived radioactive nuclides such as 26Al. This assumption is questioned. In this study, in situ 26Al-26Mg isotope systematics was studied with high precision in 12 calcium, aluminum-rich inclusions (CAIs) (1 type A, 2 type B, 5 type C, and 4 fine grained spinel-rich), 2 amoeboid olivine aggregates (AOAs), and 2 Al-rich chondrules from Efremovka and Vigarano. The (26Al/27Al)i in these early Solar System solids (the subscript ‘i’ stands for the initial isotope ratio obtained from the mineral 26Al isochron) range from ∼6.5×10-5 to 0.2×10-5 with δMgi*26 from -0.08 to +0.37‰. The (26Al/27Al)i and δMgi*26 of CAIs and chondrules can be explained by formation of their precursors from a homogeneous reservoir (Solar System Initial, noted hereafter SSI) with initial magnesium isotopic composition of δMgSSI*26=-0.052±0.013‰ and initial (26Al/27Al)SSI abundance of (5.62±0.42)×10-5. The high precision magnesium isotope data obtained in the present study and literature data allows identifying a few epochs of formation/reprocessing of CAIs. The time periods of these epochs correspond well with the median life times of the pre-main sequence evolution of stars of Solar mass if we anchor the (Al26/Al27)SSI=(5.62±0.42)×10-5 to the beginning of class I phase. This provides a natural explanation to the range of (26Al/27Al)i - (∼6 to 0.02) × 10-5 seen in corundum grains, FUN (Fractionation and Unidentified Nuclear Effects) CAIs, ultrarefractory CAIs, normal CAIs, and chondrules, and suggests a possible relationship between the astrophysical conditions and the formation of these early solids. Corundum grains, FUN CAIs, ultrarefractory CAIs would have formed during the class 0 of

  7. Al6Ti2O13, a new phase in the Al2O3-TiO2 system.

    PubMed

    Norberg, Stefan T; Hoffmann, Stefan; Yoshimura, Masahiro; Ishizawa, Nobuo

    2005-03-01

    The compound Al6Ti2O13 (hexaaluminium dititanium tridecaoxide) has been synthesized using an arc-imaging furnace, which allows fast cooling of melted oxides. The structure consists of infinite double chains of polyhedra running along the c axis. These chains are built up by four kinds of strongly distorted oxygen octahedra randomly occupied by either Ti or Al (point symmetry m or m2m), and by trigonal bipyramids exclusively occupied by Al (point symmetry m2m).

  8. The rate and significance of type 1/type 2 serum amyloid A protein gene polymorphisms in patients with ankylosing spondylitis and amyloidosis.

    PubMed

    Yildirim Cetin, Gozde; Ganiyusufoglu, Eda; Solmaz, Dilek; Cagatay, Yonca; Yılmaz Oner, Sibel; Erer, Burak; Sagliker, Hasan Sabit; Avci, Ali Berkant; Akar, Servet; Pamuk, Omer Nuri; Kılınc, Metin; Kasifoglu, Timucin; Direskeneli, Haner; Gul, Ahmet; Sayarlioglu, Mehmet

    2015-01-01

    A relationship between the presence of amyloidosis and SAA1 genotype has been shown in recent studies of (principally) familial Mediterranean fever patients. We found that the SAA1 rs12218 polymorphism was significantly more prevalent in ankylosing spondylitis patients with amyloidosis.

  9. Operation and performance of the PEP-II prototype longitudinal damping system at ALS

    SciTech Connect

    Teytelman, D.; Claus, R.; Fox, J.

    1995-05-01

    A modular programmable longitudinal feedback system has been developed as a component of the PEP-II R+D program. This system is based on a family of VME and VXI packaged signal processing functions which implement a general purpose digital feedback controller for accelerators with bunch spacings of 2 ns. A complete PEP-II prototype system has been configured and installed for use at the LBL Advanced Light Source. The system configuration used for tests at the ALS is described and results are presented showing the action of the feedback system. Open and closed loop results showing the detection and calculation of feedback signals from bunch motion are presented and the system is shown to damp coupled-bunch instabilities in the ALS. Use of the system for accelerator diagnostics is illustrated via measurement of grow-damp transients which quantify growth rates without feedback, damping rates with feedback, and identify unstable modes.

  10. High Density Hydrogen Storage System Demonstration Using NaAlH4 Based Complex Compound Hydrides

    SciTech Connect

    Daniel A. Mosher; Xia Tang; Ronald J. Brown; Sarah Arsenault; Salvatore Saitta; Bruce L. Laube; Robert H. Dold; Donald L. Anton

    2007-07-27

    This final report describes the motivations, activities and results of the hydrogen storage independent project "High Density Hydrogen Storage System Demonstration Using NaAlH4 Based Complex Compound Hydrides" performed by the United Technologies Research Center under the Department of Energy Hydrogen Program, contract # DE-FC36-02AL67610. The objectives of the project were to identify and address the key systems technologies associated with applying complex hydride materials, particularly ones which differ from those for conventional metal hydride based storage. This involved the design, fabrication and testing of two prototype systems based on the hydrogen storage material NaAlH4. Safety testing, catalysis studies, heat exchanger optimization, reaction kinetics modeling, thermochemical finite element analysis, powder densification development and material neutralization were elements included in the effort.

  11. Further insight into mechanisms of solid-state interactions in UMo/Al system

    NASA Astrophysics Data System (ADS)

    Mazaudier, F.; Proye, C.; Hodaj, F.

    2008-07-01

    In this paper, the solid-state interactions between metastable γ-UMo alloys (containing 5, 7 and 10 wt%Mo) and Al, at temperatures ranging from 440 to 600 °C and for ageing times up to 10 h, are studied using the diffusion couple technique and nuclear fuel plate annealing. The reaction product consists of three main zones, two of them presenting a periodic layered morphology. The growth kinetics is limited by solid-state diffusion and Al is the most mobile species. Both growth kinetics and its global energy of activation are similar to that found for the U/Al binary system. The diffusion path is determined and phase equilibrium relations are deduced for the Mo-poor part of the U-Mo-Al metastable ternary phase diagram.

  12. Process for preparing high-transition-temperature superconductors in the Nb-Al-Ge system

    DOEpatents

    Giorgi, A.L.; Szklarz, E.G.

    1973-01-30

    The patent describes a process for preparing superconducting materials in the Nb-Al-Ge system having transition temperatures in excess of 19K. The process comprises premixing powdered constituents, pressing them into a plug, heating the plug to 1,450-1,800C for 30 minutes to an hour under vacuum or an inert atmosphere, and annealing at moderate temperatures for reasonably long times (approximately 50 hours). High transition-temperature superconductors, including those in the Nb3(Al,Ge) system, prepared in accordance with this process exhibit little degradation in the superconducting transition temperature on being ground to -200 mesh powder. (GRA)

  13. Depletion of Spleen Macrophages Delays AA Amyloid Development: A Study Performed in the Rapid Mouse Model of AA Amyloidosis

    PubMed Central

    Lundmark, Katarzyna; Vahdat Shariatpanahi, Aida; Westermark, Gunilla T.

    2013-01-01

    AA amyloidosis is a systemic disease that develops secondary to chronic inflammatory diseases Macrophages are often found in the vicinity of amyloid deposits and considered to play a role in both formation and degradation of amyloid fibrils. In spleen reside at least three types of macrophages, red pulp macrophages (RPM), marginal zone macrophages (MZM), metallophilic marginal zone macrophages (MMZM). MMZM and MZM are located in the marginal zone and express a unique collection of scavenger receptors that are involved in the uptake of blood-born particles. The murine AA amyloid model that resembles the human form of the disease has been used to study amyloid effects on different macrophage populations. Amyloid was induced by intravenous injection of amyloid enhancing factor and subcutaneous injections of silver nitrate and macrophages were identified with specific antibodies. We show that MZMs are highly sensitive to amyloid and decrease in number progressively with increasing amyloid load. Total area of MMZMs is unaffected by amyloid but cells are activated and migrate into the white pulp. In a group of mice spleen macrophages were depleted by an intravenous injection of clodronate filled liposomes. Subsequent injections of AEF and silver nitrate showed a sustained amyloid development. RPMs that constitute the majority of macrophages in spleen, appear insensitive to amyloid and do not participate in amyloid formation. PMID:24236094

  14. Composition Optimization of Al-DOPING Lithium Manganese Oxide from Al2O3-Li2CO3-MnO2 Ternary System

    NASA Astrophysics Data System (ADS)

    He, Gang; Sun, Xinyan; Hong, Jianhe; He, Mingzhong

    2013-07-01

    In order to synthesize eutectic compound of Al doping lithium manganese oxide which can be used as cathode material in lithium battery, using γ-Al2O3, Li2CO3 and MnO2 as starting raw materials, the composition optimization research work has been done by the solid state synthesis method. A limited composition range was found in Al2O3-Li2CO3-MnO2 ternary system, in which the synthesized Al doping lithium manganese oxides have single spinel structure and good electrochemical performance. The results showed that the LiAl0.04Mn1.96O4 material presented better charge-discharge cycling behavior than pure LiMn2O4, and showed the best electrochemistry property among the compounds in the Al2O3-Li2O-Mn2O3 ternary system. LiAl0.04Mn1.96O4 still kept perfect cubic structure, but LiMn2O4 kept the coexistence of the cubic and tetragonal phases after 50 charge-discharge cycles.

  15. Kr implantation into heavy ion irradiated monolithic U-Mo/Al systems: SIMS and SEM investigations

    NASA Astrophysics Data System (ADS)

    Zweifel, T.; Valle, N.; Grygiel, C.; Monnet, I.; Beck, L.; Petry, W.

    2016-03-01

    Worldwide, high performance research and material test reactors are aiming to convert their fuel from high enriched uranium towards low enriched ones. High density U-Mo/Al based nuclear fuels are considered as a promising candidate for this conversion. However, during in-pile test irradiations, the formation of an interdiffusion layer (IDL) between the U-Mo and the Al matrix is observed, caused by irradiation enhanced U-Al interdiffusion processes. This IDL accumulates fission gases at the IDL/matrix interfaces. Together, these two effects strongly reduce the performance of this new fuel type. Recently, the out-of-pile technique of heavy ion irradiation (127I) on U-Mo/Al layer systems proved to be an alternative to time-consuming in-pile test irradiations for certain fuel behaviour aspects. Here we present SIMS and SEM investigations of non-conventional 82Kr implantation into previously heavy ion irradiated U-Mo/Al layer systems. It is shown that Kr accumulates inside μm large porosities at the IDL/matrix interfaces. This critical accumulation of μm-sized large gas bubbles is directly related to the presence of the irradiation induced IDL. Without IDL no critical accumulation of fission gas bubbles occurs.

  16. Hydrogen bonds in Al2O3 as dissipative two-level systems in superconducting qubits

    PubMed Central

    Gordon, Luke; Abu-Farsakh, Hazem; Janotti, Anderson; Van de Walle, Chris G.

    2014-01-01

    Dissipative two-level systems (TLS) have been a long-standing problem in glassy solids over the last fifty years, and have recently gained new relevance as sources of decoherence in quantum computing. Resonant absorption by TLSs in the dielectric poses a serious limitation to the performance of superconducting qubits; however, the microscopic nature of these systems has yet to be established. Based on first-principles calculations, we propose that hydrogen impurities in Al2O3 are the main source of TLS resonant absorption. Hydrogen is an ubiquitous impurity and can easily incorporate in Al2O3. We find that interstitial H in Al2O3 forms a hydrogen bond (O-H…O). At specific O-O distances, consistent with bond lengths found in amorphous Al2O3 or near Al2O3 surfaces or interfaces, the H atom feels a double well. Tunneling between two symmetric positions gives rise to resonant absorption in the range of 10 GHz, explaining the experimental observations. We also calculate the expected qubit-TLS coupling and find it to lie between 16 and 20 MHz, consistent with experimental measurements. PMID:25534108

  17. Early accretion of protoplanets inferred from a reduced inner solar system 26Al inventory

    PubMed Central

    Schiller, Martin; Connelly, James N.; Glad, Aslaug C.; Mikouchi, Takashi; Bizzarro, Martin

    2016-01-01

    The mechanisms and timescales of accretion of 10–1000 km sized planetesimals, the building blocks of planets, are not yet well understood. With planetesimal melting predominantly driven by the decay of the short-lived radionuclide 26Al (26Al→26Mg; t1/2 = 0.73 Ma), its initial abundance determines the permissible timeframe of planetesimal-scale melting and its subsequent cooling history. Currently, precise knowledge about the initial 26Al abundance [(26Al/27Al)0] exists only for the oldest known solids, calcium aluminum-rich inclusions (CAIs) – the so-called canonical value. We have determined the 26Al/27Al of three angrite meteorites, D’Orbigny, Sahara 99555 and NWA 1670, at their time of crystallization, which corresponds to (3.98 ± 0.15)×10−7, (3.64 ± 0.18)×10−7, and (5.92 ± 0.59)×10−7, respectively. Combined with a newly determined absolute U-corrected Pb–Pb age for NWA 1670 of 4564.39 ± 0.24 Ma and published U-corrected Pb–Pb ages for the other two angrites, this allows us to calculate an initial (26Al/27Al)0 of (1.33−0.18+0.21)×10−5 for the angrite parent body (APB) precursor material at the time of CAI formation, a value four times lower than the accepted canonical value of 5.25 × 10−5. Based on their similar 54Cr/52Cr ratios, most inner solar system materials likely accreted from material containing a similar 26Al/27Al ratio as the APB precursor at the time of CAI formation. To satisfy the abundant evidence for widespread planetesimal differentiation, the subcanonical 26Al budget requires that differentiated planetesimals, and hence protoplanets, accreted rapidly within 0.25 ± 0.15 Ma of the formation of canonical CAIs. PMID:27429474

  18. Explosive Fabrication of Intermetallics In Ti-Al System from Nano-Al and Coarse-Ti Powders

    NASA Astrophysics Data System (ADS)

    Chikhradze, Mikheil; Gigineishvili, Akaki; Cikhradze, Nikoloz

    2011-12-01

    Theoretical and experimental Investigations of shock wave consolidation processes of Ti-Al nano sized and ultra-disperse powder compositions are discussed. For theoretical calculations of the shock wave loaded materials were used the hydrodynamic theory and experimental adiabatics of Ti and Al. The normal and tangential stresses in the cylindrical steel tube (containers of Ti-Al reaction mixtures) were estimated using the partial solutions of elasticity theory. The mixtures of ultra-disperse Ti and nano sized (max≤50 nm) Al powder compositions were consolidated to full or near-full density by explosive-compaction technology. The ammonium nitride based industrial explosives were used for generation of shock waves. To form ultra-fine grained bulk TiAl intermetallides with different compositions, ultra-disperse Ti particles were mixed with nano-crystalline Al. Each reaction mixture was placed in a sealed container and explosively compacted using a normal and cylindrical detonation set-up. Explosive compaction experiments were performed in range of pressure impulse (5-20 GPA) at elevated temperatures. X-ray diffraction (XRD), structural investigations (SEM) and micro-hardness measurements were used to characterize the intermetallides phase composition and mechanical properties. The results of analysis revealing the effects of the compacting conditions and precursor particles sizes, affecting the consolidation and the properties of this new ultra high performance alloys are discussed.

  19. Brain amyloidosis ascertainment from cognitive, imaging, and peripheral blood protein measures

    PubMed Central

    Hwang, Kristy S.; Avila, David; Elashoff, David; Kohannim, Omid; Teng, Edmond; Sokolow, Sophie; Jack, Clifford R.; Jagust, William J.; Shaw, Leslie; Trojanowski, John Q.; Weiner, Michael W.; Thompson, Paul M.

    2015-01-01

    Background: The goal of this study was to identify a clinical biomarker signature of brain amyloidosis in the Alzheimer's Disease Neuroimaging Initiative 1 (ADNI1) mild cognitive impairment (MCI) cohort. Methods: We developed a multimodal biomarker classifier for predicting brain amyloidosis using cognitive, imaging, and peripheral blood protein ADNI1 MCI data. We used CSF β-amyloid 1–42 (Aβ42) ≤192 pg/mL as proxy measure for Pittsburgh compound B (PiB)-PET standard uptake value ratio ≥1.5. We trained our classifier in the subcohort with CSF Aβ42 but no PiB-PET data and tested its performance in the subcohort with PiB-PET but no CSF Aβ42 data. We also examined the utility of our biomarker signature for predicting disease progression from MCI to Alzheimer dementia. Results: The CSF training classifier selected Mini-Mental State Examination, Trails B, Auditory Verbal Learning Test delayed recall, education, APOE genotype, interleukin 6 receptor, clusterin, and ApoE protein, and achieved leave-one-out accuracy of 85% (area under the curve [AUC] = 0.8). The PiB testing classifier achieved an AUC of 0.72, and when classifier self-tuning was allowed, AUC = 0.74. The 36-month disease-progression classifier achieved AUC = 0.75 and accuracy = 71%. Conclusions: Automated classifiers based on cognitive and peripheral blood protein variables can identify the presence of brain amyloidosis with a modest level of accuracy. Such methods could have implications for clinical trial design and enrollment in the near future. Classification of evidence: This study provides Class II evidence that a classification algorithm based on cognitive, imaging, and peripheral blood protein measures identifies patients with brain amyloid on PiB-PET with moderate accuracy (sensitivity 68%, specificity 78%). PMID:25609767

  20. Long-Term Results of Conformal Radiotherapy for Progressive Airway Amyloidosis

    SciTech Connect

    Truong, Minh Tam; Kachnic, Lisa A.; Grillone, Gregory A.; Bohrs, Harry K.; Lee, Richard; Sakai, Osamu; Berk, John L.

    2012-06-01

    Purpose: To evaluate the efficacy of conformal external beam radiotherapy (RT) for local control of progressive airway amyloidosis. Methods and Materials: We conducted a retrospective review of patients with biopsy-proven progressive airway amyloidosis treated with conformal RT between 2000 and 2006 at Boston Medical Center. The patients were evaluated for performance status and pulmonary function, with computed tomography and endoscopy after RT compared with the pretreatment studies. Local control was defined as the lack of progression of airway wall thickening on computed tomography imaging and stable endobronchial deposits by endoscopy. Results: A total of 10 symptomatic airway amyloidosis patients (3 laryngeal and 7 tracheobronchial) received RT to a median total dose of 20 Gy in 10 fractions within 2 weeks. At a median follow-up of 6.7 years (range, 1.5-10.3), 8 of the 10 patients had local control. The remaining 2 patients underwent repeat RT 6 and 8.4 months after initial RT, 1 for persistent bronchial obstruction and 1 for progression of subglottic amyloid disease with subsequent disease control. The Eastern Cooperative Oncology Group performance status improved at a median of 18 months after RT compared with the baseline values, from a median score of 2 to a median of 1 (p = .035). Airflow (forced expiratory volume in 1 second) measurements increased compared with the baseline values at each follow-up evaluation, reaching a 10.7% increase (p = .087) at the last testing (median duration, 64.8 months). Acute toxicity was limited to Grade 1-2 esophagitis, occurring in 40% of patients. No late toxicity was observed. Conclusions: RT prevented progressive amyloid deposition in 8 of 10 patients, resulting in a marginally increased forced expiratory volume in 1 second, and improved functional capacity, without late morbidity.

  1. Association of Cerebral Amyloidosis, Blood Pressure, and Neuronal Injury with Late-Life Onset Depression

    PubMed Central

    Byun, Min Soo; Choe, Young Min; Sohn, Bo Kyung; Yi, Dahyun; Han, Ji Young; Park, Jinsick; Choi, Hyo Jung; Baek, Hyewon; Lee, Jun Ho; Kim, Hyun Jung; Kim, Yu Kyeong; Yoon, Eun Jin; Sohn, Chul-Ho; Woo, Jong Inn; Lee, Dong Young

    2016-01-01

    Previous literature suggests that Alzheimer's disease (AD) process may contribute to late-life onset depression (LLOD). Therefore, we investigated the association of LLOD with cerebral amyloidosis and neuronal injury, the two key brain changes in AD, along with vascular risks. Twenty nine non-demented individuals who first experienced major depressive disorder (MDD) after age of 60 years were included as LLOD subjects, and 27 non-demented elderly individuals without lifetime experience of MDD were included as normal controls (NC). Comorbid mild cognitive impairment (MCI) was diagnosed in 48% of LLOD subjects and in 0% of NC. LLOD, irrespective of comorbid MCI diagnosis, was associated with prominent prefrontal cortical atrophy. Compared to NC, LLOD subjects with comorbid MCI (LLODMCI) showed increased cerebral 11C-Pittsburg compound B (PiB) retention and plasma beta-amyloid 1–40 and 1–42 peptides, as measures of cerebral amyloidosis; and, such relationship was not observed in overall LLOD or LLOD without MCI (LLODwoMCI). LLOD subjects, particularly the LLODwoMCI, had higher systolic blood pressure (SBP) than NC. When analyzed in the same multiple logistic regression model that included prefrontal gray matter (GM) density, cerebral amyloidosis, and SBP as independent variables, only prefrontal GM density showed a significant independent association with LLOD regardless of MCI comorbidity status. Our findings suggest AD process might be related to LLOD via prefrontal neuronal injury in the MCI stage, whereas vascular processes—SBP elevation, in particular—are associated with LLOD via prefrontal neuronal injury even in cognitively intact or less impaired individuals. PMID:27790137

  2. Research on numerical control system based on S3C2410 and MCX314AL

    NASA Astrophysics Data System (ADS)

    Ren, Qiang; Jiang, Tingbiao

    2008-10-01

    With the rapid development of micro-computer technology, embedded system, CNC technology and integrated circuits, numerical control system with powerful functions can be realized by several high-speed CPU chips and RISC (Reduced Instruction Set Computing) chips which have small size and strong stability. In addition, the real-time operating system also makes the attainment of embedded system possible. Developing the NC system based on embedded technology can overcome some shortcomings of common PC-based CNC system, such as the waste of resources, low control precision, low frequency and low integration. This paper discusses a hardware platform of ENC (Embedded Numerical Control) system based on embedded processor chip ARM (Advanced RISC Machines)-S3C2410 and DSP (Digital Signal Processor)-MCX314AL and introduces the process of developing ENC system software. Finally write the MCX314AL's driver under the embedded Linux operating system. The embedded Linux operating system can deal with multitask well moreover satisfy the real-time and reliability of movement control. NC system has the advantages of best using resources and compact system with embedded technology. It provides a wealth of functions and superior performance with a lower cost. It can be sure that ENC is the direction of the future development.

  3. Characteristics of MCrAlY coatings sprayed by high velocity oxygen-fuel spraying system

    SciTech Connect

    Itoh, Y.; Saitoh, M.; Tamura, M.

    2000-01-01

    High velocity oxygen-fuel (HVOF) spraying system in open air has been established for producing the coatings that are extremely clean and dense. It is thought that the HVOF sprayed MCrAlY (M is Fe, Ni and/or Co) coatings can be applied to provide resistance against oxidation and corrosion to the hot parts of gas turbines. Also, it is well known that the thicker coating can be sprayed in comparison with any other thermal spraying systems due to improved residual stresses. However, thermal and mechanical properties of HVOF coatings have not been clarified. Especially, the characteristics of residual stress, that are the most important property from the view point of production technique, have not been made clear. In this paper, the mechanical properties of HVOF sprayed MCrAlY coatings were measured in both the case of as-sprayed and heat-treated coatings in comparison with a vacuum plasma sprayed MCrAlY coatings. It was confirmed that the mechanical properties of HVOF sprayed MCrAlY coatings could be improved by a diffusion heat treatment to equate the vacuum plasma sprayed MCrAlY coatings. Also, the residual stress characteristics were analyzed using a deflection measurement technique and a X-ray technique. The residual stress of HVOF coating was reduced by the shot-peening effect comparable to that of a plasma spray system in open air. This phenomena could be explained by the reason that the HVOF sprayed MCrAlY coating was built up by poorly melted particles.

  4. Beta 2-microglobulin amyloidosis presenting as esophageal perforation in a hemodialysis patient.

    PubMed

    Khan, G A; Lewis, F I; Dasgupta, M

    1997-01-01

    A 45-year-old male with hypertensive end-stage renal disease and on maintenance hemodialysis for 13 years is reported. He presented with life-threatening hematemesis, secondary to esophageal rupture. Immunohistological staining and electron microscopy examination of the esophageal perforation showed depositions of beta 2-microglobulin (beta 2-M) amyoloid. The unique aspect presented here is the localized esophageal involvement with beta 2-M amyloidosis. This is the first reported patient with esophageal perforation, due to the deposition and infiltration of the lower esophagus with beta 2-M which predisposed to its rupture. PMID:9426849

  5. [Notalgia paresthetica, "posterior pigmented pruritic patch" and macular amyloidosis. Three stages of a disease].

    PubMed

    Cerroni, L; Kopera, D; Soyer, H P; Kerl, H

    1993-12-01

    We report on nine cases of notalgia paresthetica, a cutaneous condition that has rarely been described in the dermatological literature and is characterized by localized pruritus, burning and hyperesthesia and/or paresthesia on the back. Histological and immunohistochemical studies have not clarified the pathogenesis of this disease. Several factors might be involved in various cases, including increased cutaneous innervation and neuropathy. The so-called posterior pigmented pruritic patch and macular amyloidosis may be considered as progressive evolutional stages of notalgia paresthetica. PMID:8113041

  6. High Temperature Aerogels in the Al2O3-SiO2 System

    NASA Technical Reports Server (NTRS)

    Hurwitz, Frances I.; Aranda, Denisse V.; Gallagher, Meghan E.

    2008-01-01

    Al2O3-SiO2 aerogels are of interest as constituents of thermal insulation systems for use at high temperatures. Al2O3 and mullite aerogels are expected to crystallize at higher temperatures than their SiO2 counterparts, hence avoiding the shrinkages that accompany the formation of lower temperature SiO2 phases and preserving pore structures into higher temperature regimes. The objective of this work is to determine the influence of processing parameters on shrinkage, gel structure (including surface area, pore size and distribution) and pyrolysis behavior.

  7. Modeling and simulation of AlN bulk sublimation growth systems

    NASA Astrophysics Data System (ADS)

    Wu, Bei; Ma, Ronghui; Zhang, Hui; Prasad, Vish

    2004-05-01

    In this paper, we have developed a numerical model to simulate two AlN sublimation growth systems. Temperature distributions in the growth cell for resistance and induction heating systems are presented and compared. The growth rate has been predicted and compared with experimental data. An anisotropic thermomechanical stress model is also developed to predict the thermal stress distribution in the as-grown crystal, with or without contact with the crucible wall.

  8. Top-level modeling of an als system utilizing object-oriented techniques

    NASA Astrophysics Data System (ADS)

    Rodriguez, L. F.; Kang, S.; Ting, K. C.

    The possible configuration of an Advanced Life Support (ALS) System capable of supporting human life for long-term space missions continues to evolve as researchers investigate potential technologies and configurations. To facilitate the decision process the development of acceptable, flexible, and dynamic mathematical computer modeling tools capable of system level analysis is desirable. Object-oriented techniques have been adopted to develop a dynamic top-level model of an ALS system.This approach has several advantages; among these, object-oriented abstractions of systems are inherently modular in architecture. Thus, models can initially be somewhat simplistic, while allowing for adjustments and improvements. In addition, by coding the model in Java, the model can be implemented via the World Wide Web, greatly encouraging the utilization of the model. Systems analysis is further enabled with the utilization of a readily available backend database containing information supporting the model. The subsystem models of the ALS system model include Crew, Biomass Production, Waste Processing and Resource Recovery, Food Processing and Nutrition, and the Interconnecting Space. Each subsystem model and an overall model have been developed. Presented here is the procedure utilized to develop the modeling tool, the vision of the modeling tool, and the current focus for each of the subsystem models.

  9. Top-level modeling of an ALS system utilizing object-oriented techniques.

    PubMed

    Rodriguez, L F; Kang, S; Ting, K C

    2003-01-01

    The possible configuration of an Advanced Life Support (ALS) System capable of supporting human life for long-term space missions continues to evolve as researchers investigate potential technologies and configurations. To facilitate the decision process the development of acceptable, flexible, and dynamic mathematical computer modeling tools capable of system level analysis is desirable. Object-oriented techniques have been adopted to develop a dynamic top-level model of an ALS system. This approach has several advantages; among these, object-oriented abstractions of systems are inherently modular in architecture. Thus, models can initially be somewhat simplistic, while allowing for adjustments and improvements. In addition, by coding the model in Java, the model can be implemented via the World Wide Web, greatly encouraging the utilization of the model. Systems analysis is further enabled with the utilization of a readily available backend database containing information supporting the model. The subsystem models of the ALS system model include Crew, Biomass Production, Waste Processing and Resource Recovery, Food Processing and Nutrition, and the Interconnecting Space. Each subsystem model and an overall model have been developed. Presented here is the procedure utilized to develop the modeling tool, the vision of the modeling tool, and the current focus for each of the subsystem models. PMID:14503522

  10. RASC-AL (Revolutionary Aerospace Systems Concepts-Academic Linkage): 2002 Advanced Concept Design Presentation

    NASA Technical Reports Server (NTRS)

    2002-01-01

    The Revolutionary Aerospace Systems Concepts-Academic Linkage (RASC-AL) is a program of the Lunar and Planetary Institute (LPI) in collaboration with the Universities Space Research Association's (USRA) ICASE institute through the NASA Langley Research Center. The RASC-AL key objectives are to develop relationships between universities and NASA that lead to opportunities for future NASA research and programs, and to develop aerospace systems concepts and technology requirements to enable future NASA missions. The program seeks to look decades into the future to explore new mission capabilities and discover what's possible. NASA seeks concepts and technologies that can make it possible to go anywhere, at anytime, safely, reliably, and affordably to accomplish strategic goals for science, exploration, and commercialization. University teams were invited to submit research topics from the following themes: Human and Robotic Space Exploration, Orbital Aggregation & Space Infrastructure Systems (OASIS), Zero-Emissions Aircraft, and Remote Sensing. RASC-AL is an outgrowth of the HEDS-UP (University Partners) Program sponsored by the LPI. HEDS-UP was a program of the Lunar and Planetary Institute designed to link universities with NASA's Human Exploration and Development of Space (HEDS) enterprise. The first RASC-AL Forum was held November 5-8, 2002, at the Hilton Cocoa Beach Oceanfront Hotel in Cocoa Beach, Florida. Representatives from 10 university teams presented student research design projects at this year's Forum. Each team contributed a written report and these reports are presented.

  11. The Microstructure-Processing-Property Relationships in an Al Matrix Composite System Reinforced by Al-Cu-Fe Alloy Particles

    SciTech Connect

    Tang, Fei

    2004-01-01

    Metal matrix composites (MMC), especially Al matrix composites, received a lot of attention during many years of research because of their promise for the development of automotive and aerospace materials with improved properties and performance, such as lighter weight and better structural properties, improved thermal conductivity and wear resistance. In order to make the MMC materials more viable in various applications, current research efforts on the MMCs should continue to focus on two important aspects, including improving the properties of MMCs and finding more economical techniques to produce MMCs. Solid state vacuum sintering was studied in tap densified Al powder and in hot quasi-isostatically forged samples composed of commercial inert gas atomized or high purity Al powder, generated by a gas atomization reaction synthesis (GARS) technique. The GARS process results in spherical Al powder with a far thinner surface oxide. The overall results indicated the enhanced ability of GARS-processed Al and Al alloy powders for solid state sintering, which may lead to simplification of current Al powder consolidation processing methods. Elemental Al-based composites reinforced with spherical Al-Cu-Fe alloy powders were produced by quasi-isostatic forging and vacuum hot pressing (VHP) consolidation methods. Microstructures and tensile properties of AYAl-Cu-Fe composites were characterized. It was proved that spherical Al-Cu-Fe alloy powders can serve as an effective reinforcement particulate for elemental Al-based composites, because of their high hardness and a preferred type of matrix/reinforcement interfacial bonding, with reduced strain concentration around the particles. Ultimate tensile strength and yield strength of the composites were increased over the corresponding Al matrix values, far beyond typical observations. This remarkable strengthening was achieved without precipitation hardening and without severe strain hardening during consolidation because of

  12. Ultrasound assisted combustion synthesis of TiC in Al-Ti-C system.

    PubMed

    Liu, Zhiwei; Rakita, Milan; Xu, Wilson; Wang, Xiaoming; Han, Qingyou

    2015-11-01

    This research investigated the effects of high-intensity ultrasound on the combustion synthesis of TiC particles in Al-Ti-C system. The process involved that high-intensity ultrasound was applied on the surface of a compacted Al-Ti-C pellet directly through a Nb probe during the thermal explosion reaction. By comparing with the sample without ultrasonic treatment, it was found that the thermal explosion reaction for synthesizing TiC phase could take place thoroughly in the ultrasonically treated sample. During the process of synthesizing TiC phase, the dissolution of solid graphite particles into the Al-Ti melt, as well as the nucleation and growth of TiC particles could be promoted effectively due to the effects of ultrasound, leading to an enhancement of the formation of TiC particles. Ultrasound assisted combustion synthesis as a simple and effective approach was proposed for synthesizing materials in this research.

  13. Experimental study and thermodynamic modeling of the Al-Co-Cr-Ni system

    NASA Astrophysics Data System (ADS)

    Gheno, Thomas; Liu, Xuan L.; Lindwall, Greta; Liu, Zi-Kui; Gleeson, Brian

    2015-10-01

    A thermodynamic database for the Al-Co-Cr-Ni system is built via the Calphad method by extrapolating re-assessed ternary subsystems. A minimum number of quaternary parameters are included, which are optimized using experimental phase equilibrium data obtained by electron probe micro-analysis and x-ray diffraction analysis of NiCoCrAlY alloys spanning a wide compositional range, after annealing at 900 °C, 1100 °C and 1200 °C, and water quenching. These temperatures are relevant to oxidation and corrosion resistant MCrAlY coatings, where M corresponds to some combination of nickel and cobalt. Comparisons of calculated and measured phase compositions show excellent agreement for the β-γ equilibrium, and good agreement for three-phase β-γ-σ and β-γ-α equilibria. An extensive comparison with existing Ni-base databases (TCNI6, TTNI8, NIST) is presented in terms of phase compositions.

  14. Heterogeneous distribution of 26Al at the birth of the solar system: Evidence from refractory grains and inclusions

    NASA Astrophysics Data System (ADS)

    Krot, A. N.; Makide, K.; Nagashima, K.; Huss, G. R.; Ogliore, R. C.; Ciesla, F. J.; Yang, L.; Hellebrand, E.; Gaidos, E.

    2012-12-01

    Abstract-We review recent results on O- and Mg-isotope compositions of refractory grains (corundum, hibonite) and calcium, aluminum-rich inclusions (CAIs) from unequilibrated ordinary and carbonaceous chondrites. We show that these refractory objects originated in the presence of nebular gas enriched in 16O to varying degrees relative to the standard mean ocean water value: the Δ17OSMOW value ranges from approximately -16‰ to -35‰, and recorded heterogeneous distribution of 26<span class="hlt">Al</span> in their formation region: the inferred (26<span class="hlt">Al</span>/27<span class="hlt">Al</span>)0 ranges from approximately 6.5 × 10-5 to <2 × 10-6. There is no correlation between O- and Mg-isotope compositions of the refractory objects: 26<span class="hlt">Al</span>-rich and 26<span class="hlt">Al</span>-poor refractory objects have similar O-isotope compositions. We suggest that 26<span class="hlt">Al</span> was injected into the 26<span class="hlt">Al</span>-poor collapsing protosolar molecular cloud core, possibly by a wind from a neighboring massive star, and was later homogenized in the protoplanetary disk by radial mixing, possibly at the canonical value of 26<span class="hlt">Al</span>/27<span class="hlt">Al</span> ratio (approximately 5 × 10-5). The 26<span class="hlt">Al</span>-rich and 26<span class="hlt">Al</span>-poor refractory grains and inclusions represent different generations of refractory objects, which formed prior to and during the injection and homogenization of 26<span class="hlt">Al</span>. Thus, the duration of formation of refractory grains and CAIs cannot be inferred from their 26<span class="hlt">Al</span>-26Mg systematics, and the canonical (26<span class="hlt">Al</span>/27<span class="hlt">Al</span>)0 does not represent the initial abundance of 26<span class="hlt">Al</span> in the solar <span class="hlt">system</span>; instead, it may or may not represent the average abundance of 26<span class="hlt">Al</span> in the fully formed disk. The latter depends on the formation time of CAIs with the canonical 26<span class="hlt">Al</span>/27<span class="hlt">Al</span> ratio relative to the timing of complete delivery of stellar 26<span class="hlt">Al</span> to the solar <span class="hlt">system</span>, and the degree of its subsequent homogenization in the disk. The injection of material containing 26<span class="hlt">Al</span> resulted in no observable changes in O-isotope composition of the solar <span class="hlt">system</span>. Instead, the variations in O-isotope compositions between individual</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/22426397','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/22426397"><span id="translatedtitle">Selective degeneration of septal and hippocampal GABAergic neurons in a mouse model of <span class="hlt">amyloidosis</span> and tauopathy.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Loreth, Desirée; Ozmen, Laurence; Revel, Florent G; Knoflach, Frédéric; Wetzel, Philine; Frotscher, Michael; Metzger, Friedrich; Kretz, Oliver</p> <p>2012-07-01</p> <p>Alzheimer's disease (AD) is a neurodegenerative disorder characterized by brain accumulation of amyloid-β peptide and neurofibrillary tangles, which are believed to initiate a pathological cascade that results in progressive impairment of cognitive functions and eventual neuronal death. To obtain a mouse model displaying the typical AD histopathology of <span class="hlt">amyloidosis</span> and tauopathy, we generated a triple-transgenic mouse line (TauPS2APP) by overexpressing human mutations of the amyloid precursor protein, presenilin2 and tau genes. Stereological analysis of TauPS2APP mice revealed significant neurodegeneration of GABAergic septo-hippocampal projection neurons as well as their target cells, the GABAergic hippocampal interneurons. In contrast, the cholinergic medial septum neurons remained unaffected. Moreover, the degeneration of hippocampal GABAergic interneurons was dependent on the hippocampal subfield and interneuronal subtype investigated, whereby the dentate gyrus and the NPY-positive interneurons, respectively, were most strongly affected. Neurodegeneration was also accompanied by a change in the mRNA expression of markers for inhibitory interneurons. In line with the loss of inhibitory neurons, we observed functional changes in TauPS2APP mice relative to WT mice, with strongly enhanced long-term potentiation in the medial-perforant pathway input to the dentate gyrus, and stereotypic hyperactivity. Our data indicate that inhibitory neurons are the targets of neurodegeneration in a mouse model of <span class="hlt">amyloidosis</span> and tauopathy, thus pointing to a possible role of the inhibitory network in the pathophysiological and functional cascade of Alzheimer's disease.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/17013609','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/17013609"><span id="translatedtitle">Role of <span class="hlt">amyloidosis</span> in determining the prognosis of dialyzed patients with rheumatoid arthritis.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Sanai, Toru; Nanishi, Fumio; Nagata, Masatoshi; Hirano, Tadashi; Suematsu, Eiichi; Esaki, Yukio; Miyahara, Hisaaki; Iida, Mitsuo</p> <p>2007-02-01</p> <p>The role of secondary <span class="hlt">amyloidosis</span> in determining the prognosis of dialyzed patients with rheumatoid arthritis (RA) was examined in 22 patients with a mean age of 60.1 years included 21 renal <span class="hlt">amyloidosis</span>. RA duration until the start of dialysis was 19.5 +/- 7.2 years and the observation period after introduction 27.1 +/- 26.4 months. Of the 14 dead cases, four died due to sepsis, three due to gastrointestinal tract bleeding, two due to congestive heart failure, and eight cases died within 5 months after starting dialysis. When comparing the eight survivors and the nine non-survivors who died within 2 years after the start of dialysis, the former patients showed significantly higher serum albumin, and lower electrocardiogram score and cardiothoracic ratios at the time of introduction to dialysis. The careful prevention and treatment of infection, cerebrovascular and/or gastrointestinal tract complications seem to be necessary to improve the prognosis of RA patients after the initiation of renal replacement therapy.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4215215','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4215215"><span id="translatedtitle">Methylene Blue Modulates β-Secretase, Reverses Cerebral <span class="hlt">Amyloidosis</span>, and Improves Cognition in Transgenic Mice*</span></a></p> <p><a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pmc">PubMed Central</a></p> <p>Mori, Takashi; Koyama, Naoki; Segawa, Tatsuya; Maeda, Masahiro; Maruyama, Nobuhiro; Kinoshita, Noriaki; Hou, Huayan; Tan, Jun; Town, Terrence</p> <p>2014-01-01</p> <p>Amyloid precursor protein (APP) proteolysis is required for production of amyloid-β (Aβ) peptides that comprise β-amyloid plaques in the brains of patients with Alzheimer disease (AD). Here, we tested whether the experimental agent methylene blue (MB), used for treatment of methemoglobinemia, might improve AD-like pathology and behavioral deficits. We orally administered MB to the aged transgenic PSAPP mouse model of cerebral <span class="hlt">amyloidosis</span> and evaluated cognitive function and cerebral amyloid pathology. Beginning at 15 months of age, animals were gavaged with MB (3 mg/kg) or vehicle once daily for 3 months. MB treatment significantly prevented transgene-associated behavioral impairment, including hyperactivity, decreased object recognition, and defective spatial working and reference memory, but it did not alter nontransgenic mouse behavior. Moreover, brain parenchymal and cerebral vascular β-amyloid deposits as well as levels of various Aβ species, including oligomers, were mitigated in MB-treated PSAPP mice. These effects occurred with inhibition of amyloidogenic APP proteolysis. Specifically, β-carboxyl-terminal APP fragment and β-site APP cleaving enzyme 1 protein expression and activity were attenuated. Additionally, treatment of Chinese hamster ovary cells overexpressing human wild-type APP with MB significantly decreased Aβ production and amyloidogenic APP proteolysis. These results underscore the potential for oral MB treatment against AD-related cerebral <span class="hlt">amyloidosis</span> by modulating the amyloidogenic pathway. PMID:25157105</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/cgi-bin/nph-data_query?bibcode=2015APS..MARA16004N&link_type=ABSTRACT','NASAADS'); return false;" href="http://adsabs.harvard.edu/cgi-bin/nph-data_query?bibcode=2015APS..MARA16004N&link_type=ABSTRACT"><span id="translatedtitle">Computational materials design of negative effective U <span class="hlt">system</span> in the hole-doped Delafossite of Cu<span class="hlt">Al</span>O2, Ag<span class="hlt">Al</span>O2 and Au<span class="hlt">Al</span>O2</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Nakanishi, Akitaka; Fukushima, Tetsuya; Uede, Hiroki; Katayama-Yoshida, Hiroshi</p> <p>2015-03-01</p> <p>In order to realize the super-high-TC superconductors (TC>1,000K) based on the general design rules for the negative Ueff <span class="hlt">system</span>, we have performed computational materials design for theUeff<0 <span class="hlt">system</span> in the hole-doped two-dimensional (2D) Delafossite Cu<span class="hlt">Al</span>O2, Ag<span class="hlt">Al</span>O2 and Au<span class="hlt">Al</span>O2 from the first principles. We find the interesting chemical trend of TC in 2D and 3D <span class="hlt">systems</span>; where the TC increases exponentially in the weak coupling regime (|Ueff (-0.44eV)|< W(2eV), W is the band width) for hole-doped CuFeS2, then the TC goes through a maximum when |Ueff (-4.88eV, -4.14eV)| ~ W (2.8eV, 3.5eV) for hole-doped Ag<span class="hlt">Al</span>O2 and Au<span class="hlt">Al</span>O2, and the TC decreases with increasing |Ueff|in strong coupling regime, where |Ueff (-4.53eV)|> W(1.7eV) for hole-doped Cu<span class="hlt">Al</span>O2</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2012MS%26E...35a2009J','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2012MS%26E...35a2009J"><span id="translatedtitle">Qualitative and quantitative description of microstructure of alloys from the Fe-<span class="hlt">Al</span> <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Jabłońska, M.; Mikuśkiewicz, M.; Tomaszewska, A.</p> <p>2012-05-01</p> <p>The paper presents the test results of qualitative and quantitative analysis of the structure of alloys from Fe-<span class="hlt">Al</span> <span class="hlt">system</span> after casting and heat treatment. The analysis were carried out for three alloys, with different content of <span class="hlt">Al</span> at.%: 36, 38, 48 at %, which were produced by melting and gravity casting. A quantitative evaluation of the structure was made with use of "MET-ILO" application on the basis of images acquired from a light microscope. Moreover the influence of the chemical composition and results of quantitative description of microstructure on the hardness of alloys from the aluminium - iron <span class="hlt">system</span> were analysed. The obtained research will be used for the development of mathematical models determining the influence of primary structure on the opportunities for plastic deformation of alloys. Structural examination was carried out using scanning electron microscopy (SEM) and scanning transmission electron microscopy (STEM). X-ray diffraction measurements were performed on this alloys.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://ntrs.nasa.gov/search.jsp?R=19760050746&hterms=pepper&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D90%26Ntt%3Dpepper','NASA-TRS'); return false;" href="http://ntrs.nasa.gov/search.jsp?R=19760050746&hterms=pepper&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D90%26Ntt%3Dpepper"><span id="translatedtitle">Effect of adsorbed films on friction of <span class="hlt">Al</span>2O3-metal <span class="hlt">systems</span></span></a></p> <p><a target="_blank" href="http://ntrs.nasa.gov/search.jsp">NASA Technical Reports Server (NTRS)</a></p> <p>Pepper, S. V.</p> <p>1976-01-01</p> <p>The kinetic friction of polycrystalline <span class="hlt">Al</span>2O3 sliding on Cu, Ni, and Fe in ultrahigh vacuum was studied as a function of the surface chemistry of the metal. Clean metal surfaces were exposed to O2, Cl2, C2H4, and C2H3Cl, and the change in friction due to the adsorbed species was observed. Auger electron spectroscopy assessed the elemental composition of the metal surface. It was found that the <span class="hlt">systems</span> exposed to Cl2 exhibited low friction, interpreted as the van der Waals force between the <span class="hlt">Al</span>2O3 and metal chloride. The generation of metal oxide by oxygen exposures resulted in an increase in friction, interpreted as due to strong interfacial bonds established by reaction of metal oxide with <span class="hlt">Al</span>2O3 to form the complex oxide (spinel). The only effect of C2H4 was to increase the friction of the Fe <span class="hlt">system</span>, but C2H3Cl exposures decreases friction in both Ni and Fe <span class="hlt">systems</span>, indicating the dominance of the chlorine over the ethylene complex on the surface</p> </li> </ol> <div class="pull-right"> <ul class="pagination"> <li><a href="#" onclick='return showDiv("page_1");'>«</a></li> <li><a href="#" onclick='return showDiv("page_15");'>15</a></li> <li><a href="#" onclick='return showDiv("page_16");'>16</a></li> <li class="active"><span>17</span></li> <li><a href="#" onclick='return showDiv("page_18");'>18</a></li> <li><a href="#" onclick='return showDiv("page_19");'>19</a></li> <li><a href="#" onclick='return showDiv("page_25");'>»</a></li> </ul> </div> </div><!-- col-sm-12 --> </div><!-- row --> </div><!-- page_17 --> <div id="page_18" class="hiddenDiv"> <div class="row"> <div class="col-sm-12"> <div class="pull-right"> <ul class="pagination"> <li><a href="#" onclick='return showDiv("page_1");'>«</a></li> <li><a href="#" onclick='return showDiv("page_16");'>16</a></li> <li><a href="#" onclick='return showDiv("page_17");'>17</a></li> <li class="active"><span>18</span></li> <li><a href="#" onclick='return showDiv("page_19");'>19</a></li> <li><a href="#" onclick='return showDiv("page_20");'>20</a></li> <li><a href="#" onclick='return showDiv("page_25");'>»</a></li> </ul> </div> </div> </div> <div class="row"> <div class="col-sm-12"> <ol class="result-class" start="341"> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/1985JAP....57.3241S','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/1985JAP....57.3241S"><span id="translatedtitle">Magnetic properties of the Fe<span class="hlt">Al</span>1 - xCux <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Saleh, A. S.; Mankikar, R. M.; Yoon, S.; Okpalugo, D. E.; Booth, J. G.</p> <p>1985-04-01</p> <p>The substitution of a 3d transition metal (T) for <span class="hlt">Al</span> in the equiatomic intermetallic compound Fe<span class="hlt">Al</span> results in the onset of ferromagnetism beyond a certain critical concentration of (T). The <span class="hlt">system</span> appears magnetically and structurally similar to the equivalent Co(GaT) alloys for which the critical concentration appeared to be related to an average electron concentration parameter. The present paper discusses Cu substitution in Fe<span class="hlt">Al</span>, i.e., the series Fe<span class="hlt">Al</span>1-xCux. It was found using x-ray and neutron diffraction techniques that the alloys remain single phase up to x=0.35 and that the crystallographic order remains B2 throughout. The site occupancy has also been determined and compared with the predictions of theoretical models. Ferromagnetism is observed to occur in the alloys with x>0.2. For these ferromagnetic alloys the inverse susceptibility against temperature curves (for T>TC) all have the same slope independent of composition suggesting an interpretation in terms of an itinerant model. This interpretation is also supported by a Rhodes-Wohlfarth plot. A region of superparamagnetism presages the onset of ferromagnetism and from this the size of the magnetic clusters is estimated to be 20-25 Å in radius.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/22255054','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/22255054"><span id="translatedtitle">Hydrogen migration dynamics in hydrated <span class="hlt">Al</span> clusters: The <span class="hlt">Al</span>{sub 17}{sup (−)}·H{sub 2}O <span class="hlt">system</span> as an example</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Álvarez-Barcia, S.; Flores, J. R.</p> <p>2014-02-28</p> <p>The <span class="hlt">Al</span>{sub m}{sup (−)}·(H{sub 2}O){sub n} <span class="hlt">systems</span> are known to undergo water splitting processes in the gas phase giving H{sub k}<span class="hlt">Al</span>{sub m}(OH){sub k}{sup (−)}·(H{sub 2}O){sub n−k} <span class="hlt">systems</span>, which can generate H{sub 2}. The migration of H atoms from one <span class="hlt">Al</span> atom to another on the cluster's surface is of critical importance to the mechanism of the complete H{sub 2} production process. We have applied a combination of Molecular Dynamics and Rice-Ramsperger-Kassel-Marcus theory including tunneling effects to study the gas-phase evolution of HAl{sub 17}(OH){sup (−)}, which can be considered a model <span class="hlt">system</span>. First, we have performed an extensive search for local minima and the connecting saddle points using a density functional theory method. It is found that in the water-splitting process <span class="hlt">Al</span>{sub 17}{sup (−)}·(H{sub 2}O) → HAl{sub 17}(OH){sup (−)}, the H atom which bonds to the <span class="hlt">Al</span> cluster losses rather quickly its excess energy, which is easily “absorbed” by the cluster because of its flexibility. This fact ultimately determines that long-range hydrogen migration is not a very fast process and that, probably, tunneling only plays a secondary role in the migration dynamics, at least for moderate energies. Reduction of the total energy results in the process being very much slowed down. The consequences on the possible mechanisms of H{sub 2} generation from the interaction of <span class="hlt">Al</span> clusters and water molecules are discussed.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/5390175','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/5390175"><span id="translatedtitle">Obtaining shrink-free mullite lightweight ceramics in the <span class="hlt">Al</span>F/sub 3/-<span class="hlt">Al</span>/sub 2/O/sub 3/-SiO/sub 2/ <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Goncharov, Yu.I.; Skomorokhin, V.Yu.</p> <p>1987-09-01</p> <p>Using the mullite-forming mechanism in the <span class="hlt">system</span> <span class="hlt">Al</span>F/sub 3/-<span class="hlt">Al</span>/sub 2/O/sub 3/-SiO/sub 2/ it is possible to synthesize mullite during firing of the goods without preheating the starting components. The solid phase synthesis of crystals of mullite leads to self-reinforcing of the ceramic material. The experimental batches of goods showed that it is possible to obtain mullite articles with an apparent density of 0.65-2.00 g/cm/sup 3/.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2000MMTB...31.1305L','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2000MMTB...31.1305L"><span id="translatedtitle">Examination of solidification pathways and the liquidus surface in the Nb-Ti-<span class="hlt">Al</span> <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Leonard, Keith J.; Mishurda, Joseph C.; Vasudevan, Vijay K.</p> <p>2000-12-01</p> <p>The solidification pathways, subsequent solid-state transformations, and the liquidus surface in the Nb-Ti-<span class="hlt">Al</span> <span class="hlt">system</span> have been examined as part of a larger investigation of phase equilibria in Nb-Ti-<span class="hlt">Al</span> intermetallic alloys. Fifteen alloys ranging in composition from 15 to 40 at. pct <span class="hlt">Al</span>, with Nb to Ti ratios of 4:1, 2:1, 1.5:1, 1:1, and 1:1.5, were prepared by arc melting and the as-cast microstructures were characterized by optical microscopy (OM), microhardness, X-ray diffraction (XRD), differential thermal analysis (DTA), backscattered electron imaging (BSEI), electron probe microanalysis (EPMA), and transmission electron microscopy (TEM). The results indicate that the range of primary β solidification is much wider than that indicated in previously reported liquidus surfaces, both experimental and calculated. Differential thermal analysis has identified the existence of a β to σ+γ transformation in three alloys where it was previously thought not to exist; confirmation was provided by high-temperature vacuum heat treatments in the single-phase β region followed by rapid quenching. The location of the boundary between the β, σ, and δ primary solidification fields has been redefined. A massive β → δ transformation, which was observed in the cast microstructure of a Nb-25Ti-25<span class="hlt">Al</span> alloy, was repeatable through cooling following homogenization. A β → δ+σ eutectoid-like transformation in the 25 at. pct <span class="hlt">Al</span> alloys, was detected by DTA and evaluated through microstructural analysis of heat-treated samples. Trends in the β phase with variations in composition were established for both lattice parameters and microhardness. As a result of this wider extent of the primary β solidification field, a greater possibility exists for microstructural control through thermal processing for alloys consisting of either σ+γ, β+σ, or β+δ phases.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/1993fttq.book.....K','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/1993fttq.book.....K"><span id="translatedtitle">Formation of three types of quasi-crystals in <span class="hlt">Al</span>-Pd-Mg <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Koshikawa, Naokiyo; Edagawa, Keiichi; Honda, Yuko; Takeuchi, Shin</p> <p>1993-04-01</p> <p>In the <span class="hlt">Al</span>-Pd-Mg <span class="hlt">system</span>, a Mackay-Icosahedron (MI) type Icosahedral (I) phase with the F-type superlattice order and a Decagonal (D) phase were found to form in a melt-quenched state, in addition to the Frank-Kasper (FK) type stable I-phase reported previously. This is the first example in which the three types of quasi-crystalline phases are formed in the same alloy <span class="hlt">system</span>. The formation range of the FK-type I-phase in melt-quenched state is rather wide: 5 - 20 at percent Pd and 20 - 45 at percent Mg. In contrast, the MI-type, I-phase and D-phase are formed in small composition ranges around <span class="hlt">Al</span>52Pd31Mg17 and <span class="hlt">Al</span>74Pd21Mg5, respectively. Electron diffraction studies showed that the period along the tenfold axis of the D-phase is about 1.6 nm. The formation and stability of the MI-type and FK-type I-phases were discussed in terms of a Hume-Rothery rule.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/6000894','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/6000894"><span id="translatedtitle">Computer analysis of phase diagrams and thermodynamic properties of cryolite based <span class="hlt">systems</span>: 1. The <span class="hlt">Al</span>F/sub 3/-LiF-NaF <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Saboungi, M.L.; Lin, P.I.; Cerisier, P.; Pelton, A.D.</p> <p>1980-09-01</p> <p>Calculations of the phase diagram of the <span class="hlt">Al</span>F/sub 3/-LiF-NaF <span class="hlt">system</span> up to 35 mol pct <span class="hlt">Al</span>F/sub 3/ and of the binary subsystems are presented. For the binary LiF-NaF, <span class="hlt">Al</span>F/sub 3/-NaF, <span class="hlt">Al</span>F/sub 3/-LiF, and the quasibinary Li/sub 3/<span class="hlt">Al</span>F/sub 6/-Na/sub 3/<span class="hlt">Al</span>F/sub 6/ <span class="hlt">systems</span>, thermodynamic properties of the unary and binary phases are used to numerically generate the corresponding phase diagram. The calculated and measured values are in good agreement. The broad data base thus constructed is used with equations from the conformal ionic solution theory to derive a priori phase equilibria of the <span class="hlt">Al</span>F/sub 3/-LiF-NaF <span class="hlt">system</span>. Ternary liquidus temperatures are calculated covering compositions outside the range of measurements. 47 references, 7 figures, 2 tables.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016JSSCh.243..168M','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016JSSCh.243..168M"><span id="translatedtitle">Investigation of the phase relations in the U-<span class="hlt">Al</span>-Ge ternary <span class="hlt">system</span>: Influence of the <span class="hlt">Al</span>/Ge substitution on the properties of the intermediate phases</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Moussa, C.; El Sayah, Z.; Chajewski, G.; Berche, A.; Dorcet, V.; Pikul, A. P.; Pasturel, M.; Joanny, L.; Stepnik, B.; Tougait, O.</p> <p>2016-11-01</p> <p>The phase relations within the U-<span class="hlt">Al</span>-Ge ternary <span class="hlt">system</span> were studied for two isothermal sections, at 673 K for the whole Gibbs triangle and at 1173 K for the concentration range 25-100 at% U. The identification of the phases, their composition ranges and stability were determined by x-ray powder diffraction, scanning electron microscopy coupled to energy dispersive spectroscopy and differential thermal analysis. The tie-lines and the solubility domains were determined for the U-Ge and U-<span class="hlt">Al</span> binaries, the UAl3-UGe3 solid-solution and for the unique ternary intermediate phase U3<span class="hlt">Al</span>2-xGe3+x. The experimental isopleth section of the pseudo-binary UAl3-UGe3 reveals an isomorphous solid solution based on the Cu3Au-type below the solidus. The U3<span class="hlt">Al</span>2-xGe3+x solid solution extends for -0.1≤x≤1.35 and -0.2≤x≤1.5 at 673 K and 1173 K respectively. It crystallizes in the I-centered tetragonal symmetry. The reciprocal lattice of several compositions of the U3<span class="hlt">Al</span>2-xGe3+x solid solution was examined by electron diffraction at room temperature, revealing the presence of a c-glide plane. Their crystal structure was refined by single crystal x-ray diffraction suggesting an isomorphous solid solution best described with the non-centrosymmetric space group I4cm in the paramagnetic domain. The magnetic measurements confirm the ferromagnetic ordering of the solid solution U3<span class="hlt">Al</span>2-xGe3+x with an increase of Tc with the <span class="hlt">Al</span> content. The thermal variation of the specific heat bear out the magnetic transitions with some delocalized character of the uranium 5f electrons.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/26344185','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/26344185"><span id="translatedtitle">On the microscopic dynamics of the 'Einstein solids' <span class="hlt">Al</span>V2<span class="hlt">Al</span>20 and GaV2<span class="hlt">Al</span>20, and of YV2<span class="hlt">Al</span>20: a benchmark <span class="hlt">system</span> for 'rattling' excitations.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Koza, Michael Marek; Mutka, Hannu; Okamoto, Yoshihiko; Yamaura, Jun-ichi; Hiroi, Zenji</p> <p>2015-10-14</p> <p>The inelastic response of AV2<span class="hlt">Al</span>20 (with A = <span class="hlt">Al</span>, Ga and Y) was probed by high-resolution inelastic neutron scattering experiments and density functional theory (DFT) based lattice dynamics calculations (LDC). Features characteristic of the dynamics of <span class="hlt">Al</span>, Ga and Y are established experimentally in the low-energy range of the compounds. In the stereotype 'Einstein-solid' compound <span class="hlt">Al</span>V2<span class="hlt">Al</span>20 we identify a unique spectral density extending up to 10 meV at 1.6 K. Its dominating feature is a peak centred at 2 meV at the base temperature. A very similar spectral distribution is established in GaV2<span class="hlt">Al</span>20 albeit the strong peak is located at 1 meV at 1.6 K. In YV2<span class="hlt">Al</span>20 signals characteristic of Y dynamics are located above 8 meV. The spectral distributions are reproduced by the DFT-based LDC and identified as a set of phonons. The response to temperature changes between 1.6 and ∼300 K is studied experimentally and the exceptionally vivid renormalization of the A characteristic modes in <span class="hlt">Al</span>V2<span class="hlt">Al</span>20 and GaV2<span class="hlt">Al</span>20 is quantified by following the energy of the strong peak. At about 300 K it is shifted to higher energies by 300% for A = <span class="hlt">Al</span> and 450% for A = Ga. The dynamics of A = Y in YV2<span class="hlt">Al</span>20 show a minor temperature effect. This holds in general for modes located above 10 meV in any of the compounds. They are associated with vibrations of the V2<span class="hlt">Al</span>20 matrix. Atomic potentials derived through DFT calculations indicate the propensity of A = <span class="hlt">Al</span> and Ga to a strong positive energy shift upon temperature increase by a high quartic component. The effect of the strong phonon renormalization on thermodynamic observables is computed on grounds of the LDC results. It is shown that through the hybridization of A = <span class="hlt">Al</span> and Ga with the V2<span class="hlt">Al</span>20 dynamics the matrix vibrations in the low-energy range follow this renormalization.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016ApPhL.108t1606L','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016ApPhL.108t1606L"><span id="translatedtitle">Formation mechanism for the nanoscale amorphous interface in pulse-welded <span class="hlt">Al</span>/Fe bimetallic <span class="hlt">systems</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Li, Jingjing; Yu, Qian; Zhang, Zijiao; Xu, Wei; Sun, Xin</p> <p>2016-05-01</p> <p>Pulse or impact welding traditionally has been referred to as "solid-state" welding. By integrating advanced interface characterizations and diffusion calculations, we report that the nanoscale amorphous interface in the pulse-welded <span class="hlt">Al</span>/Fe bimetallic <span class="hlt">system</span> is formed by rapid heating and melting of a thin <span class="hlt">Al</span> layer at the interface, diffusion of iron atoms in the liquid aluminum, and subsequent rapid quenching with diffused iron atoms in solution. This finding challenges the commonly held belief regarding the solid-state nature of the impact-based welding process for dissimilar metals. Elongated ultra-fine grains with high dislocation density and ultra-fine equiaxed grains also are observed in the weld interface vicinity on the steel and aluminum sides, respectively, which further confirms that melting and the subsequent recrystallization occurred on the aluminum side of the interface.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/22111950','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/22111950"><span id="translatedtitle">Elastic scattering measurements for {sup 7}Be+{sup 27}<span class="hlt">Al</span> <span class="hlt">system</span> at RIBRAS facility</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Morcelle, V.; Lichtenthaeler, R.; Morais, M. C.; Lepine-Szily, A.; Guimaraes, V.; Faria, P. N. de; Gasques, L.; Pires, K. C. C.; Condori, R. P.; Gomes, P. R. S.; Lubian, J.; Mendes, D. R. Jr.; Barioni, A.; Shorto, J. M. B.; Zamora, J. C.</p> <p>2013-05-06</p> <p>Elastic scattering angular distribution measurements of {sup 7}Be+{sup 27}<span class="hlt">Al</span> <span class="hlt">system</span> were performed at the laboratory energy of 15.6 MeV. The {sup 7}Be secondary beam was produced by the proton transfer reaction {sup 3}He({sup 6}Li,{sup 7}Be) and impinged on {sup 27}<span class="hlt">Al</span> and {sup 197}Au targets, using the Radioactive Ion Beam (RIB) facility, RIBRAS. The elastic angular distribution was obtained within the angular range of 15{sup 0} - 80{sup 0} at the center of mass frame. Optical model calculations have been performed using the Woods- Saxon form factors and the Sao Paulo potential to fit the experimental data. The total reaction cross section was derived.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.ncbi.nlm.nih.gov/pubmed/157110','PUBMED'); return false;" href="http://www.ncbi.nlm.nih.gov/pubmed/157110"><span id="translatedtitle">Concurrent feline immune-complex nephritis. Tubular antigen-positive and renal <span class="hlt">amyloidosis</span>.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Saegusa, S; Shimizu, F; Nagase, M; Kasegawa, A</p> <p>1979-08-01</p> <p>We describe tubular antigen-positive immune-complex nephritis in a case of feline renal <span class="hlt">amyloidosis</span>. Amyloid deposition was observed in mesangial area, and thickening of capillary walls was shown in the majority of the glomeruli. This case was also characterized with typical fluorescent granular depositions of cat IgG and C3 along the glomerular capillary walls as seen in human membranous glomerulonephritis. The fluorescent pattern of tubular antigen was identical with that of IgG and C3. Electron micrograph showed the thickening and irregularity of glomerular basement membranes, fusion of foot processes, and deposits of electron-dense or sometimes translucent materials, mostly in the intramembranous location. The causal sequence of the coincidental deposition of amyloid and immune complexes is discussed. PMID:157110</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/24881274','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/24881274"><span id="translatedtitle">[<span class="hlt">Amyloidosis</span> in turkeys (Meleagris gallopavo f. domestica)--a case report].</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Breuer, Wolfram; Moser, Hubert; De Souza-Pilz, Magdalena; Lüschow, Dörte; Hafner-Marx, Angela; Deischl, Karin; Hafez, Hafez Mohamed</p> <p>2014-01-01</p> <p>High prevalence of leg disorders in fattening meat turkey farm was observed. Four birds as well as tracheal and joint swabs were submitted to the Bavarian Health and Food Safety Authority in Oberschleissheim and to the Institute of Poultry Diseases, Faculty of Veterinary Medicine, Free University of Berlin. At the post-mortem, all birds showed an inflammation of the hock joints (intertarsal joint). The histopatholical investigations revealed a chronic inflammation of the joint and amyloid deposits in the joints in two cases as well as in different tissues (liver, spleen and kidneys) in another two cases. Using polymerase chain reaction, Ornithobacterium rhinotracheale-DNA could be detected in the examined tracheal and joint swabs. On the other hand, Mycoplasma gallisepticum- and Mycoplasma synoviae-DNA could not be detected. A causal correlation between the detected infectious agent and <span class="hlt">amyloidosis</span> in relation to the leg disorders were discussed.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2000JCrGr.211..485M','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2000JCrGr.211..485M"><span id="translatedtitle">Directional solidification processing of eutectic alloys in the Ni <span class="hlt">Al</span> V <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Milenkovic, S.; Coelho, A. A.; Caram, R.</p> <p>2000-04-01</p> <p>Intermetallic matrix composites (IMCs) offer attractive properties, such as high toughness of the metal coupled with low density, high modulus and high strength of the intermetallics. Among a large number of the intermetallics, a particular interest has been shown in the Ni<span class="hlt">Al</span> intermetallic compound, since it exhibits several advantages over the currently used nickel-based superalloys. Recently, there has been a renewed interest in directional solidification of the eutectic alloys as a concept of reinforcing intermetallics with in situ refractory metals. The present study is related to the study of the eutectic alloys in the ternary Ni<span class="hlt">Al</span>-V <span class="hlt">system</span>. The eutectic composition and temperature were accurately determined. It was concluded that the solidification behaviour of the Ni-<span class="hlt">Al</span>-V eutectic is strongly dependent on the growth conditions, namely growth rate and orientation, and that it can be easily modified. Also, it was observed that the orientation of the grain, i.e., the direction of growth is the determining factor in the lamellar/rod transition as well as in the morphology of the degenerated structure.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4323459','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4323459"><span id="translatedtitle">Digitally reinforced hematoxylin-eosin polarization technique in diagnosis of rectal <span class="hlt">amyloidosis</span></span></a></p> <p><a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pmc">PubMed Central</a></p> <p>Doganavsargil, Basak; Buberal, Gulruh Emiroglu; Toz, Huseyin; Sarsik, Banu; Pehlivanoglu, Burcin; Sezak, Murat; Sen, Sait</p> <p>2015-01-01</p> <p>AIM: To investigate the efficacy of the digitally reinforced hematoxylin-eosin polarization (DRHEP) technique for detection of <span class="hlt">amyloidosis</span> in rectal biopsies. METHODS: One hundred hematoxylin-eosin (HE) stained rectal biopsies with Congo-red (CR)-positive amyloid depositions and 50 control cases with CR-negative amyloid-mimicking areas were scanned blinded to the CR results for amyloid depositions under both bright and polarized light, and digitally photographed using the DRHEP technique, to accentuate the faint birefringence observed in HE slides under polarization. The results of DRHEP and HE evaluation were statistically correlated with CR polarization results with respect to presence and localization of amyloid deposits as well as amyloid types. RESULTS: Amyloid deposits showed yellowish-green birefringence by DRHEP, which allowed identification of <span class="hlt">amyloidosis</span> in 41 HE-unsuspected cases (P = 0.016), 31 of which only had vascular deposits. True positivity was higher, and false negativity and positivity were lower by DRHEP, compared to evaluation by HE (69%, 31%, and 0.8% vs 33%, 67%, and 33%, respectively; P < 0.0001). The sensitivity, specificity, accuracy, and positive and negative predictive values for DRHEP were 69%, 98%, 78.6%, 98.5%, and 61.25%, respectively. Reasons for DRHEP false negativity were presence of extensive background birefringence in 12 cases, absence of CR birefringent vessel in 3 cases, and missing of the tiny deposits in 9 cases, which could be improved by experience, especially in the latter case. No correlation was found between age, gender, sites of deposits, or amyloid types. CONCLUSION: The DRHEP technique improves diagnostic accuracy when used as an adjunct or a prior step to CR staining, especially for cases with limited tissues for further analysis. PMID:25684948</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/21448880','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/21448880"><span id="translatedtitle">{sup 60}Fe AND {sup 26}<span class="hlt">Al</span> IN CHONDRULES FROM UNEQUILIBRATED CHONDRITES: IMPLICATIONS FOR EARLY SOLAR <span class="hlt">SYSTEM</span> PROCESSES</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Mishra, R. K.; Goswami, J. N.; Rudraswami, N. G.; Tachibana, S.; Huss, G. R.</p> <p>2010-05-10</p> <p>The presence of about a dozen short-lived nuclides in the early solar <span class="hlt">system</span>, including {sup 60}Fe and {sup 26}<span class="hlt">Al</span>, has been established from isotopic studies of meteorite samples. An accurate estimation of solar <span class="hlt">system</span> initial abundance of {sup 60}Fe, a distinct product of stellar nucleosynthesis, is important to infer the stellar source of this nuclide. Previous studies in this regard suffered from the lack of exact knowledge of the time of formation of the analyzed meteorite samples. We present here results obtained from the first combined study of {sup 60}Fe and {sup 26}<span class="hlt">Al</span> records in early solar <span class="hlt">system</span> objects to remove this ambiguity. Chondrules from unequilibrated ordinary chondrites belonging to low petrologic grades were analyzed for their Fe-Ni and <span class="hlt">Al</span>-Mg isotope systematics. The <span class="hlt">Al</span>-Mg isotope data provide the time of formation of the analyzed chondrules relative to the first solar <span class="hlt">system</span> solids, the Ca-<span class="hlt">Al</span>-rich inclusions. The inferred initial {sup 60}Fe/{sup 56}Fe values of four chondrules, combined with their time of formation based on <span class="hlt">Al</span>-Mg isotope data, yielded a weighted mean value of (6.3 {+-} 2) x 10{sup -7} for solar <span class="hlt">system</span> initial {sup 60}Fe/{sup 56}Fe. This argues for a high-mass supernova as the source of {sup 60}Fe along with {sup 26}<span class="hlt">Al</span> and several other short-lived nuclides present in the early solar <span class="hlt">system</span>.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2014AJ....148...79Q','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2014AJ....148...79Q"><span id="translatedtitle"><span class="hlt">AL</span> Cassiopeiae: An F-type Contact Binary <span class="hlt">System</span> with a Cool Stellar Companion</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Qian, S.-B.; Zhou, X.; Zola, S.; Zhu, L.-Y.; Zhao, E.-G.; Liao, W.-P.; Leung, K.-C.</p> <p>2014-11-01</p> <p>According to the general catalog of variable stars, <span class="hlt">AL</span> Cas was classified as an EW-type eclipsing binary with a spectral type of B and an orbital period of P = 0.5005555 days. The first photometric light curves of the close binary in the B, V, R, and I bands are presented. New low-resolution spectra indicate that its spectral type is about F7 rather than B-type. A photometric analysis with the Wilson-Devinney method suggests that it is a contact binary (f = 39.3%) with a mass ratio of 0.61. Using 17 newly determined eclipse times together with those collected from the literature, we found that the observed-calculated (O - C) curve of <span class="hlt">AL</span> Cas shows a cyclic change with a period of 86.6 yr and an amplitude of 0.0181 days. The periodic variation was analyzed for the light-travel time effect via the presence of a third body. The mass of the third body was determined to be M 3sin i' = 0.29(± 0.05) M ⊙ when a total mass of 2.14 M ⊙ for <span class="hlt">AL</span> Cas is adopted. It is expected that the cool companion star may have played an important role in the origin and evolution of the <span class="hlt">system</span> by removing angular momentum from the central binary <span class="hlt">system</span> during early dynamical interaction and/or late dynamical evolution. This causes the original detached <span class="hlt">system</span> to have a low angular momentum and a short initial orbital period. Then it can evolve into the present contact configuration via a case A mass transfer.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/22342232','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/22342232"><span id="translatedtitle"><span class="hlt">AL</span> Cassiopeiae: An F-type contact binary <span class="hlt">system</span> with a cool stellar companion</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Qian, S.-B.; Zhou, X.; Zhu, L.-Y.; Zhao, E.-G.; Liao, W.-P.; Zola, S.; Leung, K.-C.</p> <p>2014-11-01</p> <p>According to the general catalog of variable stars, <span class="hlt">AL</span> Cas was classified as an EW-type eclipsing binary with a spectral type of B and an orbital period of P = 0.5005555 days. The first photometric light curves of the close binary in the B, V, R, and I bands are presented. New low-resolution spectra indicate that its spectral type is about F7 rather than B-type. A photometric analysis with the Wilson-Devinney method suggests that it is a contact binary (f = 39.3%) with a mass ratio of 0.61. Using 17 newly determined eclipse times together with those collected from the literature, we found that the observed–calculated (O – C) curve of <span class="hlt">AL</span> Cas shows a cyclic change with a period of 86.6 yr and an amplitude of 0.0181 days. The periodic variation was analyzed for the light-travel time effect via the presence of a third body. The mass of the third body was determined to be M {sub 3}sin i' = 0.29(± 0.05) M {sub ☉} when a total mass of 2.14 M {sub ☉} for <span class="hlt">AL</span> Cas is adopted. It is expected that the cool companion star may have played an important role in the origin and evolution of the <span class="hlt">system</span> by removing angular momentum from the central binary <span class="hlt">system</span> during early dynamical interaction and/or late dynamical evolution. This causes the original detached <span class="hlt">system</span> to have a low angular momentum and a short initial orbital period. Then it can evolve into the present contact configuration via a case A mass transfer.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2015PhDT.......131D','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2015PhDT.......131D"><span id="translatedtitle">Determining Phase Stability in the Co-<span class="hlt">Al</span>-W <span class="hlt">System</span> using First Principles Methods</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Dodge, Philip C.</p> <p></p> <p>Co-based superalloys are a promising new material for high strength, high temperature applications due to their gamma-gamma' microstructure. Within the Co-<span class="hlt">Al</span>-W ternary <span class="hlt">system</span>, the gamma' phase manifests as an L1 2 structure in a small temperature range. Determining the thermodynamic stability of the L12 phase relative to other phases in this <span class="hlt">system</span> is important for guiding the alloy development process. Density functional theory allows the direct calculation of free energy for these phases. The configurational energy component of the energy for the L12 structure was calculated via a cluster expansion, and the vibrational energy component for all structures was calculated in the quasiharmonic approximation. The results show that the L12 structures become stable at 600K and increase stability at higher temperatures, which has not been shown in previous work. The purpose of this thesis is both to show the encouraging research results in the Co-<span class="hlt">Al</span>-W <span class="hlt">system</span>, and also to be a useful guide for performing first principles calculations and connecting theory, VASP usage, and the utilization of the CASM code.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/143707','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/143707"><span id="translatedtitle">Nano-structured FGM of the <span class="hlt">system</span> <span class="hlt">Al</span>N-Ni</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Atarashiya, Koji; Uda, Masahiro</p> <p>1993-12-31</p> <p>A nano-structured FGM block of the <span class="hlt">system</span> <span class="hlt">Al</span>N-Ni was prepared by the method of powder metallurgy. The raw material i.e. an ultrafine particle, whose size was 200 nm in average diameter, was prepared by means of the arc-plasma processing that a vaporized substance was collected in a bag filter. A compositionally gradient mixture of a nickel ultrafine particle and an aluminum-aluminum nitride ultrafine particle was pressed (200 MPa) in a steel die, followed by two-step sintering (873K and 1273K) in flowing nitrogen under null pressure. In this process, the aluminum ultrafine particle reacted with nitrogen into aluminum nitride, but the nickel ultrafine particle did not react with any species. Thus, FGM block of the <span class="hlt">system</span> <span class="hlt">Al</span>N-Ni was completely accomplished. The techniques of an ultrafine-particle mixing and sintering without crystal-grain growth were invented. Generally speaking the preparation of FGM block especially in the metal nitride <span class="hlt">systems</span>, needs an extremely high temperature and high pressure. However, in this work using an ultrafine particle, the nano-structured FGM block was easily prepared. The nitriding reaction of aluminum is exothermic. Thus, it considerably progresses by self-propagating high temperature synthesis (SHS) when it has once begun. The structure and the compositional distributions of the FGM block were examined by SEM and EDX. The physical properties of the FGM block such as density, thermal expansion coefficient and micro harness were measured.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/1164045','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/1164045"><span id="translatedtitle">Solution-based thermodynamic modeling of the Ni-<span class="hlt">Al</span>-Mo <span class="hlt">system</span> using first-principles calculations</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Zhou, S H; Wang, Y; Chen, L -Q; Liu, Z -K; Napolitano, R E</p> <p>2014-09-01</p> <p>A solution-based thermodynamic description of the ternary Ni–Al–Mo <span class="hlt">system</span> is developed here, incorporating first-principles calculations and reported modeling of the binary Ni–<span class="hlt">Al</span>, Ni–Mo and Al–Mo <span class="hlt">systems</span>. To search for the configurations with the lowest energies of the N phase, the Alloy Theoretic Automated Toolkit (ATAT) was employed and combined with VASP. The liquid, bcc and γ-fcc phases are modeled as random atomic solutions, and the γ'-Ni3<span class="hlt">Al</span> phase is modeled by describing the ordering within the fcc structure using two sublattices, summarized as (<span class="hlt">Al</span>,Mo,Ni)0.75(<span class="hlt">Al</span>,Mo,Ni)0.25. Thus, γ-fcc and γ'-Ni3<span class="hlt">Al</span> are modeled with a single Gibbs free energy function with appropriate treatment of the chemical ordering contribution. In addition, notable improvements are the following: first, the ternary effects of Mo and <span class="hlt">Al</span> in the B2-Ni<span class="hlt">Al</span> and D0a-Ni3Mo phases, respectively, are considered; second, the N-Ni<span class="hlt">Al</span>8Mo3 phase is described as a solid solution using a three-sublattice model; third, the X-Ni14<span class="hlt">Al</span>75Mo11 phase is treated as a stoichiometric compound. Model parameters are evaluated using first-principles calculations of zero-Kelvin formation enthalpies and reported experimental data. In comparison with the enthalpies of formation for the compounds ψ-<span class="hlt">Al</span>Mo, θ-<span class="hlt">Al</span>8Mo3 and B2-Ni<span class="hlt">Al</span>, the first-principles results indicate that the N-Ni<span class="hlt">Al</span>8Mo3 phase, which is stable at high temperatures, decomposes into other phases at low temperature. Resulting phase equilibria are summarized in the form of isothermal sections and liquidus projections. To clearly identify the relationship between the γ-fcc and γ'-Ni3<span class="hlt">Al</span> phases in the ternary Ni–Al–Mo <span class="hlt">system</span>, the specific γ-fcc and γ'-Ni3<span class="hlt">Al</span> phase fields are plotted in x(<span class="hlt">Al</span>)–x(Mo)–T space for a temperature range 1200–1800 K.</p> </li> </ol> <div class="pull-right"> <ul class="pagination"> <li><a href="#" onclick='return showDiv("page_1");'>«</a></li> <li><a href="#" onclick='return showDiv("page_16");'>16</a></li> <li><a href="#" onclick='return showDiv("page_17");'>17</a></li> <li class="active"><span>18</span></li> <li><a href="#" onclick='return showDiv("page_19");'>19</a></li> <li><a href="#" onclick='return showDiv("page_20");'>20</a></li> <li><a href="#" onclick='return showDiv("page_25");'>»</a></li> </ul> </div> </div><!-- col-sm-12 --> </div><!-- row --> </div><!-- page_18 --> <div id="page_19" class="hiddenDiv"> <div class="row"> <div class="col-sm-12"> <div class="pull-right"> <ul class="pagination"> <li><a href="#" onclick='return showDiv("page_1");'>«</a></li> <li><a href="#" onclick='return showDiv("page_17");'>17</a></li> <li><a href="#" onclick='return showDiv("page_18");'>18</a></li> <li class="active"><span>19</span></li> <li><a href="#" onclick='return showDiv("page_20");'>20</a></li> <li><a href="#" onclick='return showDiv("page_21");'>21</a></li> <li><a href="#" onclick='return showDiv("page_25");'>»</a></li> </ul> </div> </div> </div> <div class="row"> <div class="col-sm-12"> <ol class="result-class" start="361"> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2015ApSS..355..930S','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2015ApSS..355..930S"><span id="translatedtitle">Influence of SiC surface polarity on the wettability and reactivity in an <span class="hlt">Al</span>/SiC <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Shen, Ping; Wang, Yi; Ren, Lihua; Li, Shixin; Liu, Yuhua; Jiang, Qichuan</p> <p>2015-11-01</p> <p>The wetting of (0 0 0 1) 6H-SiC single crystals by molten <span class="hlt">Al</span> was investigated using a dispensed sessile drop method in a high vacuum at 973-1173 K. The wettability and reactivity in this <span class="hlt">system</span> are sensitive to the surface polarity of SiC. The interfacial reaction on the Si-terminated surface is rapid. The formation of a continuous <span class="hlt">Al</span>4C3 product layer at the interface leads to an equilibrium contact angle of 56 ± 1° at 1173 K. In comparison, the interfacial reaction on the C-terminated surface is sluggish. The interface is only partially covered by discrete <span class="hlt">Al</span>4C3 platelets even after dwelling at 1173 K for 2 h. The final wettability, however, is much better (θF = 41 ± 1°) than that of the Si-terminated surface which was covered by a dense <span class="hlt">Al</span>4C3 layer, suggesting that the formation of <span class="hlt">Al</span>4C3 should not always contribute to the wetting in the <span class="hlt">Al</span>/SiC <span class="hlt">system</span>. A plausible explanation is that the clean (i.e., deoxidized) C-terminated surface should be well wetted by molten <span class="hlt">Al</span> in nature, owing to the strong chemical interactions between liquid <span class="hlt">Al</span> and the surface atoms of the C-terminated SiC. It is likely that the presence of the oxide film at the surface of the molten <span class="hlt">Al</span> drop or the SiC substrate and the rapid formation of <span class="hlt">Al</span>4C3, which prevent the establishment of a real <span class="hlt">Al</span>/SiC interface, conceal the intrinsic wettability of this <span class="hlt">system</span>.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/26720646','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/26720646"><span id="translatedtitle">Propagation Properties of the Precipitation Band in an <span class="hlt">Al</span>Cl₃/NaOH <span class="hlt">System</span>.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Ban, Takahiko; Nagatsu, Yuichiro; Tokuyama, Hideaki</p> <p>2016-01-19</p> <p>When inherently immobile solid particles collectively form precipitates in a reaction-diffusion <span class="hlt">system</span> involving a redissolution reaction, a propagation phenomenon may occur in which a dynamic pattern of precipitation bands forms. This propagating precipitation phenomenon has been studied by many researchers. However, two completely different processes-i.e., the reaction-diffusion of reactants and the crystal growth of products-progress simultaneously in the <span class="hlt">system</span>, thereby rendering the phenomenon complex. There are no well-established experimental laws for this propagating precipitation phenomenon, such as the spacing, time, and width laws associated with the well-known Liesegang phenomenon, which is static in the sense that precipitation bands form and remain at the same position. In fact, it has not been clarified which of the processes controls the propagation phenomenon. Accordingly, we have investigated the apparent diffusion coefficient associated with the dynamics of propagating precipitation band in an <span class="hlt">Al</span>Cl3/NaOH <span class="hlt">system</span> for the case in which a large excess of outer electrolytes (i.e., OH(-)) diffuses into gel in which inner electrolytes (i.e.,<span class="hlt">Al</span>(3+)) are homogeneously distributed. An isolated precipitation band of <span class="hlt">Al</span>(OH)3 was formed horizontally in a test tube and propagated vertically in proportion to the square root of time. In our experimental results, we found that the apparent diffusion coefficient, D(p), possesses an exponential dependence on the initial concentrations of the outer electrolyte, and the inner electrolyte; the measured relation was D(p) = D[<span class="hlt">Al</span>(3+)](-0.6)[OH(-)](0.6), where D = (0.63 ± 0.04) × 10(5) cm(2)/s. From our model equations based on the prenucleation theory, which take into account a redissolution reaction, we found that the dynamics of the reaction front of the outer and the inner electrolytes was an important factor in controlling the propagation of the precipitation band. In our simulation results, we obtained a similar</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://ntrs.nasa.gov/search.jsp?R=19920041145&hterms=ALS&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D10%26Ntt%3DALS','NASA-TRS'); return false;" href="http://ntrs.nasa.gov/search.jsp?R=19920041145&hterms=ALS&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D10%26Ntt%3DALS"><span id="translatedtitle">Aerodynamics of the advanced launch <span class="hlt">system</span> (<span class="hlt">ALS</span>) propulsion and avionics (P/A) module</span></a></p> <p><a target="_blank" href="http://ntrs.nasa.gov/search.jsp">NASA Technical Reports Server (NTRS)</a></p> <p>Ferguson, Stan; Savage, Dick</p> <p>1992-01-01</p> <p>This paper discusses the design and testing of candidate Advanced Launch <span class="hlt">System</span> (<span class="hlt">ALS</span>) Propulsion and Avionics (P/A) Module configurations. The P/A Module is a key element of future launch <span class="hlt">systems</span> because it is essential to the recovery and reuse of high-value propulsion and avionics hardware. The <span class="hlt">ALS</span> approach involves landing of first stage (booster) and/or second stage (core) P/A modules near the launch site to minimize logistics and refurbishment cost. The key issue addressed herein is the aerodynamic design of the P/A module, including the stability characteristics and the lift-to-drag (L/D) performance required to achieve the necessary landing guidance accuracy. The reference P/A module configuration was found to be statically stable for the desired flight regime, to provide adequate L/D for targeting, and to have effective modulation of the L/D performance using a body flap. The hypersonic aerodynamic trends for nose corner radius, boattail angle and body flap deflections were consistent with pretest predictions. However, the levels for the L/D and axial force for hypersonic Mach numbers were overpredicted by impact theories.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016ISPAnIII3..233W','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016ISPAnIII3..233W"><span id="translatedtitle">Uls LiDAR Supported Analyses of Laser Beam Penetration from Different <span class="hlt">ALS</span> <span class="hlt">Systems</span> Into Vegetation</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Wieser, M.; Hollaus, M.; Mandlburger, G.; Glira, P.; Pfeifer, N.</p> <p>2016-06-01</p> <p>This study analyses the underestimation of tree and shrub heights for different airborne laser scanner <span class="hlt">systems</span> and point cloud distribution within the vegetation column. Reference data was produced by a novel UAV-borne laser scanning (ULS) with a high point density in the complete vegetation column. With its physical parameters (e.g. footprint) and its relative accuracy within the block as stated in Section 2.2 the reference data is supposed to be highly suitable to detect the highest point of the vegetation. An airborne topographic (<span class="hlt">ALS</span>) and topo-bathymetric (ALB) <span class="hlt">system</span> were investigated. All data was collected in a period of one month in leaf-off condition, while the dominant tree species in the study area are deciduous trees. By robustly estimating the highest 3d vegetation point of each laser <span class="hlt">system</span> the underestimation of the vegetation height was examined in respect to the ULS reference data. This resulted in a higher under-estimation of the airborne topographic <span class="hlt">system</span> with 0.60 m (trees) and 0.55 m (shrubs) than for the topo-bathymetric <span class="hlt">system</span> 0.30 m (trees) and 0.40 m (shrubs). The degree of the underestimation depends on structural characteristics of the vegetation itself and physical specification of the laser <span class="hlt">system</span>.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2011JApSp..78..445I','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2011JApSp..78..445I"><span id="translatedtitle">Selective modulation of infrared radiation by an <span class="hlt">Al</span>2O3 particles — liquid crystal <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Ibragimov, T. D.; Allakhverdiev, E. A.; Bairamov, G. M.; Imamaliev, A. R.</p> <p>2011-07-01</p> <p>We have developed a dual-frequency liquid crystal consisting of 4-n-pentyl-4'-cyanobiphenyl (5CB), 4-hexyloxyphenyl-4'-hexyloxy-3-nitrobenzoate (C2), and 4-butyl-4'-(hexyloxyphenyloxycarbonyl)phenyl carbonate (H22). We have measured the frequency dependences of the longitudinal and transverse components of the dielectric constant of the mixture and we have determined the frequency regions of positive and negative dielectric anisotropy. We show that for certain changes in the frequency of the applied electric field while the magnitude of the field remains the same, the transmission region of the <span class="hlt">Al</span>2O3 particles — dual-frequency liquid crystal <span class="hlt">system</span> switches from one wavelength to the other. The experimental results are explained by the optical homogeneity of the <span class="hlt">system</span> in a narrow wavelength interval when the refractive indices of the particle material and the matrix are close, and also by reorientation of the liquid crystal molecules as the frequency of the applied voltage changes.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/21370547','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/21370547"><span id="translatedtitle">Thermal transformation of quaternary compounds in NaF-CaF{sub 2}-<span class="hlt">Al</span>F{sub 3} <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Zaitseva, Julia N.; Yakimov, Igor S.; Kirik, Sergei D.</p> <p>2009-08-15</p> <p>Details of quaternary compounds formation in the <span class="hlt">system</span> NaF-CaF{sub 2}-<span class="hlt">Al</span>F{sub 3} are specified. To achieve this aim, the samples of phases NaCa<span class="hlt">Al</span>F{sub 6} and Na{sub 2}Ca{sub 3}<span class="hlt">Al</span>{sub 2}F{sub 14} have been obtained by high-temperature solid-phase synthesis. Their thermal behavior when heated up to 800 deg. C has been studied using the methods of high-temperature X-ray diffraction (XRD) and thermal analysis (TA). The <span class="hlt">system</span> under consideration can be regarded as a quasibinary section CaF{sub 2}-Na<span class="hlt">Al</span>F{sub 4}, where at T=745-750 deg. C invariant equilibrium is implemented with the phases CaF{sub 2}-NaCa<span class="hlt">Al</span>F{sub 6}-Na{sub 2}Ca{sub 3}<span class="hlt">Al</span>{sub 2}F{sub 14}-(liquid melt)-(Na<span class="hlt">Al</span>F{sub 4}). The peculiarity of the equilibrium is Na<span class="hlt">Al</span>F{sub 4} metastability at normal pressure. Below the equilibrium temperature the quaternary phase Na{sub 2}Ca{sub 3}<span class="hlt">Al</span>{sub 2}F{sub 14} is stable and NaCa<span class="hlt">Al</span>F{sub 6} above this temperature. The phase NaCa<span class="hlt">Al</span>F{sub 6} fixed by rapid quenching from high temperatures and when heated up to 640 deg. C decomposes, yielding Na{sub 2}Ca{sub 3}<span class="hlt">Al</span>{sub 2}F{sub 14}. Further heating in vacuum at temperature up to 740 deg. C results in decomposition of Na{sub 2}Ca{sub 3}<span class="hlt">Al</span>{sub 2}F{sub 14} into CaF{sub 2} and Na{sub 3}<span class="hlt">Al</span>F{sub 6}. The expected reverse transformation of Na{sub 2}Ca{sub 3}<span class="hlt">Al</span>{sub 2}F{sub 14} into NaCa<span class="hlt">Al</span>F{sub 6} has not been observed under experimental conditions. Transformations in bulk samples reveal direct and reverse transformation of quaternary phases. Synopsis: Thermal transformation of the quaternary compounds in <span class="hlt">system</span> (NaF-CaF{sub 2}-<span class="hlt">Al</span>F{sub 3}) was investigated using high-temperature X-ray diffraction (XRD) and thermal analysis (TA). In the <span class="hlt">system</span> the invariant equilibrium is implemented with the phases CaF{sub 2}-NaCa<span class="hlt">Al</span>F{sub 6}-Na{sub 2}Ca{sub 3}<span class="hlt">Al</span>{sub 2}F{sub 14}-(liquid melt)-(Na<span class="hlt">Al</span>F{sub 4}) at T=745-750 deg. C. - Graphical Abstract: The paper concerns of a small piece of the ternary <span class="hlt">system</span> (NaF-CaF{sub 2}-<span class="hlt">Al</span>F{sub 3}) which is very important for</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://ntrs.nasa.gov/search.jsp?R=19890030008&hterms=Mn&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D50%26Ntt%3DMn','NASA-TRS'); return false;" href="http://ntrs.nasa.gov/search.jsp?R=19890030008&hterms=Mn&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D50%26Ntt%3DMn"><span id="translatedtitle">Iron-base superalloys - A phase analysis of the multicomponent <span class="hlt">system</span> (Fe-Mn-Cr-Mo-Nb-<span class="hlt">Al</span>-Si-C)</span></a></p> <p><a target="_blank" href="http://ntrs.nasa.gov/search.jsp">NASA Technical Reports Server (NTRS)</a></p> <p>Gupta, H.; Nowotny, H.; Lemkey, F. D.</p> <p>1988-01-01</p> <p>In the course of studies on the iron-rich multicomponent <span class="hlt">system</span> Fe-Mn-Cr-Mo-Nb-<span class="hlt">Al</span>-Si-C, work was concentrated on pertinent quinary and six-component combinations namely Fe-Mn-<span class="hlt">Al</span>-Si-C, Fe-Cr-<span class="hlt">Al</span>-Si-C and Fe-Mn-Cr-<span class="hlt">Al</span>-Si-C which had been elaborated at 65, 72, and 80 wt pct Fe. Manganese acts as a strong stabilizer for the cementite carbide. Chromium seems to stabilize the iron aluminide Fe2<span class="hlt">Al</span>5 which forms in a considerable amount within an alloy of nominal composition Fe(65)Mn(15)Cr(12)<span class="hlt">Al</span>(5)Si(2)C(1) (percent by weight). Although the Mn3<span class="hlt">Al</span>C carbide is, like Fe3<span class="hlt">Al</span>C, a perovskite carbide, manganese does not appear to favor the formation of the perovskite carbide. Because of the relatively low sintering temperature (700 C), for <span class="hlt">al</span> large portion of the samples equilibria conditions are not always reached.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.gpo.gov/fdsys/pkg/FR-2011-06-15/pdf/2011-14628.pdf','FEDREG'); return false;" href="https://www.gpo.gov/fdsys/pkg/FR-2011-06-15/pdf/2011-14628.pdf"><span id="translatedtitle">76 FR 35017 - United States et <span class="hlt">al</span>. v. United Regional Health Care <span class="hlt">System</span>; Public Comments and Response on...</span></a></p> <p><a target="_blank" href="http://www.gpo.gov/fdsys/browse/collection.action?collectionCode=FR">Federal Register 2010, 2011, 2012, 2013, 2014</a></p> <p></p> <p>2011-06-15</p> <p>... Antitrust Division United States et <span class="hlt">al</span>. v. United Regional Health Care <span class="hlt">System</span>; Public Comments and Response... States and State of Texas v. United Regional Health Care <span class="hlt">System</span>, Civil Action No. 7:11-cv- 00030-0, which.... United Regional Health Care <span class="hlt">System</span>, Defendant. Case No.: 7:11-cv-00030 Response Of Plaintiff...</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/1980GeCoA..44.1409N','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/1980GeCoA..44.1409N"><span id="translatedtitle">Thermochemistry of glasses and liquids in the <span class="hlt">systems</span> CaMgSi 2O 6-Ca<span class="hlt">Al</span> 2Si 2O 8-Na<span class="hlt">Al</span>Si 3O 8, SiO 2-Ca<span class="hlt">Al</span> 2Si 2O 8-Na<span class="hlt">Al</span>Si 3O 8 and SiO 2-<span class="hlt">Al</span> 2O 3-CaO-Na 2O</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Navrotsky, A.; Hon, R.; Weill, D. F.; Henry, D. J.</p> <p>1980-10-01</p> <p>Enthalpies of solution in 2PbO· B 2O 3 at 712°C have been measured for glasses in the <span class="hlt">systems</span> albite anorthite diopside, Na<span class="hlt">Al</span>O 2-SiO 2, Ca 0.5<span class="hlt">Al</span>O 2-SiO 2 and albite-anorthite-quartz. The <span class="hlt">systems</span> albite-anorthite and diopside-anorthite show substantial negative enthalpies of mixing, albite-diopside shows significant positive heats of mixing. For compositions up to Na<span class="hlt">Al</span>O2 = 0.42 (which includes the subsystem albite-silica) the <span class="hlt">system</span> Na<span class="hlt">Al</span>O 2-SiO 2 shows essentially zero heats of mixing. A negative ternary excess heat of mixing is found in the plagioclase-rich portion of the albite-anorthite-diopside <span class="hlt">system</span>. The join Si 4O 8-Ca<span class="hlt">Al</span> 2Si 2O 8 shows small but significant heats of mixing. In albite-anorthite-quartz. ternary glasses, the ternary excess enthalpy of mixing is positive. Based on available heat capacity data and appropriate consideration of the glass transition, the enthalpy of the crystal-glass transition (vitrification) is a serious underestimate of the enthalpy of the crystal-liquid transition (fusion) especially when the melting point, Tf, is many hundreds of degrees higher than the glass transition temperature, Tg. On the other hand, the same heat capacity data suggest that the enthalpies of mixing in albite-anorthite-diopside liquids are calculated to be quite similar to those in the glasses. The enthalpies of mixing observed in general support the structural models proposed by TAYLOR and BROWN (1979a, b) and others for the structure of aluminosilicate glasses.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2015MMTB...46.2005F','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2015MMTB...46.2005F"><span id="translatedtitle">Effect of Fluorine on the Structure of High <span class="hlt">Al</span>2O3-Bearing <span class="hlt">System</span> by Molecular Dynamics Simulation</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Fan, Guozheng; He, Shengping; Wu, Ting; Wang, Qian</p> <p>2015-08-01</p> <p>Calcium aluminosilicate is the most important slag <span class="hlt">system</span> in continuous casting, while CaF2 is commonly used as a fluxing agent in mold fluxes. In this study, the structural properties of the CaO-<span class="hlt">Al</span>2O3-SiO2-CaF2 <span class="hlt">system</span> with varying fluorine content have been investigated by molecular dynamics simulation using the pairwise potential model. The results showed that with the substitution of oxygen ions by fluorine ions, the average bond length of Si-O remained unchanged, while the average bond length of <span class="hlt">Al</span>-O increased from 1.74 to 1.75 Å. The addition of fluorine contributed to the increase in the bridging oxygen proportion and the network connectivity ( Q n ) degree, suggesting that the polymerization of melts was enhanced. Meanwhile, the threefold-coordinated <span class="hlt">Al</span> was found when mass fraction of fluorine was increased, and it was due to that the fluorine ions substituted the oxygen ions and formed the [<span class="hlt">Al</span>O3F] tetrahedron. The <span class="hlt">Al</span> avoidance principle is not applicable in the CaO-<span class="hlt">Al</span>2O3-SiO2-CaF2 <span class="hlt">system</span> with a high content of <span class="hlt">Al</span>2O3.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://ntrs.nasa.gov/search.jsp?R=20010048750&hterms=SI+system&qs=N%3D0%26Ntk%3DAll%26Ntx%3Dmode%2Bmatchall%26Ntt%3DSI%2Bsystem','NASA-TRS'); return false;" href="http://ntrs.nasa.gov/search.jsp?R=20010048750&hterms=SI+system&qs=N%3D0%26Ntk%3DAll%26Ntx%3Dmode%2Bmatchall%26Ntt%3DSI%2Bsystem"><span id="translatedtitle">Lamellar Spacing Selection in <span class="hlt">Al</span>-Si Eutectic <span class="hlt">System</span>: a Theoretical Investigation</span></a></p> <p><a target="_blank" href="http://ntrs.nasa.gov/search.jsp">NASA Technical Reports Server (NTRS)</a></p> <p>Catalina, Adrian V.; Sen, Subhayu; Curreri, Peter A.; Rose, M. Franklin (Technical Monitor)</p> <p>2001-01-01</p> <p>It is well known that irregular eutectics such as <span class="hlt">Al</span>-Si and Fe-C exhibit larger lamellar spacings and undercoolings compared to the predictions made by the Jackson and Hunt (JH) theory. In this paper, we reexamine the JH theory and relax some of the assumptions used in that treatment. The modified theoretical model has enhanced capabilities to predict the lamellar spacing in both regular and irregular eutectics. For the <span class="hlt">Al</span>-Si <span class="hlt">system</span> in particular we identified two different spacing selection mechanisms:a) for a particular growth rate, a nearly isothermal interface can be achieved at a unique minimum spacing lambda(sub I); b) the average spacing in the microstructure (lambda(sub av) greater than lambda(sub I)) is essentially dictated by the undercooling of the faceted phase. Based on the modified theoretical model a semi-empirical expression has been developed to account for the influence of the temperature gradient. Application of a Mullin and Sekerka type stability analysis for eutectics will also be presented and the results compared to the modified JH model. It will be shown that the both theoretical approaches are in good agreement with each other and also with the published experimental measurements.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2005AGUFM.P32A..01C','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2005AGUFM.P32A..01C"><span id="translatedtitle">26<span class="hlt">Al</span> in the Saturnian <span class="hlt">System</span> - New Interior Models for the Saturnian satellites</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Castillo, J. C.; Matson, D. L.; Johnson, T. V.; Lunine, J. I.; McCord, T. B.; Sotin, C.; Thomas, P. C.; Turtle, E. B.</p> <p>2005-12-01</p> <p>Recent study of Iapetus' spin rate evolution highlights the need to form this satellite between between 1.0+/- 0.2 to 1.6+/- 0.4 My after the production of Calcium-Aluminum Inclusions (CAIs). We study the implications of this time constraint on the thermal evolution of other "icy" Saturnian satellites, assuming that they formed at the same time as Iapetus and from the same rocky material in proportion to their densities. Heat provided by 26<span class="hlt">Al</span> decay contributes to partial to full melting and thus differentiation of all Saturn's medium-sized satellites, except Tethys. We also consider the effect of silicate hydration on the internal and geological evolution of these satellites. These results are compared with classical models (that do not include short-lived radiogenic species), in the light of the observational constraints available for these satellites. Including 26<span class="hlt">Al</span> decay in the heat budget of the satellites allows to explain the observation of geological activity in silicate-poor satellites such as Tethys. We note that in Enceladus and Titan conditions might have been such that the boiling point of water was reached and water might have been lost very early in the history of these satellites. This opens the door to some explanation for the variations in density within the Saturnian <span class="hlt">system</span>. Part of this work was carried out at the Jet Propulsion Laboratory, California Institute of Technology, under contract to NASA.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2013ApSS..271..311D','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2013ApSS..271..311D"><span id="translatedtitle">The effect of silicon on the oxidation behavior of Ni<span class="hlt">Al</span>Hf coating <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Dai, Pengchao; Wu, Qiong; Ma, Yue; Li, Shusuo; Gong, Shengkai</p> <p>2013-04-01</p> <p>Two types of Ni<span class="hlt">Al</span>Hf coatings doped with different content of Si (1 at.% and 2 at.%) were deposited on a Ni3<span class="hlt">Al</span> based single crystal superalloy IC32 by electron beam physical vapor deposition (EB-PVD) method, respectively. For comparison, Ni<span class="hlt">Al</span>Hf coating with 0 at.% Si was also prepared. The oxidation tests were carried out at 1423 K in air. At the initial stage of oxidation, large amount of flake-like θ-<span class="hlt">Al</span>2O3 was found on Ni<span class="hlt">Al</span>Hf coating surface. However, no θ-<span class="hlt">Al</span>2O3 was observed in 2 at.% Si doped Ni<span class="hlt">Al</span>Hf coating except α-<span class="hlt">Al</span>2O3. It revealed that the Si additions could contribute to the transformation from θ-<span class="hlt">Al</span>2O3 to α-<span class="hlt">Al</span>2O3. When oxidation time prolonged to 100 h, it was found that the degradation of Ni<span class="hlt">Al</span>Hf coating was very severe with no residual β-phase, which was due to the serious inter-diffusion between the coating and substrate. In contrast, the inter-diffusion in Si-doped coating was reduced with some residual β-phase and R-Ni(Mo, Re) precipitates. The presence of Si could retard the inter-diffusion of elements between coating and substrate, indicating a barrier diffusion effect. As a result, the oxidation resistance of Ni<span class="hlt">Al</span>Hf coating was improved significantly.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016RuPhJ..59..134S','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016RuPhJ..59..134S"><span id="translatedtitle">Semi-Empirical Study of the Indirect Exchange Interaction in the Rem - <span class="hlt">Al</span> <span class="hlt">System</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Shakarov, Kh. O.</p> <p>2016-05-01</p> <p>The Ruderman-Kittel-Kasuya-Yosida exchange interaction (RKKY) is semi-empirically studied for the first time in compounds of binary REM - <span class="hlt">Al</span> <span class="hlt">systems</span> (REM - rare-earth metals: Gd, Dy, Ho, Er) using experimental values of paramagnetic Curie point (θp) of these compounds. Prediction of the RKKY theory was confirmed, i.e. there is a direct proportional dependence of θp value on de Gennes factor for equiatomic compounds of heavy REM with aluminum, just as in the case of pure REM. Values of the indirect exchange interaction parameter were semi-empirically estimated for the studied compounds. In general, it was established that RKKY-type exchange interaction is typical for REM compounds with aluminum, just as for pure REM.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2007APS..SHK.C6002A','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2007APS..SHK.C6002A"><span id="translatedtitle">A study of reactant interfaces in Ni+<span class="hlt">Al</span> particle <span class="hlt">systems</span> during shock wave propagation</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Austin, Ryan A.; McDowell, David L.; Horie, Yasuyuki; Benson, David J.</p> <p>2007-06-01</p> <p>Macro-scale responses of energetic materials during shock compression are influenced strongly by thermo-mechano-chemical processes occurring at the level of the microstructure. For example, it is believed that the propagation of chemical reactions in reactive particle <span class="hlt">systems</span> is intimately linked to conditions at reactant interfaces such as surface temperature, phase changes, defect density, and mass mixing due to inelastic deformation. To provide explicit resolution of such interfacial conditions, numerical models are constructed. The finite element method is used to numerically solve the differential equations that govern the coupled thermomechanical response of micron-size particle mixtures of Ni and <span class="hlt">Al</span> during shock wave propagation (interface chemistry is not yet modeled). The size and temperature distributions of contiguous reactant contact surfaces are quantified for a range of shock strengths. A parametric study of mixture attributes is undertaken to assess the sensitivity of the aforementioned distributions to variations of the microstructure.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016JPSJ...85k4602N','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016JPSJ...85k4602N"><span id="translatedtitle">Crystallographic Features and State Stability of the Decagonal Quasicrystal in the <span class="hlt">Al</span>-Co-Cu Alloy <span class="hlt">System</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Nakayama, Kei; Mizutani, Akito; Koyama, Yasumasa</p> <p>2016-11-01</p> <p>In the <span class="hlt">Al</span>-Co-Cu alloy <span class="hlt">system</span>, both the decagonal quasicrystal with the space group of Poverline{10}m2 and its approximant <span class="hlt">Al</span>13Co4 phase with monoclinic Cm symmetry are present around 20 at. % Co-10 at. % Cu. In this study, we examined the crystallographic features of prepared <span class="hlt">Al</span>-(30 - x) at. % Co-x at. % Cu samples mainly by transmission electron microscopy in order to make clear the crystallographic relation between the decagonal quasicrystal and the monoclinic <span class="hlt">Al</span>13Co4 structure. The results revealed a coexistence state consisting of decagonal quasicrystal and approximant <span class="hlt">Al</span>13Co4 regions in <span class="hlt">Al</span>-20 at. % Co-10 at. % Cu alloy samples. With the help of the coexistence state, the orientation relationship was established between the monoclinic <span class="hlt">Al</span>13Co4 structure and the decagonal quasicrystal. In the determined relationship, the crystallographic axis in the quasicrystal was found to be parallel to the normal direction of the (010)m plane in the <span class="hlt">Al</span>13Co4 structure, where the subscript m denotes the monoclinic <span class="hlt">system</span>. Based on data obtained experimentally, the state stability of the decagonal quasicrystal was also examined in terms of the Hume-Rothery (HR) mechanism on the basis of the nearly-free-electron approximation. It was found that a model based on the HR mechanism could explain the crystallographic features such as electron diffraction patterns and atomic arrangements found in the decagonal quasicrystal. In other words, the HR mechanism is most likely appropriate for the stability of the decagonal quasicrystal in the <span class="hlt">Al</span>-Co-Cu alloy <span class="hlt">system</span>.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2011SSSci..13.1813D','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2011SSSci..13.1813D"><span id="translatedtitle">In-situ laser synthesis of rare earth aluminate coatings in the <span class="hlt">system</span> Ln-<span class="hlt">Al</span>-O ( Ln = Y, Gd)</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>de Francisco, I.; Lennikov, V. V.; Bea, J. A.; Vegas, A.; Carda, J. B.; de la Fuente, G. F.</p> <p>2011-09-01</p> <p>Laser zone melting (LZM) was employed in this work to prepare Ln-<span class="hlt">Al</span>-O coatings on polycrystalline <span class="hlt">Al</span> 2O 3 substrates, using the corresponding mixtures of powdered rare-earth oxides and <span class="hlt">Al</span> 2O 3 as starting materials. In-situ synthesis of the compounds Ln = Y, Gd was performed using a CO 2 laser, emitting at 10.6 μm. Microstructure (SEM) and phase nature (XRD) demonstrated in-situ formation of <span class="hlt">Al</span> 2O 3/Y 3<span class="hlt">Al</span> 5O 12(YAG) and <span class="hlt">Al</span> 2O 3/Gd<span class="hlt">Al</span>O 3(GAP) eutectic <span class="hlt">systems</span>. The interaction with the substrate resulted in mechanically stable, well integrated 200-500 μm thick composite coatings, as observed in nanoindentation tests. The phase relations found in these materials are consistent with the crystallographic concepts advanced by Vegas (Ramos-Gallardo & Vegas, J. Solid State Chem. 128 (1997) 69), where cation sub-arrays are proposed to play an important role in governing metal oxide structures. These sub-arrays are suggested as the structural drive behind eutectic oxide formation. LZM proves to be a convenient method to investigate the behaviour of complex oxide <span class="hlt">systems</span> at high temperature, to apply a rational concept towards the understanding of phase relations and to develop design criteria for oxide coatings.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2015SuMi...82..158Y','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2015SuMi...82..158Y"><span id="translatedtitle">Wettability, interfacial phenomena and shear strength for <span class="hlt">Al</span>/ZrO2 joining <span class="hlt">system</span> driven by direct current</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Yang, Nan-Nan; Gu, Yan; Cao, Kang-Zhan</p> <p>2015-06-01</p> <p>We realized the wetting for molten <span class="hlt">Al</span> on the 3 mol.% Yttria-Stabilized Zirconia (ZrO2) with applying a direct current (DC). The interfacial microstructures and shear stress were evaluated after wetting to reveal their native relationship with DC application. The results showed that not only the wettability of <span class="hlt">Al</span>/ZrO2 <span class="hlt">system</span> but also the shear stress can be optimized by controlling the DC intensity and applying time, which strongly depends on the formation of Zr<span class="hlt">Al</span>3 at the interface of <span class="hlt">Al</span>/ZrO2 <span class="hlt">system</span>. When the current intensity is 20 mA and applying time is 10 min, the shear stress reaches the maximum in our case.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4251983','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4251983"><span id="translatedtitle">A Reconstruction Method Based on <span class="hlt">AL</span>0FGD for Compressed Sensing in Border Monitoring WSN <span class="hlt">System</span></span></a></p> <p><a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pmc">PubMed Central</a></p> <p>Wang, Yan; Wu, Xi; Li, Wenzao; Zhang, Yi; Li, Zhi; Zhou, Jiliu</p> <p>2014-01-01</p> <p>In this paper, to monitor the border in real-time with high efficiency and accuracy, we applied the compressed sensing (CS) technology on the border monitoring wireless sensor network (WSN) <span class="hlt">system</span> and proposed a reconstruction method based on approximately l0 norm and fast gradient descent (<span class="hlt">AL</span>0FGD) for CS. In the frontend of the <span class="hlt">system</span>, the measurement matrix was used to sense the border information in a compressed manner, and then the proposed reconstruction method was applied to recover the border information at the monitoring terminal. To evaluate the performance of the proposed method, the helicopter sound signal was used as an example in the experimental simulation, and three other typical reconstruction algorithms 1)split Bregman algorithm, 2)iterative shrinkage algorithm, and 3)smoothed approximate l0 norm (SL0), were employed for comparison. The experimental results showed that the proposed method has a better performance in recovering the helicopter sound signal in most cases, which could be used as a basis for further study of the border monitoring WSN <span class="hlt">system</span>. PMID:25461759</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4617755','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4617755"><span id="translatedtitle">Role of Cholesterol and Phospholipids in Amylin Misfolding, Aggregation and Etiology of Islet <span class="hlt">Amyloidosis</span></span></a></p> <p><a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pmc">PubMed Central</a></p> <p>Singh, Sanghamitra; Trikha, Saurabh; Bhowmick, Diti Chatterjee; Sarkar, Anjali A.</p> <p>2015-01-01</p> <p><span class="hlt">Amyloidosis</span> is a biological event in which proteins undergo structural transitions from soluble monomers and oligomers to insoluble fibrillar aggregates that are often toxic to cells. Exactly how amyloid proteins, such as the pancreatic hormone amylin, aggregate and kill cells is still unclear. Islet amyloid polypeptide, or amylin, is a recently discovered hormone that is stored and co-released with insulin from pancreatic islet β-cells. The pathology of type 2 diabetes mellitus (T2DM) is characterized by an excessive extracellular and intracellular accumulation of toxic amylin species, soluble oligomers and insoluble fibrils, in islets, eventually leading to β-cell loss. Obesity and elevated serum cholesterol levels are additional risk factors implicated in the development of T2DM. Because the homeostatic balance between cholesterol synthesis and uptake is lost in diabetics, and amylin aggregation is a hallmark of T2DM, this chapter focuses on the biophysical and cell biology studies exploring molecular mechanisms by which cholesterol and phospholipids modulate secondary structure, folding and aggregation of human amylin and other amyloid proteins on membranes and in cells. Amylin turnover and toxicity in pancreatic cells and the regulatory role of cholesterol in these processes are also discussed. PMID:26149927</p> </li> </ol> <div class="pull-right"> <ul class="pagination"> <li><a href="#" onclick='return showDiv("page_1");'>«</a></li> <li><a href="#" onclick='return showDiv("page_17");'>17</a></li> <li><a href="#" onclick='return showDiv("page_18");'>18</a></li> <li class="active"><span>19</span></li> <li><a href="#" onclick='return showDiv("page_20");'>20</a></li> <li><a href="#" onclick='return showDiv("page_21");'>21</a></li> <li><a href="#" onclick='return showDiv("page_25");'>»</a></li> </ul> </div> </div><!-- col-sm-12 --> </div><!-- row --> </div><!-- page_19 --> <div id="page_20" class="hiddenDiv"> <div class="row"> <div class="col-sm-12"> <div class="pull-right"> <ul class="pagination"> <li><a href="#" onclick='return showDiv("page_1");'>«</a></li> <li><a href="#" onclick='return showDiv("page_18");'>18</a></li> <li><a href="#" onclick='return showDiv("page_19");'>19</a></li> <li class="active"><span>20</span></li> <li><a href="#" onclick='return showDiv("page_21");'>21</a></li> <li><a href="#" onclick='return showDiv("page_22");'>22</a></li> <li><a href="#" onclick='return showDiv("page_25");'>»</a></li> </ul> </div> </div> </div> <div class="row"> <div class="col-sm-12"> <ol class="result-class" start="381"> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2002177','PMC'); return false;" href="http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2002177"><span id="translatedtitle">Evidence for the experimental transmission of cerebral beta-<span class="hlt">amyloidosis</span> to primates.</span></a></p> <p><a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pmc">PubMed Central</a></p> <p>Baker, H. F.; Ridley, R. M.; Duchen, L. W.; Crow, T. J.; Bruton, C. J.</p> <p>1993-01-01</p> <p>The brains of three marmosets (Callithrix jacchus) injected intracerebrally 6-7 years earlier with brain tissue from a patient with early onset Alzheimer's disease were found to contain moderate numbers of amyloid plaques with associated argyrophilic dystrophic neurites and cerebral amyloid angiopathy but no neurofibrillary tangles. The plaques and vascular amyloid stained positively with antibodies to beta (A4)-protein. The brains of three age-matched control marmosets from the same colony did not show these neuropathological features. The brain of one of two marmosets injected with brain tissue from a patient with prion disease with concomitant beta-amyloid plaques and cerebral amyloid angiopathy also showed beta-amyloid plaques and angiopathy but no spongiform encephalopathy. An occasional plaque was found in the brains of two of four marmosets injected with brain tissue from three elderly patients with age-related pathology, two of whom had an additional diagnosis of possible prion disease. Neither plaques nor cerebral amyloid angiopathy were found in six other marmosets who were older than the injected animals, in 12 further marmosets who were slightly younger but who had been injected several years previously with brain tissue which did not contain beta-amyloid, or in 10 younger marmosets who had been subjected to various neurosurgical procedures. These results suggest that cerebral beta-<span class="hlt">amyloidosis</span> may be induced by the introduction of exogenous amyloid beta-protein. Images Figure 1 Figure 2 Figure 3 Figure 4 PMID:8217779</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/24452946','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/24452946"><span id="translatedtitle">The role of 3D and speckle tracking echocardiography in cardiac <span class="hlt">amyloidosis</span>: a case report.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Nucci, E M; Lisi, M; Cameli, M; Baldi, L; Puccetti, L; Mondillo, S; Favilli, R; Lunghetti, S</p> <p>2014-01-01</p> <p>Cardiac <span class="hlt">amyloidosis</span> (CA) is a disorder characterized by amyloid fibrils deposition in cardiac interstitium; it results in a restrictive cardiomyopathy with heart failure (HF) and conduction abnormalities. The "gold standard" for diagnosis of CA is myocardial biopsy but possible sampling errors and procedural risks, limit it's use. Magnetic resonance (RMN) offers more information than traditional echocardiography and allows diagnosis of CA but often it's impossible to perform. We report the case of a man with HF and symptomatic bradyarrhythmia that required an urgent pacemaker implant. Echocardiography was strongly suggestive of CA but wasn't impossible to perform an RMN to confirm this hypothesis because the patient was implanted with a definitive pacemaker. So was performed a Speckle Tracking Echocardiography (STE) and a 3D echocardiography: STE allows to differentiate CA from others hypertrophic cardiomyopathy by longitudinal strain value < 12% and 3D echocardiography shows regional left ventricular dyssynchrony with a characteristic temporal pattern of dispersion of regional volume systolic change. On the basis of these results, finally was performed an endomyocardial biopsy that confirmed the diagnosis of CA. This case underlines the importance of news, noninvasive techniques such as eco 3D and STE for early diagnosis of CA, especially when RMN cannot be performed.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.ncbi.nlm.nih.gov/pubmed/1362414','PUBMED'); return false;" href="http://www.ncbi.nlm.nih.gov/pubmed/1362414"><span id="translatedtitle">Cutaneous lesion associated with multiple endocrine neoplasia type 2A: lichen <span class="hlt">amyloidosis</span> or notalgia paresthetica?</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Chabre, O; Labat, F; Pinel, N; Berthod, F; Tarel, V; Bachelot, I</p> <p>1992-01-01</p> <p>Three patients of a French family demonstrated an association of multiple endocrine neoplasia type 2A (MEN 2A) with a pruritic scapular skin lesion. The lesions are similar to those described as familial cutaneous lichen <span class="hlt">amyloidosis</span> in unrelated MEN 2A and medullary thyroid carcinoma families, but histological, immunohistochemical, and ultrastructural analysis of skin biopsies from each patient in the French family did not show amyloid deposition. The topography of the lesion follows dermatomes C8-D3. The patients report not only pruritus but also paresthesia and hyperalgesia, and one showed touch hypoesthesia and pain hyperesthesia in the area of the lesion. Such an association of cutaneous and neurological features suggests notalgia paresthetica (NP), a neuropathy of the posterior dorsal rami nerves. We thus suggest that the cutaneous lesions associated with MEN 2A might be secondary to pathology in the neural crest-derived dorsal sensory nerves. The amyloid, when present, would be secondary to scratching. We propose that patients presenting with familial NP be suspect for MEN 2A. PMID:1362414</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/22412999','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/22412999"><span id="translatedtitle">Quasiperiodic <span class="hlt">Al</span>GaAs superlattices for neuromorphic networks and nonlinear control <span class="hlt">systems</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Malyshev, K. V.</p> <p>2015-01-28</p> <p>The application of quasiperiodic <span class="hlt">Al</span>GaAs superlattices as a nonlinear element of the FitzHugh–Nagumo neuromorphic network is proposed and theoretically investigated on the example of Fibonacci and figurate superlattices. The sequences of symbols for the figurate superlattices were produced by decomposition of the Fibonacci superlattices' symbolic sequences. A length of each segment of the decomposition was equal to the corresponding figurate number. It is shown that a nonlinear network based upon Fibonacci and figurate superlattices provides better parallel filtration of a half-tone picture; then, a network based upon traditional diodes which have cubic voltage-current characteristics. It was found that the figurate superlattice F{sup 0}{sub 11}(1) as a nonlinear network's element provides the filtration error almost twice less than the conventional “cubic” diode. These advantages are explained by a wavelike shape of the decreasing part of the quasiperiodic superlattice's voltage-current characteristic, which leads to multistability of the network's cell. This multistability promises new interesting nonlinear dynamical phenomena. A variety of wavy forms of voltage-current characteristics opens up new interesting possibilities for quasiperiodic superlattices and especially for figurate superlattices in many areas—from nervous <span class="hlt">system</span> modeling to nonlinear control <span class="hlt">systems</span> development.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/988753','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/988753"><span id="translatedtitle">Solid Solution Effects on the Mg<span class="hlt">Al</span>2O4-MgGa2O4 <span class="hlt">System</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>O'Hara, Kelley; Smith, Jeffrey D; Hemrick, James Gordon</p> <p>2009-01-01</p> <p>Phase relations between two spinel compounds (Mg<span class="hlt">Al</span>2O4 and MgGa2O4) were studied. Stoichiometric Mg<span class="hlt">Al</span>2O4 was formed in the laboratory through a coprecipitation method. Complete solid solution formation int eh Mg<span class="hlt">Al</span>2O4-MgGa2O4 <span class="hlt">systems</span> was confirmed through X-ray diffraction analysis. Solid solution between Mg<span class="hlt">Al</span>2O4-MgGa2O4 decreases thermal conductivity at all temperatures up to 900oC. At 200oC with 10 mol% additoin of MgGa2O4 thermal conductivity decreases approximately 25%, and at 900oC there was still an 8% decrease. Additionally, preliminary studies show that porosity between 5% and 10% does not have an appreciable effect on the thermal conductivity in this study.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.ncbi.nlm.nih.gov/pubmed/8748646','PUBMED'); return false;" href="http://www.ncbi.nlm.nih.gov/pubmed/8748646"><span id="translatedtitle">27<span class="hlt">Al</span> magic-angle spinning nuclear magnetic resonance satellite transition spectroscopy of glasses in the <span class="hlt">system</span> K2O-<span class="hlt">Al</span>2O3-SiO2.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Mundus, C; Müller-Warmuth, W</p> <p>1995-10-01</p> <p>27<span class="hlt">Al</span> magic-angle spinning nuclear magnetic resonance satellite transition spectroscopy at 78 MHz has been applied to determine (true) chemical shift and quadrupole coupling parameters of glasses in the <span class="hlt">system</span> K2O-<span class="hlt">Al</span>2O3-SiO2 with 60-80 mol% SiO2 and K2O concentrations between 0 and 24 mol%. The powdered crystalline aluminosilicates andalusite and sillimanite have also been examined. In the glasses, all <span class="hlt">Al</span> appears to be tetrahedrally bound in the aluminosilicate network unless x = mol% K2O:mol% <span class="hlt">Al</span>2O3 becomes extremely small. Upon decreasing x the distortion of the tetrahedral <span class="hlt">Al</span>(OSi)4 units increases in steps, and possible explanations are discussed. Six-coordinated aluminum observed for x < 0.2 is connected with the occurrence of interstitial <span class="hlt">Al</span>3+ ions which charge-compensate the <span class="hlt">Al</span>O4 units in addition to K+. PMID:8748646</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.ncbi.nlm.nih.gov/pubmed/27443609','PUBMED'); return false;" href="http://www.ncbi.nlm.nih.gov/pubmed/27443609"><span id="translatedtitle">Antibiotic-induced perturbations in gut microbial diversity influences neuro-inflammation and <span class="hlt">amyloidosis</span> in a murine model of Alzheimer's disease.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Minter, Myles R; Zhang, Can; Leone, Vanessa; Ringus, Daina L; Zhang, Xiaoqiong; Oyler-Castrillo, Paul; Musch, Mark W; Liao, Fan; Ward, Joseph F; Holtzman, David M; Chang, Eugene B; Tanzi, Rudolph E; Sisodia, Sangram S</p> <p>2016-01-01</p> <p>Severe <span class="hlt">amyloidosis</span> and plaque-localized neuro-inflammation are key pathological features of Alzheimer's disease (AD). In addition to astrocyte and microglial reactivity, emerging evidence suggests a role of gut microbiota in regulating innate immunity and influencing brain function. Here, we examine the role of the host microbiome in regulating <span class="hlt">amyloidosis</span> in the APPSWE/PS1ΔE9 mouse model of AD. We show that prolonged shifts in gut microbial composition and diversity induced by long-term broad-spectrum combinatorial antibiotic treatment regime decreases Aβ plaque deposition. We also show that levels of soluble Aβ are elevated and that levels of circulating cytokine and chemokine signatures are altered in this setting. Finally, we observe attenuated plaque-localised glial reactivity in these mice and significantly altered microglial morphology. These findings suggest the gut microbiota community diversity can regulate host innate immunity mechanisms that impact Aβ <span class="hlt">amyloidosis</span>. PMID:27443609</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4956742','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4956742"><span id="translatedtitle">Antibiotic-induced perturbations in gut microbial diversity influences neuro-inflammation and <span class="hlt">amyloidosis</span> in a murine model of Alzheimer’s disease</span></a></p> <p><a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pmc">PubMed Central</a></p> <p>Minter, Myles R.; Zhang, Can; Leone, Vanessa; Ringus, Daina L.; Zhang, Xiaoqiong; Oyler-Castrillo, Paul; Musch, Mark W.; Liao, Fan; Ward, Joseph F.; Holtzman, David M.; Chang, Eugene B.; Tanzi, Rudolph E.; Sisodia, Sangram S.</p> <p>2016-01-01</p> <p>Severe <span class="hlt">amyloidosis</span> and plaque-localized neuro-inflammation are key pathological features of Alzheimer’s disease (AD). In addition to astrocyte and microglial reactivity, emerging evidence suggests a role of gut microbiota in regulating innate immunity and influencing brain function. Here, we examine the role of the host microbiome in regulating <span class="hlt">amyloidosis</span> in the APPSWE/PS1ΔE9 mouse model of AD. We show that prolonged shifts in gut microbial composition and diversity induced by long-term broad-spectrum combinatorial antibiotic treatment regime decreases Aβ plaque deposition. We also show that levels of soluble Aβ are elevated and that levels of circulating cytokine and chemokine signatures are altered in this setting. Finally, we observe attenuated plaque-localised glial reactivity in these mice and significantly altered microglial morphology. These findings suggest the gut microbiota community diversity can regulate host innate immunity mechanisms that impact Aβ <span class="hlt">amyloidosis</span>. PMID:27443609</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/24521938','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/24521938"><span id="translatedtitle">An autopsy case of macroglobulinemia complicated with syndrome of inappropriate secretion of ADH (SIADH) like hyponatremia, hypopituitarism and <span class="hlt">AL</span> <span class="hlt">amyloidosis</span>.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Yamada, Chika; Yoneda, Chihiro; Ogino, Jun; Fukushima, Sayaka; Kodama, Shoko; Asano, Chihiro; Masuda, Michihiko; Horie-Tajima, Kanako; Toyonaga, Aiko; Hiroshima, Kenzo; Kawamura, Shunji; Hashimoto, Naotake</p> <p>2014-01-01</p> <p>An 88-year-old male patient with macroglobulinemia was admitted to our hospital because of severe hyponatremia and unconsciousness. Laboratory findings showed decreased inhibition of antidiuretic hormone (ADH) and he was diagnosed with syndrome of inappropriate secretion of ADH (SIADH). Hyponatremia improved with only limitation of water intake and the patient was followed up on a continuing outpatient basis. However, soon after discharge from hospital, his legs started swelling with edema and hyponatremia worsened. He was re-admitted due to a fall at home. Hyponatremia was observed at re-admission. A CRH challenge test showed partial dysfunction of ACTH secretion. Corticosteroid therapy was performed, but the patient subsequently died from pneumonia. Pathological findings at autopsy revealed invasion of plasma cells and amyloid depositions in multiple organs, including the pituitary, adrenal cortex, heart, liver, kidney, lymph nodes and bone marrow. Consistent with these results, fibrosis was observed in the anterior lobe of the pituitary, suggesting that the autopsy findings were related to the clinical observations and diagnosis. This is the first reported case of macroglobulinemia complicated with multiple hormone dysfunction.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/25391376','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/25391376"><span id="translatedtitle">Experimental transmission of AA <span class="hlt">amyloidosis</span> by injecting the AA amyloid protein into interleukin-1 receptor antagonist knockout (IL-1raKO) mice.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Watanabe, K; Uchida, K; Chambers, J K; Tei, M; Shoji, A; Ushio, N; Nakayama, H</p> <p>2015-05-01</p> <p>The incidence of AA <span class="hlt">amyloidosis</span> is high in humans with rheumatoid arthritis and several animal species, including cats and cattle with prolonged inflammation. AA <span class="hlt">amyloidosis</span> can be experimentally induced in mice using severe inflammatory stimuli and a coinjection of AA amyloid; however, difficulties have been associated with transmitting AA <span class="hlt">amyloidosis</span> to a different animal species, and this has been attributed to the "species barrier." The interleukin-1 receptor antagonist knockout (IL-1raKO) mouse, a rodent model of human rheumatoid arthritis, has been used in the transmission of AA amyloid. When IL-1raKO and BALB/c mice were intraperitoneally injected with mouse AA amyloid together with a subcutaneous pretreatment of 2% AgNO3, all mice from both strains that were injected with crude or purified murine AA amyloid developed AA <span class="hlt">amyloidosis</span>. However, the amyloid index, which was determined by the intensity of AA amyloid deposition, was significantly higher in IL-1raKO mice than in BALB/c mice. When IL-1raKO and BALB/c mice were injected with crude or purified bovine AA amyloid together with the pretreatment, 83% (5/6 cases) and 38% (3/8 cases) of IL-1raKO mice and 17% (1/6 cases) and 0% (0/6 cases) of BALB/c mice, respectively, developed AA <span class="hlt">amyloidosis</span>. Similarly, when IL-1raKO and BALB/c mice were injected with crude or purified feline AA amyloid, 33% (2/6 cases) and 88% (7/8 cases) of IL-1raKO mice and 0% (0/6 cases) and 29% (2/6 cases) of BALB/c mice, respectively, developed AA <span class="hlt">amyloidosis</span>. These results indicated that IL-1raKO mice are a useful animal model for investigating AA amyloidogenesis.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4395364','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4395364"><span id="translatedtitle">First-Principles Calculations, Experimental Study, and Thermodynamic Modeling of the <span class="hlt">Al</span>-Co-Cr <span class="hlt">System</span></span></a></p> <p><a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pmc">PubMed Central</a></p> <p>Liu, Xuan L.; Gheno, Thomas; Lindahl, Bonnie B.; Lindwall, Greta; Gleeson, Brian; Liu, Zi-Kui</p> <p>2015-01-01</p> <p>The phase relations and thermodynamic properties of the condensed <span class="hlt">Al</span>-Co-Cr ternary alloy <span class="hlt">system</span> are investigated using first-principles calculations based on density functional theory (DFT) and phase-equilibria experiments that led to X-ray diffraction (XRD) and electron probe micro-analysis (EPMA) measurements. A thermodynamic description is developed by means of the calculations of phase diagrams (CALPHAD) method using experimental and computational data from the present work and the literature. Emphasis is placed on modeling the bcc-A2, B2, fcc-γ, and tetragonal-σ phases in the temperature range of 1173 to 1623 K. Liquid, bcc-A2 and fcc-γ phases are modeled using substitutional solution descriptions. First-principles special quasirandom structures (SQS) calculations predict a large bcc-A2 (disordered)/B2 (ordered) miscibility gap, in agreement with experiments. A partitioning model is then used for the A2/B2 phase to effectively describe the order-disorder transitions. The critically assessed thermodynamic description describes all phase equilibria data well. A2/B2 transitions are also shown to agree well with previous experimental findings. PMID:25875037</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/25875037','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/25875037"><span id="translatedtitle">First-principles calculations, experimental study, and thermodynamic modeling of the <span class="hlt">Al</span>-Co-Cr <span class="hlt">system</span>.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Liu, Xuan L; Gheno, Thomas; Lindahl, Bonnie B; Lindwall, Greta; Gleeson, Brian; Liu, Zi-Kui</p> <p>2015-01-01</p> <p>The phase relations and thermodynamic properties of the condensed <span class="hlt">Al</span>-Co-Cr ternary alloy <span class="hlt">system</span> are investigated using first-principles calculations based on density functional theory (DFT) and phase-equilibria experiments that led to X-ray diffraction (XRD) and electron probe micro-analysis (EPMA) measurements. A thermodynamic description is developed by means of the calculations of phase diagrams (CALPHAD) method using experimental and computational data from the present work and the literature. Emphasis is placed on modeling the bcc-A2, B2, fcc-γ, and tetragonal-σ phases in the temperature range of 1173 to 1623 K. Liquid, bcc-A2 and fcc-γ phases are modeled using substitutional solution descriptions. First-principles special quasirandom structures (SQS) calculations predict a large bcc-A2 (disordered)/B2 (ordered) miscibility gap, in agreement with experiments. A partitioning model is then used for the A2/B2 phase to effectively describe the order-disorder transitions. The critically assessed thermodynamic description describes all phase equilibria data well. A2/B2 transitions are also shown to agree well with previous experimental findings.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/22392177','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/22392177"><span id="translatedtitle">Structural and optical properties of low temperature grown <span class="hlt">Al</span>N films on sapphire using helicon sputtering <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Chen, Meei-Ru; Chen, Hou-Guang; Kao, Hui-Ling Wu, Ming-Guei; Tzou, An-Jye; Chen, Jyh Shin; Chou, Hsiung</p> <p>2015-05-15</p> <p><span class="hlt">Al</span>N thin films have been deposited directly on c-plane sapphire substrates at low temperatures by a helicon sputtering <span class="hlt">system</span>. The structural quality of <span class="hlt">Al</span>N epitaxial films was characterized by x-ray diffractometry and transmission electron microscopy. The films exhibit smooth surface with root-mean-square roughness as small as 0.7 nm evaluated by atomic force microscope. The optical transmittance spectra show a steep absorption edge at the wavelength of 200 nm and a high transmittance of over 80% in the visible range. The band-edge transition (6.30 eV) of <span class="hlt">Al</span>N film was observed in the cathodoluminescence spectrum recorded at 11 K. The spectral response of metal–semiconductor–metal photodetectors constructed with <span class="hlt">Al</span>N/sapphire reveals the peak responsivity at 200 nm and a UV/visible rejection ratio of about two orders of magnitude. The results of this low temperature deposition suggest the feasibility of the epitaxial growth of <span class="hlt">Al</span>N on sapphire substrates and the incorporation of the <span class="hlt">Al</span>N films in the surface acoustic wave devices and the optical devices at deep ultraviolet region.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://hdl.handle.net/2060/19900019290','NASA-TRS'); return false;" href="http://hdl.handle.net/2060/19900019290"><span id="translatedtitle">Liquid Rocket Booster (LRB) for the Space Transportation <span class="hlt">System</span> (STS) <span class="hlt">systems</span> study. Appendix F: Performance and trajectory for <span class="hlt">ALS</span>/LRB launch vehicles</span></a></p> <p><a target="_blank" href="http://ntrs.nasa.gov/search.jsp">NASA Technical Reports Server (NTRS)</a></p> <p></p> <p>1989-01-01</p> <p>By simply combining two baseline pump-fed LOX/RP-1 Liquid Rocket Boosters (LRBs) with the Denver core, a launch vehicle (Option 1 Advanced Launch <span class="hlt">System</span> (<span class="hlt">ALS</span>)) is obtained that can perform both the 28.5 deg (<span class="hlt">ALS</span>) mission and the polar orbit <span class="hlt">ALS</span> mission. The Option 2 LRB was obtained by finding the optimum LOX/LH2 engine for the STS/LRB reference mission (70.5 K lb payload). Then this engine and booster were used to estimate <span class="hlt">ALS</span> payload for the 28.5 deg inclination <span class="hlt">ALS</span> mission. Previous studies indicated that the optimum number of STS/LRB engines is four. When the engine/booster sizing was performed, each engine had 478 K lb sea level thrust and the booster carried 625,000 lb of useable propellant. Two of these LRBs combined with the Denver core provided a launch vehicle that meets the payload requirements for both the <span class="hlt">ALS</span> and STS reference missions. The Option 3 LRB uses common engines for the cores and boosters. The booster engines do not have the nozzle extension. These engines were sized as common <span class="hlt">ALS</span> engines. An <span class="hlt">ALS</span> launch vehicle that has six core engines and five engines per booster provides 109,100 lb payload for the 28.5 deg mission. Each of these LOX/LH2 LRBs carries 714,100 lb of useable propellant. It is estimated that the STS/LRB reference mission payload would be 75,900 lb.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/605755','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/605755"><span id="translatedtitle">Formation and stability of {beta}-quartz solid-solution phase in the Li-Si-<span class="hlt">Al</span>-O-N <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Nordmann, A.; Cheng, Y.B.</p> <p>1997-12-01</p> <p>The development of crystalline phases in lithium oxynitride glass-ceramics was examined, with particular emphasis placed on the effect of the nitrogen source (<span class="hlt">Al</span>N or Si{sub 3}N{sub 4}) on the formation and stability of a {beta}-quartz solid-solution (ss) phase. Oxynitride glasses derived from the Li-Si-<span class="hlt">Al</span>-O-N <span class="hlt">system</span> were heated-treated at temperatures up to 1,200 C to yield glass-ceramics in which {beta}-quartz(ss) and {beta}-spodumene(ss) of approximate composition Li{sub 2}O {center_dot} <span class="hlt">Al</span>{sub 2}O{sub 3} {center_dot} 4SiO{sub 2} formed as major phases and in which X-phase (Si{sub 3}<span class="hlt">Al</span>{sub 6}O{sub 12}N{sub 2}) and silicon oxynitride (Si{sub 2}N{sub 2}O) were present as minor phases. The nitrogen-containing {beta}-quartz(ss) phase that was prepared with <span class="hlt">Al</span>N was stable at 1,200 C; however, the use of Si{sub 3}N{sub 4} as the nitrogen source was significantly less effective in promoting such thermal stabilization. Lattice parameter measurements revealed that <span class="hlt">Al</span>N and Si{sub 3}N{sub 4} had different effects on the crystalline structures, and it was proposed that the enhanced thermal stability of the {beta}-quartz(ss) phase that was prepared with <span class="hlt">Al</span>N was due to both the replacement of oxygen by nitrogen and the positioning of excess <span class="hlt">Al</span>{sup 3+} ions into interstitial sites within the {beta}-quartz(ss) crystal lattice.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=501470','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=501470"><span id="translatedtitle">Pseudotumoral <span class="hlt">amyloidosis</span> of beta 2-microglobulin origin in the buttock of a patient receiving long term haemodialysis.</span></a></p> <p><a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pmc">PubMed Central</a></p> <p>Fernández-Alonso, J; Rios-Camacho, C; Valenzuela-Castaño, A; Rocha-Castilla, J L</p> <p>1993-01-01</p> <p>A 52 year old man who had been receiving haemodialysis for 13 years, with a history of renal tuberculosis, right ischial tuberculous osteomyelitis, and dialysis arthropathy, developed a soft tissue tumour in his left buttock. Histological analysis, immunohistological staining, and electron microscopic examination of the surgically removed tumour showed massive deposits of beta 2-microglobulin (beta 2-M) amyloid. This case shows the expanding clinical spectrum of this type of <span class="hlt">amyloidosis</span>, and it is suggested that amyloid infiltration should be considered in the differential diagnosis of gluteal tumours in these patients. Images PMID:8408708</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016JPCM...28q5302D','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016JPCM...28q5302D"><span id="translatedtitle">A basin-hopping Monte Carlo investigation of the structural and energetic properties of 55- and 561-atom bimetallic nanoclusters: the examples of the ZrCu, Zr<span class="hlt">Al</span>, and Cu<span class="hlt">Al</span> <span class="hlt">systems</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>De Souza, Douglas G.; Cezar, Henrique M.; Rondina, Gustavo G.; de Oliveira, Marcelo F.; Da Silva, Juarez L. F.</p> <p>2016-05-01</p> <p>We report a basin-hopping Monte Carlo investigation within the embedded-atom method of the structural and energetic properties of bimetallic ZrCu, Zr<span class="hlt">Al</span>, and Cu<span class="hlt">Al</span> nanoclusters with 55 and 561 atoms. We found that unary Zr55, Zr561, Cu55, Cu561, <span class="hlt">Al</span>55, and <span class="hlt">Al</span>561 <span class="hlt">systems</span> adopt the well known compact icosahedron (ICO) structure. The excess energy is negative for all <span class="hlt">systems</span> and compositions, which indicates an energetic preference for the mixing of both chemical species. The ICO structure is preserved if a few atoms of the host <span class="hlt">system</span> are replaced by different species, however, the composition limit in which the ICO structure is preserved depends on both the host and new chemical species. Using several structural analyses, three classes of structures, namely ideal ICO, nearly ICO, and distorted ICO structures, were identified. As the amounts of both chemical species change towards a more balanced composition, configurations far from the ICO structure arise and the dominant structures are nearly spherical, which indicates a strong minimization of the surface energy by decreasing the number of atoms with lower coordination on the surface. The average bond lengths follow Vegard’s law almost exactly for ZrCu and Zr<span class="hlt">Al</span>, however, this is not the case for Cu<span class="hlt">Al</span>. Furthermore, the radial distribution allowed us to identify the presence of an onion-like behavior in the surface of the 561-atom Cu<span class="hlt">Al</span> nanocluster with the <span class="hlt">Al</span> atoms located in the outermost surface shell, which can be explained by the lower surface energies of the <span class="hlt">Al</span> surfaces compared with the Cu surfaces. In ZrCu and Zr<span class="hlt">Al</span> the radial distribution indicates a nearly homogeneous distribution for the chemical species, however, with a slightly higher concentration of <span class="hlt">Al</span> atoms on the Zr<span class="hlt">Al</span> surface, which can also be explained by the lower surface energy.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/27045947','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/27045947"><span id="translatedtitle">A basin-hopping Monte Carlo investigation of the structural and energetic properties of 55- and 561-atom bimetallic nanoclusters: the examples of the ZrCu, Zr<span class="hlt">Al</span>, and Cu<span class="hlt">Al</span> <span class="hlt">systems</span>.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>De Souza, Douglas G; Cezar, Henrique M; Rondina, Gustavo G; de Oliveira, Marcelo F; Da Silva, Juarez L F</p> <p>2016-05-01</p> <p>We report a basin-hopping Monte Carlo investigation within the embedded-atom method of the structural and energetic properties of bimetallic ZrCu, Zr<span class="hlt">Al</span>, and Cu<span class="hlt">Al</span> nanoclusters with 55 and 561 atoms. We found that unary Zr55, Zr561, Cu55, Cu561, <span class="hlt">Al</span>55, and <span class="hlt">Al</span>561 <span class="hlt">systems</span> adopt the well known compact icosahedron (ICO) structure. The excess energy is negative for all <span class="hlt">systems</span> and compositions, which indicates an energetic preference for the mixing of both chemical species. The ICO structure is preserved if a few atoms of the host <span class="hlt">system</span> are replaced by different species, however, the composition limit in which the ICO structure is preserved depends on both the host and new chemical species. Using several structural analyses, three classes of structures, namely ideal ICO, nearly ICO, and distorted ICO structures, were identified. As the amounts of both chemical species change towards a more balanced composition, configurations far from the ICO structure arise and the dominant structures are nearly spherical, which indicates a strong minimization of the surface energy by decreasing the number of atoms with lower coordination on the surface. The average bond lengths follow Vegard's law almost exactly for ZrCu and Zr<span class="hlt">Al</span>, however, this is not the case for Cu<span class="hlt">Al</span>. Furthermore, the radial distribution allowed us to identify the presence of an onion-like behavior in the surface of the 561-atom Cu<span class="hlt">Al</span> nanocluster with the <span class="hlt">Al</span> atoms located in the outermost surface shell, which can be explained by the lower surface energies of the <span class="hlt">Al</span> surfaces compared with the Cu surfaces. In ZrCu and Zr<span class="hlt">Al</span> the radial distribution indicates a nearly homogeneous distribution for the chemical species, however, with a slightly higher concentration of <span class="hlt">Al</span> atoms on the Zr<span class="hlt">Al</span> surface, which can also be explained by the lower surface energy.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/servlets/purl/1221954','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/servlets/purl/1221954"><span id="translatedtitle">Simulation of atomic diffusion in the Fcc Ni<span class="hlt">Al</span> <span class="hlt">system</span>: A kinetic Monte Carlo study</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Alfonso, Dominic R.; Tafen, De Nyago</p> <p>2015-04-28</p> <p>The atomic diffusion in fcc Ni<span class="hlt">Al</span> binary alloys was studied by kinetic Monte Carlo simulation. The environment dependent hopping barriers were computed using a pair interaction model whose parameters were fitted to relevant data derived from electronic structure calculations. Long time diffusivities were calculated and the effect of composition change on the tracer diffusion coefficients was analyzed. These results indicate that this variation has noticeable impact on the atomic diffusivities. A reduction in the mobility of both Ni and <span class="hlt">Al</span> is demonstrated with increasing <span class="hlt">Al</span> content. As a result, examination of the pair interaction between atoms was carried out for the purpose of understanding the predicted trends.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://hdl.handle.net/2060/20060051711','NASA-TRS'); return false;" href="http://hdl.handle.net/2060/20060051711"><span id="translatedtitle">A Preliminary Assessment of Phase Separator Ground-Based and Reduced-Gravity Testing for <span class="hlt">ALS</span> <span class="hlt">Systems</span></span></a></p> <p><a target="_blank" href="http://ntrs.nasa.gov/search.jsp">NASA Technical Reports Server (NTRS)</a></p> <p>Hall, Nancy Rabel</p> <p>2006-01-01</p> <p>A viewgraph presentation of phase separator ground-based and reduced-gravity testing for Advanced Life Support (<span class="hlt">ALS</span>) <span class="hlt">systems</span> is shown. The topics include: 1) Multiphase Flow Technology Program; 2) Types of Separators; 3) MOBI Phase Separators; 4) Experiment set-up; and 5) Preliminary comparison/results.</p> </li> </ol> <div class="pull-right"> <ul class="pagination"> <li><a href="#" onclick='return showDiv("page_1");'>«</a></li> <li><a href="#" onclick='return showDiv("page_18");'>18</a></li> <li><a href="#" onclick='return showDiv("page_19");'>19</a></li> <li class="active"><span>20</span></li> <li><a href="#" onclick='return showDiv("page_21");'>21</a></li> <li><a href="#" onclick='return showDiv("page_22");'>22</a></li> <li><a href="#" onclick='return showDiv("page_25");'>»</a></li> </ul> </div> </div><!-- col-sm-12 --> </div><!-- row --> </div><!-- page_20 --> <div id="page_21" class="hiddenDiv"> <div class="row"> <div class="col-sm-12"> <div class="pull-right"> <ul class="pagination"> <li><a href="#" onclick='return showDiv("page_1");'>«</a></li> <li><a href="#" onclick='return showDiv("page_19");'>19</a></li> <li><a href="#" onclick='return showDiv("page_20");'>20</a></li> <li class="active"><span>21</span></li> <li><a href="#" onclick='return showDiv("page_22");'>22</a></li> <li><a href="#" onclick='return showDiv("page_23");'>23</a></li> <li><a href="#" onclick='return showDiv("page_25");'>»</a></li> </ul> </div> </div> </div> <div class="row"> <div class="col-sm-12"> <ol class="result-class" start="401"> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/1130193','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/1130193"><span id="translatedtitle">Displacement Threshold Energy and Recovery in an <span class="hlt">Al</span>-Ti Nanolayered <span class="hlt">System</span> with Intrinsic Point Defect Partitioning</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Gerboth, Matthew D.; Setyawan, Wahyu; Henager, Charles H.</p> <p>2014-01-07</p> <p>A method is established and validated using molecular dynamics (MD) to determine the displacement threshold energies as Ed in nanolayered, multilayered <span class="hlt">systems</span> of dissimilar metals. The method is applied to specifically oriented nanolayered films of <span class="hlt">Al</span>-Ti where the crystal structure and interface orientations are varied in atomic models and Ed is calculated. Methods for defect detection are developed and discussed based on prior research in the literature and based on specific crystallographic directions available in the nanolayered <span class="hlt">systems</span>. These are compared and contrasted to similar calculations in corresponding bulk materials, including fcc <span class="hlt">Al</span>, fcc Ti, hcp <span class="hlt">Al</span>, and hcp Ti. In all cases, the calculated Ed in the multilayers are intermediate to the corresponding bulk values but exhibit some important directionality. In the nanolayer, defect detection demonstrated systematic differences in the behavior of Ed in each layer. Importantly, collision cascade damage exhibits significant defect partitioning within the <span class="hlt">Al</span> and Ti layers that is hypothesized to be an intrinsic property of dissimilar nanolayered <span class="hlt">systems</span>. This type of partitioning could be partly responsible for observed asymmetric radiation damage responses in many multilayered <span class="hlt">systems</span>. In addition, a pseudo-random direction was introduced to approximate the average Ed without performing numerous simulations with random directions.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/19824733','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/19824733"><span id="translatedtitle">K3 fragment of amyloidogenic beta(2)-microglobulin forms ion channels: implication for dialysis related <span class="hlt">amyloidosis</span>.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Mustata, Mirela; Capone, Ricardo; Jang, Hyunbum; Arce, Fernando Teran; Ramachandran, Srinivasan; Lal, Ratnesh; Nussinov, Ruth</p> <p>2009-10-21</p> <p>Beta(2)-microglobulin (beta(2)m) amyloid deposits are linked to dialysis-related <span class="hlt">amyloidosis</span> (DRA) in hemodialysis patients. The mechanism by which beta(2)m causes DRA is not understood. It is also unclear whether only the full-length beta(2)m induces pathophysiology or if proteolytic fragments are sufficient for inducing this effect. Ser20-Lys41 (K3) is a digestion fragment of full-length beta(2)m. Solid state NMR (ssNMR) combined with X-ray diffraction and atomic force microscopy (AFM) revealed the characteristic oligomeric amyloid conformation of the U-turn beta-strand-turn-beta-strand motif stacked in parallel and stabilized by intermolecular interactions also shown by Abeta(9-40)/Abeta(17-42) and the CA150 WW domain. Here we use the K3 U-turn atomic coordinates and molecular dynamic (MD) simulations to model K3 channels in the membrane. Consistent with previous AFM imaging of other amyloids that show channel-like structures in the membrane, in the simulations K3 also forms ion channels with 3-6 loosely attached mobile subunits. We carry out AFM, single channel electrical recording, and fluorescence imaging experiments. AFM images display 3D ion channel topography with shapes, morphologies, and dimensions consistent with the theoretical model. Electrical conductance measurements indicate multiple single channel conductances, suggesting that various K3 oligomer sizes can constitute the channel structure. Fluorescence measurements in kidney cells show channel-mediated cell calcium uptake. These results suggest that the beta(2)m-induced DRA can be mediated by ion channels formed by its K3 fragment. Because the beta-strand-turn-beta-strand motif appears to be a universal amyloid feature, its ability to form ion channels further suggests that the motif may play a generic role in toxicity.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/cgi-bin/nph-data_query?bibcode=2016APLM....4f6101A&link_type=ABSTRACT','NASAADS'); return false;" href="http://adsabs.harvard.edu/cgi-bin/nph-data_query?bibcode=2016APLM....4f6101A&link_type=ABSTRACT"><span id="translatedtitle">Tunnel and electrostatic coupling in graphene-La<span class="hlt">Al</span>O3/SrTiO3 hybrid <span class="hlt">systems</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Aliaj, I.; Torre, I.; Miseikis, V.; di Gennaro, E.; Sambri, A.; Gamucci, A.; Coletti, C.; Beltram, F.; Granozio, F. M.; Polini, M.; Pellegrini, V.; Roddaro, S.</p> <p>2016-06-01</p> <p>We report on the transport properties of hybrid devices obtained by depositing graphene on a La<span class="hlt">Al</span>O3/SrTiO3 oxide junction hosting a 4 nm-deep 2-dimensional electron <span class="hlt">system</span>. At low graphene-oxide inter-layer bias, the two electron <span class="hlt">systems</span> are electrically isolated, despite their small spatial separation. A very efficient reciprocal gating of the two neighboring 2-dimensional <span class="hlt">systems</span> is shown. A pronounced rectifying behavior is observed for larger bias values and ascribed to the interplay between electrostatic field-effects and tunneling across the La<span class="hlt">Al</span>O3 barrier. The relevance of these results in the context of strongly coupled bilayer <span class="hlt">systems</span> is discussed.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/24196169','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/24196169"><span id="translatedtitle">Local decomposition induced by dislocation motions inside tetragonal <span class="hlt">Al</span>(2)Cu compound: slip <span class="hlt">system</span>-dependent dynamics.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Chen, D; Ma, X L</p> <p>2013-11-07</p> <p>Dislocations in a crystal are usually classified into several independent slip <span class="hlt">systems</span>. Motion of a partial dislocation in monometallic crystals may remove or create stacking fault characterized with a partial of a lattice translation vector. However, it is recently known that motion of partial dislocations in complex structure, such as that inside an intermetallic <span class="hlt">Al</span>2Cu compound, lead to a local composition deviation from its stoichiometric ratio and the resultant structure collapse. Here we report such a local decomposition behaviors are strongly dependent on slip <span class="hlt">system</span> of dislocations. Under applied external stress, we have studied dislocation motion behaviors in the three independent slip <span class="hlt">systems</span> of [001](110), [100]() and [110]() within tetragonal <span class="hlt">Al</span>2Cu crystal by using molecular dynamics method. We found dislocation motions in all these slip <span class="hlt">systems</span> result in local decomposition but their physical details differ significantly.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2013ApPhL.102x2113Y','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2013ApPhL.102x2113Y"><span id="translatedtitle">Remotely sensed transport in microwave photoexcited GaAs/<span class="hlt">Al</span>GaAs two-dimensional electron <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Ye, Tianyu; Mani, R. G.; Wegscheider, W.</p> <p>2013-06-01</p> <p>We demonstrate a strong correlation between the magnetoresistive response and the concurrent microwave reflection from the microwave photo-excited GaAs/<span class="hlt">Al</span>GaAs two-dimensional electron <span class="hlt">system</span> (2DES). These correlations are followed as a function of the microwave power, the microwave frequency, and the applied current. Notably, the character of the reflection signal remains unchanged even when the current is switched off in the GaAs/<span class="hlt">Al</span>GaAs Hall bar specimen. The results suggest a perceptible microwave-induced change in the electronic properties of the 2DES, even in the absence of an applied current.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/1985JaJAP..24L.143M','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/1985JaJAP..24L.143M"><span id="translatedtitle">Direct Evidence for the DX Center Being a Substitutional Donor in <span class="hlt">Al</span>GaAs Alloy <span class="hlt">System</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Mizuta, Masashi; Tachikawa, Masami; Kukimoto, Hiroshi; Minomura, Shigeru</p> <p>1985-02-01</p> <p>An experimental result that the DX center appears in GaAs:Si and GaAsd:Sn under hydrostatic pressure of about 30 kbars has been obtained for the first time. This indicates clearly that the DX center in the <span class="hlt">Al</span>GaAs alloy <span class="hlt">system</span> is due to a substitutional donor itself (not a complex referred to as “DX”). The change in nature from the the shallow donor to the deep DX center is discussed based on the complex multivalley conduction band structure of GaAs under various pressures and of <span class="hlt">Al</span>GaAs with various compositions.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/22492517','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/22492517"><span id="translatedtitle">Study of the magnetic properties, structure, and phase transformation in the alloys of the Co-<span class="hlt">Al</span>-W <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Davidov, D. I. Stepanova, N. N. Kazantseva, N. V. Rigmant, M. B. Shishkin, D. A.</p> <p>2015-10-27</p> <p>An experimental study of phase transformations in the <span class="hlt">system</span> of Co-<span class="hlt">Al</span>-W in the concentration area of the intermetallic compound Co{sub 3}(<span class="hlt">Al</span>, W) is presented. The structure and phase composition of the Co–9 at % Al–X at % W (X = 4.5, 6.8, 8.5, 10, 12.5) alloys in depending on the tungsten content are analyzed. The Curie temperature and magnetic properties of the alloys with the different phase composition are determined.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://ntrs.nasa.gov/search.jsp?R=19860047008&hterms=Ytterbium&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D50%26Ntt%3DYtterbium','NASA-TRS'); return false;" href="http://ntrs.nasa.gov/search.jsp?R=19860047008&hterms=Ytterbium&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D50%26Ntt%3DYtterbium"><span id="translatedtitle">Optimization of the Ni-Cr-<span class="hlt">Al</span>-Y/ZrO2-Y2O3 thermal barrier <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://ntrs.nasa.gov/search.jsp">NASA Technical Reports Server (NTRS)</a></p> <p>Stecura, S.</p> <p>1986-01-01</p> <p>The effects of bond and thermal barrier coating compositions, thicknesses, and densities on air plasma spray deposited Ni-Cr-<span class="hlt">Al</span>-Y/ZrO2-Y2O3 life were evaluated in cyclic furnace oxidation tests at temperatures from 1110 to 1220 C. An empirical relation was developed to give life as a function of the above parameters. The thermal barrier <span class="hlt">system</span> tested which had the longest life consisted of Ni-35.0 wt pct Cr-5.9 wt pct <span class="hlt">Al</span>-0.95 wt pct Y bond coating and ZrO2-6.1 wt pct Y2O3 thermal barrier coating.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://hdl.handle.net/2060/19850007569','NASA-TRS'); return false;" href="http://hdl.handle.net/2060/19850007569"><span id="translatedtitle">Optimization of the NiCr<span class="hlt">Al</span>-Y/ZrO-Y2O3 thermal barrier <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://ntrs.nasa.gov/search.jsp">NASA Technical Reports Server (NTRS)</a></p> <p>Stecura, S.</p> <p>1985-01-01</p> <p>The effects of bond and thermal barrier coating compositions, thicknesses, and densities on air plasma spray deposited Ni-Cr-<span class="hlt">Al</span>-Y/ZrO2-Y2O3 life were evaluated in cyclic furnace oxidation tests at temperatures from 1110 to 1220 C. An empirical relation was developed to give life as a function of the above parameters. The thermal barrier <span class="hlt">system</span> tested which had the longest life consisted of Ni-35.0 wt% Cr-5.9 wt% <span class="hlt">Al</span>-0.95 wt% Y bond coating and ZrO2-6.1 wt% Y2O3 thermal barrier coating.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/servlets/purl/1185327','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/servlets/purl/1185327"><span id="translatedtitle">Metastability in the Mg<span class="hlt">Al</span><sub>2</sub>O<sub>4</sub>-<span class="hlt">Al</span><sub>2</sub>O<sub>3</sub> <span class="hlt">System</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Wilkerson, Kelley R.; Smith, Jeffrey D.; Hemrick, James G.</p> <p>2014-07-22</p> <p>Aluminum oxide must take a spinel form ( γ-<span class="hlt">Al</span><sub>2</sub>O<sub>3</sub>) at elevated temperatures in order for extensive solid solution to form between Mg<span class="hlt">Al</span><sub>2</sub>O<sub>4</sub> and α-<span class="hlt">Al</span><sub>2</sub>O<sub>3</sub>. The solvus line between Mg<span class="hlt">Al</span><sub>2</sub>O<sub>4</sub> and <span class="hlt">Al</span><sub>2</sub>O<sub>3</sub> has been defined at 79.6 wt% <span class="hlt">Al</span><sub>2</sub>O<sub>3</sub> at 1500°C, 83.0 wt% <span class="hlt">Al</span><sub>2</sub>O<sub>3</sub> at 1600°C, and 86.5 wt% <span class="hlt">Al</span><sub>2</sub>O<sub>3</sub> at 1700°C. A metastable region has been defined at temperatures up to 1700°C which could have significant implications for material processing and properties. Additionally, initial processing could have major implications on final chemistry. The spinel solid solution region has been extended to form an infinite solid solution with <span class="hlt">Al</span><sub>2</sub>O<sub>3</sub> at elevated temperatures. A minimum in melting at 1975°C and a chemistry of 96 wt% <span class="hlt">Al</span><sub>2</sub>O<sub>3</sub> rather than a eutectic is present, resulting in no eutectic crystal formation during solidification.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://pubs.er.usgs.gov/publication/70014774','USGSPUBS'); return false;" href="http://pubs.er.usgs.gov/publication/70014774"><span id="translatedtitle">Heat capacity measurements for cryolite (Na3<span class="hlt">Al</span>F6) and reactions in the <span class="hlt">system</span> NaFe<span class="hlt">Al</span>SiOF</span></a></p> <p><a target="_blank" href="http://pubs.er.usgs.gov/pubs/index.jsp?view=adv">USGS Publications Warehouse</a></p> <p>Anovitz, Lawrence M.; Hemingway, B.S.; Westrum, E.F.; Metz, G.W.; Essene, E.J.</p> <p>1987-01-01</p> <p>The heat capacity of cryolite (Na3<span class="hlt">Al</span>F6) has been measured from 7 to 1000 K by low-temperature adiabatic and high-temperature differential scanning calorimetry. Low-temperature data were obtained on material from the same hand specimen in the calorimetric laboratories of the University of Michigan and U.S. Geological Survey. The results obtained are in good agreement, and yield average values for the entropy of cryolite of: S0298 = 238.5 J/mol KS0T-S0298 = 145.114 ln T+ 193.009*10-3T- 10.366* 105 T2- 872.89 J/mol K (273-836.5 K)??STrans = 9.9J/mol KS0T-S0298 =198.414 ln T+73.203* 10-3T-63.814* 105 T2-1113.11 J/mol K (836.5-1153 K) with the transition temperature between ??- and ??-cryolite taken at 836.5 K. These data have been combined with data in the literature to calculate phase equilibria for the <span class="hlt">system</span> NaFe<span class="hlt">Al</span>SiOF. The resultant phase diagrams allow constraints to be placed on the fO2, fF2, aSiO2 and T conditions of formation for assemblages in alkalic rocks. A sample application suggests that log fO2 is approximately -19.2, log fF2 is -31.9 to -33.2, and aSiO2 is -1.06 at assumed P T conditions of 1000 K, 1 bar for the villiaumite-bearing Ilimaussaq intrusion in southwestern Greenland. ?? 1987.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016ApSS..360..559S','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016ApSS..360..559S"><span id="translatedtitle">Single- and dual-wavelength laser pulses induced modification in 10×(<span class="hlt">Al</span>/Ti)/Si multilayer <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Salatić, B.; Petrović, S.; Peruško, D.; Čekada, M.; Panjan, P.; Pantelić, D.; Jelenković, B.</p> <p>2016-01-01</p> <p>The surface morphology of the ablation craters created in the multilayer 10×(<span class="hlt">Al</span>/Ti)/Si <span class="hlt">system</span> by nanosecond laser pulses at single- and dual wavelength has been studied experimentally and numerically. A complex multilayer thin film including ten (<span class="hlt">Al</span>/Ti) bilayers deposited by ion sputtering on Si(1 0 0) substrate to a total thickness of 260 nm were illuminated at different laser irradiance in the range 0.25-3.5 × 109 W cm-2. Single pulse laser irradiation was done at normal incidence in air, with the single wavelength, either at 532 nm or 1064 nm or with both laser light simultaneously in the ratio of 1:10 for energy per pulse between second harmonic and 1064 nm. Most of the absorbed laser energy was rapidly transformed into heat, producing intensive modifications of composition and morphology on the sample surface. The results show an increase in surface roughness, formation of specific nanostructures, appearance of hydrodynamic features and ablation of surface material with crater formation. Applying a small fraction (10%) of the second harmonic in dual-wavelength pulses, a modification of the 10×(<span class="hlt">Al</span>/Ti)/Si <span class="hlt">system</span> by a single laser pulse was reflected in the formation of wider and/or deeper craters. Numerical calculations show that the main physical mechanism in ablation process is normal evaporation without phase explosion. The calculated and experimental results agree relatively well for the whole irradiance range, what makes the model applicable to complex <span class="hlt">Al</span>/Ti multilayer <span class="hlt">systems</span>.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2014ChPhL..31b8502X','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2014ChPhL..31b8502X"><span id="translatedtitle">Sensing Characteristics of Shear-Mode <span class="hlt">Al</span>N Solidly Mounted Resonators with a Silicone Microfluidic <span class="hlt">System</span> in Viscous Media</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Xiong, Juan; Guo, Peng; Sun, Xi-Liang; Wang, Sheng-Fu; Hu, Ming-Zhe; Gu, Hao-Shuang</p> <p>2014-02-01</p> <p><span class="hlt">Al</span>N solidly mounted resonators with silicone microfluidic <span class="hlt">systems</span> vibrating in shear mode are fabricated and characterized. The fabrication process is compatible with integrated circuits and the c-axis tilted <span class="hlt">Al</span>N films are deposited, which allow in-liquid operation through excitation of the shear mode. The silicone microfluidic <span class="hlt">system</span> is mounted on top of the sensor chip to transport the analyses and confine the flow to the active area. The properties of sensor operation in air, deionized water, ethanol, isopropanol, 80% glycol aqueous solution, glycol, and olive oil are characterized. The effects of different viscosities on the resonance frequency shift and Q-factor of the sensor have been discussed. The sensitivity and Q value in glycol of the sensor are 1.52 MHz cm2/μg and around 60, respectively. The results indicate the potential of a highly sensitive microfluidic sensor <span class="hlt">system</span> for the applications in viscous media.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.ncbi.nlm.nih.gov/pubmed/22609675','PUBMED'); return false;" href="http://www.ncbi.nlm.nih.gov/pubmed/22609675"><span id="translatedtitle">Conversion of glucose into furans in the presence of <span class="hlt">Al</span>Cl3 in an ethanol-water solvent <span class="hlt">system</span>.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Yang, Yu; Hu, Changwei; Abu-Omar, Mahdi M</p> <p>2012-07-01</p> <p>Glucose was converted into furans (5-hydroxymethylfurfural and 5-ethoxymethylfurfural) in the presence of <span class="hlt">Al</span>Cl(3) in an ethanol-water solvent <span class="hlt">system</span>. The <span class="hlt">system</span> showed high activity for the conversion of glucose into furans but low activity for the subsequent formation of LAs (levulinic acid and ethyl levulinate). High furans yield of 57% with low LAs yield of 11% can be obtained at 160 °C within 15 min. Glucose-based disaccharides (sucrose, maltose and cellobiose) and polysaccharides (starch but not cellulose) can also be converted to furans effectively under the same condition. <span class="hlt">Al</span>Cl(3) can be used to prepare furans from biomass-derived compounds in ethanol-water, a green solvent <span class="hlt">system</span>. PMID:22609675</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/21284495','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/21284495"><span id="translatedtitle">A marked decline in the incidence of renal replacement therapy for <span class="hlt">amyloidosis</span> associated with inflammatory rheumatic diseases - data from nationwide registries in Finland.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Immonen, Kai; Finne, Patrik; Grönhagen-Riska, Carola; Pettersson, Tom; Klaukka, Timo; Kautiainen, Hannu; Hakala, Markku</p> <p>2011-03-01</p> <p>Risk for <span class="hlt">amyloidosis</span> in rheumatic diseases is associated with a long-lasting inflammation. To assess possible changes in the incidence of terminal uraemia due to <span class="hlt">amyloidosis</span> associated with rheumatic diseases on a nationwide basis, we scrutinised the files of the Finnish Registry for Kidney Diseases for patients suffering from <span class="hlt">amyloidosis</span> associated with rheumatoid arthritis (RA), ankylosing spondylitis (AS) or juvenile idiopathic arthritis (JIA) over the period 1995-2008. The registry has an estimated 97-99% coverage of all patients accepted for renal replacement therapy (RRT) in the country. Data on the consumption of antirheumatic drugs were collected from two sources: the Social Insurance Institution's Drug Reimbursement Register, and the Sales Register of the National Agency for Medicines from the above period. Altogether 264 cases were identified. Two hundred twenty-nine of them had RA, 15 AS and 20 JIA. When the total annual number of new admissions to RRT varied between 20 and 37 at the end of 1990s, it was under half of that from 2002 onwards. Over this period, the number of users of low-dose methotrexate (MTX) has increased 3.6-fold, the drug being the most frequently used disease modifying anti-rheumatic drug in Finland. The present nationwide series is the first to show that the incidence of end-stage renal disease due to <span class="hlt">amyloidosis</span> associated with rheumatic diseases is decreasing. An obvious reason for this is intensive anti-rheumatic drug therapy.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/pages/biblio/1221954-simulation-atomic-diffusion-fcc-nial-system-kinetic-monte-carlo-study','SCIGOV-DOEP'); return false;" href="http://www.osti.gov/pages/biblio/1221954-simulation-atomic-diffusion-fcc-nial-system-kinetic-monte-carlo-study"><span id="translatedtitle">Simulation of atomic diffusion in the Fcc Ni<span class="hlt">Al</span> <span class="hlt">system</span>: A kinetic Monte Carlo study</span></a></p> <p><a target="_blank" href="http://www.osti.gov/pages">DOE PAGESBeta</a></p> <p>Alfonso, Dominic R.; Tafen, De Nyago</p> <p>2015-04-28</p> <p>The atomic diffusion in fcc Ni<span class="hlt">Al</span> binary alloys was studied by kinetic Monte Carlo simulation. The environment dependent hopping barriers were computed using a pair interaction model whose parameters were fitted to relevant data derived from electronic structure calculations. Long time diffusivities were calculated and the effect of composition change on the tracer diffusion coefficients was analyzed. These results indicate that this variation has noticeable impact on the atomic diffusivities. A reduction in the mobility of both Ni and <span class="hlt">Al</span> is demonstrated with increasing <span class="hlt">Al</span> content. As a result, examination of the pair interaction between atoms was carried out formore » the purpose of understanding the predicted trends.« less</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2011RJPCA..85.1495L','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2011RJPCA..85.1495L"><span id="translatedtitle">Activities of the components in a spinel solid solution of the Fe-<span class="hlt">Al</span>-O <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Lykasov, A. A.; Kimyashev, A. A.</p> <p>2011-09-01</p> <p>The conditions of the equilibrium between the Fe3O4-Fe<span class="hlt">Al</span>2O4 solution and wustite are determined by measuring the EMF of galvanic cells containing a solid electrolyte, and the activities of the components in the Fe3O4-Fe<span class="hlt">Al</span>2O4 solution are calculated by treating the results of the experiment on the equilibrium between the spinel solution and wustite. Their properties are found to be different from those of ideal solutions at temperatures of 1000-1300 K. A significant positive deviation from the Raoult's law is believed to indicate the tendency of the solution to decompose. The experimental data are treated in terms of the theory of regular solutions, assuming the energy of mixing to be a function of temperature only. The critical temperature of decomposition for the Fe3O4-Fe<span class="hlt">Al</span>2O4 solution is found to be 1084 K.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://ntrs.nasa.gov/search.jsp?R=20050168081&hterms=copper&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D70%26Ntt%3Dcopper','NASA-TRS'); return false;" href="http://ntrs.nasa.gov/search.jsp?R=20050168081&hterms=copper&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D70%26Ntt%3Dcopper"><span id="translatedtitle">Blanch Resistant and Thermal Barrier Ni<span class="hlt">Al</span> Coating <span class="hlt">Systems</span> for Advanced Copper Alloys</span></a></p> <p><a target="_blank" href="http://ntrs.nasa.gov/search.jsp">NASA Technical Reports Server (NTRS)</a></p> <p>Raj, Sai V. (Inventor)</p> <p>2005-01-01</p> <p>A method of forming an environmental resistant thermal barrier coating on a copper alloy is disclosed. The steps include cleansing a surface of a copper alloy, depositing a bond coat on the cleansed surface of the copper alloy, depositing a Ni<span class="hlt">Al</span> top coat on the bond coat and consolidating the bond coat and the Ni<span class="hlt">Al</span> top coat to form the thermal barrier coating. The bond coat may be a nickel layer or a layer composed of at least one of copper and chromium-copper alloy and either the bond coat or the Ni<span class="hlt">Al</span> top coat or both may be deposited using a low pressure or vacuum plasma spray.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2015ChPhB..24h7304L','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2015ChPhB..24h7304L"><span id="translatedtitle">Charge trapping behavior and its origin in <span class="hlt">Al</span>2O3/SiC MIS <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Liu, Xin-Yu; Wang, Yi-Yu; Peng, Zhao-Yang; Li, Cheng-Zhan; Wu, Jia; Bai, Yun; Tang, Yi-Dan; Liu, Ke-An; Shen, Hua-Jun</p> <p>2015-08-01</p> <p>Charge trapping behavior and its origin in <span class="hlt">Al</span>2O3/SiC MOS structure are investigated by analyzing the capacitance-voltage (C-V) hysteresis and the chemical composition of the interface. The C-V hysteresis is measured as a function of oxide thickness series for an <span class="hlt">Al</span>2O3/SiC MIS capacitor. The distribution of the trapped charges, extracted from the C-V curves, is found to mainly follow a sheet charge model rather than a bulk charge model. Therefore, the electron injection phenomenon is evaluated by using linear fitting. It is found that most of the trapped charges are not distributed exactly at the interface but are located in the bulk of the <span class="hlt">Al</span>2O3 layers, especially close to the border. Furthermore, there is no detectable oxide interface layer in the x-ray photoelectron spectroscope (XPS) and transmission electron microscope (TEM) measurements. In addition, Rutherford back scattering (RBS) analysis shows that the width of the <span class="hlt">Al</span>2O3/SiC interface is less than 1 nm. It could be concluded that the charge trapping sites in <span class="hlt">Al</span>2O3/SiC structure might mainly originate from the border traps in <span class="hlt">Al</span>2O3 film rather than the interface traps in the interfacial transition layer. Project supported by the National Natural Science Foundation of China (Grant No. 61106080) and the National Science and Technology Major Project of the Ministry of Science and Technology of China (Grant No. 2013ZX02305).</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016JMMM..411..103Z','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016JMMM..411..103Z"><span id="translatedtitle">Spin Hall magnetoresistance in an ultrathin Co2Fe<span class="hlt">Al</span> <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Zhang, Yan-qing; Fu, Hua-rui; Sun, Niu-yi; Che, Wen-ru; Ding, Ding; Qin, Juan; You, Cai-yin; Shan, Rong; Zhu, Zhen-gang</p> <p>2016-08-01</p> <p>Spin Hall magnetoresistance (SMR) is observed in an ultrathin Co2Fe<span class="hlt">Al</span> layer covered by a thin Pt film. The Co2Fe<span class="hlt">Al</span> layer grown on a MgO substrate should be too thin to be continuous. The result reveals that the magnetic insulator layer, such as yttrium iron garnet (YIG) substrate which is frequently used so far, is actually not a requisite for the observation of SMR. This work may greatly help to understand the true nature of SMR effect.</p> </li> </ol> <div class="pull-right"> <ul class="pagination"> <li><a href="#" onclick='return showDiv("page_1");'>«</a></li> <li><a href="#" onclick='return showDiv("page_19");'>19</a></li> <li><a href="#" onclick='return showDiv("page_20");'>20</a></li> <li class="active"><span>21</span></li> <li><a href="#" onclick='return showDiv("page_22");'>22</a></li> <li><a href="#" onclick='return showDiv("page_23");'>23</a></li> <li><a href="#" onclick='return showDiv("page_25");'>»</a></li> </ul> </div> </div><!-- col-sm-12 --> </div><!-- row --> </div><!-- page_21 --> <div id="page_22" class="hiddenDiv"> <div class="row"> <div class="col-sm-12"> <div class="pull-right"> <ul class="pagination"> <li><a href="#" onclick='return showDiv("page_1");'>«</a></li> <li><a href="#" onclick='return showDiv("page_20");'>20</a></li> <li><a href="#" onclick='return showDiv("page_21");'>21</a></li> <li class="active"><span>22</span></li> <li><a href="#" onclick='return showDiv("page_23");'>23</a></li> <li><a href="#" onclick='return showDiv("page_24");'>24</a></li> <li><a href="#" onclick='return showDiv("page_25");'>»</a></li> </ul> </div> </div> </div> <div class="row"> <div class="col-sm-12"> <ol class="result-class" start="421"> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016PhSS...58.1930A','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016PhSS...58.1930A"><span id="translatedtitle">Study of the KNO3-<span class="hlt">Al</span>2O3 <span class="hlt">system</span> by differential scanning calorimetry</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Amirov, A. M.; Gafurov, M. M.; Rabadanov, K. Sh.</p> <p>2016-09-01</p> <p>The structural and the thermodynamic properties of potassium nitrate KNO3 and its composites with nanosized aluminum oxide <span class="hlt">Al</span>2O3 have been studied by differential scanning calorimetry. It has been found that an amorphous phase forms in composites (1- x)KNO3- x <span class="hlt">Al</span>2O3. The thermal effect corresponding to this phase has been observed at 316°C. It has been found that the phase transition heats of potassium nitrate decreased as the aluminum oxide fraction increased.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/22261726','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/22261726"><span id="translatedtitle">Solid state amorphization in the <span class="hlt">Al</span>-Fe binary <span class="hlt">system</span> during high energy milling</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Urban, P. Montes, J. M.; Cintas, J.</p> <p>2013-12-16</p> <p>In the present study, mechanical alloying (MA) of <span class="hlt">Al</span>75Fe25 elemental powders mixture was carried out in argon atmosphere, using a high energy attritor ball mill. The microstructure of the milled products at different stages of milling was characterized by X-ray diffraction (XRD), scanning electron microscopy (SEM), transmission electron microscopy (TEM) and differential scanning calorimetry (DSC). The results showed that the amorphous phase content increased by increasing the milling time, and after 50 hours the amorphization process became complete. Heating the samples resulted in the crystallization of the synthesized amorphous alloys and the appearance of the equilibrium intermetallic compounds <span class="hlt">Al</span>{sub 5}Fe{sub 2}.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016AcSpA.156...22L','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016AcSpA.156...22L"><span id="translatedtitle">A simple and rapid method for direct determination of <span class="hlt">Al</span>(III) based on the enhanced resonance Rayleigh scattering of hemin-functionalized graphene-<span class="hlt">Al</span>(III) <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Ling, Yu; Chen, Ling Xiao; Dong, Jiang Xue; Li, Nian Bing; Luo, Hong Qun</p> <p>2016-03-01</p> <p>A novel method for direct determination of <span class="hlt">Al</span>(III) by using hemin-functionalized graphene (H-GO) has been established based on the enhancement of resonance Rayleigh scattering (RRS) intensity. The characteristics of RRS spectra, the optimum reaction conditions, and the reaction mechanism have been investigated. In this experiment, the <span class="hlt">Al</span>(III) would exist in sol-gel <span class="hlt">Al</span>(OH)3 species under the condition of pH 5.9 in aqueous solutions. When H-GO existed in the solution, the sol-gel <span class="hlt">Al</span>(OH)3 would react with H-GO and result in enhancement of RRS intensity, owing to the enhanced hydrophobicity of H-GO surface. Therefore, a simple and rapid sensor for <span class="hlt">Al</span>(III) was developed. The increased intensity of RRS is directly proportional to the concentration of <span class="hlt">Al</span>(III) in the range of 10 nM-6 μM, along with a detection limit of 0.87 nM. Moreover, the sensor has been applied to determination of <span class="hlt">Al</span>(III) concentration in real water and aspirin tablet samples with satisfactory results. Therefore, the proposed method is promising as an effective means for selective and sensitive determination of <span class="hlt">Al</span>(III).</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=3951756','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=3951756"><span id="translatedtitle">CMR Imaging With Rapid Visual T1 Assessment Predicts Mortality in Patients Suspected of Cardiac <span class="hlt">Amyloidosis</span></span></a></p> <p><a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pmc">PubMed Central</a></p> <p>White, James A.; Kim, Han W.; Shah, Dipan; Fine, Nowell; Kim, Ki-Young; Wendell, David C.; Al-Jaroudi, Wael; Parker, Michele; Patel, Manesh; Gwadry-Sridhar, Femida; Judd, Robert M.; Kim, Raymond J.</p> <p>2014-01-01</p> <p>OBJECTIVES This study tested the diagnostic and prognostic utility of a rapid, visual T1 assessment method for identification of cardiac <span class="hlt">amyloidosis</span> (CA) in a “real-life” referral population undergoing cardiac magnetic resonance for suspected CA. BACKGROUND In patients with confirmed CA, delayed-enhancement cardiac magnetic resonance (DE-CMR) frequently shows a diffuse, global hyperenhancement (HE) pattern. However, imaging is often technically challenging, and the prognostic significance of diffuse HE is unclear. METHODS Ninety consecutive patients referred for suspected CA and 64 hypertensive patients with left ventricular hypertrophy (LVH) were prospectively enrolled and underwent a modified DE-CMR protocol. After gadolinium administration a method for rapid, visual T1 assessment was used to identify the presence of diffuse HE during the scan, allowing immediate optimization of settings for the conventional DE-CMR that followed. The primary endpoint was all-cause mortality. RESULTS Among patients with suspected CA, 66% (59 of 90) demonstrated HE, with 81% (48 of 59) of these meeting pre-specified visual T1 assessment criteria for diffuse HE. Among hypertensive LVH patients, 6% (4 of 64) had HE, with none having diffuse HE. During 29 months of follow-up (interquartile range: 12 to 44 months), there were 50 (56%) deaths in patients with suspected CA and 4 (6%) in patients with hypertensive LVH. Multivariable analysis demonstrated that the presence of diffuse HE was the most important predictor of death in the group with suspected CA (hazard ratio: 5.5, 95% confidence interval: 2.7 to 11.0; p < 0.0001) and in the population as a whole (hazard ratio: 6.0, 95% confidence interval 3.0 to 12.1; p < 0.0001). Among 25 patients with myocardial histology obtained during follow-up, the sensitivity, specificity, and accuracy of diffuse HE in the diagnosis of CA were 93%, 70%, and 84%, respectively. CONCLUSIONS Among patients suspected of CA, the presence of diffuse HE by</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/servlets/purl/7000272','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/servlets/purl/7000272"><span id="translatedtitle">Phase stability of fcc- and hcp-based intermetallics: The Ti-<span class="hlt">Al</span> and Cd-Mg <span class="hlt">systems</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Asta, M.; McCormack, R. . Dept. of Materials Science and Mineral Engineering); van Schilfgaarde, M. ); Ceder, G. . Dept. of Materials Science); de Fontaine, D. . Dept. of Materials Science and Mi</p> <p>1992-06-01</p> <p>In this paper we summarize results of first-principles phase stability studies of fcc- and hcp-based Ti-<span class="hlt">Al</span> alloys and of the hcp-based Cd-Mg <span class="hlt">system</span>. In particular, heats of formation for ordered alloy compounds are calculated with the linear muffin tin orbital method; effective cluster interactions are determined from the results of these calculations and are used to derive thermodynamic properties and composition-temperature phase diagrams.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/servlets/purl/10154302','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/servlets/purl/10154302"><span id="translatedtitle">In situ studies of amorphization of the Ge-<span class="hlt">Al</span> and Si-<span class="hlt">Al</span> <span class="hlt">systems</span> induced by 1 MeV electron irradiation</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Lin, X.W.; Seidman, D.N. |; Okamoto, P.R.</p> <p>1990-10-01</p> <p>Ge/<span class="hlt">Al</span> and Si/<span class="hlt">Al</span> bilayer specimens, as well as <span class="hlt">Al</span>-2.3 at. % Ge two-phase alloy specimens, were irradiated in situ with 1 MeV electrons at temperatures in the range 10--190 K in a high voltage electron microscope. At 10 K, it is found that for a Ge/<span class="hlt">Al</span> bilayer specimen, amorphization at the interface occurs only when the Ge layer faces the incident electron beam, while for a Si/<span class="hlt">Al</span> bilayer specimen amorphization occurs regardless of the direction of the incident beam. In this case, the critical fluence for amorphization ({Phi}{sub c}) to occur is {approx} 3 {times} 10{sup 23} cm{sup {minus}2} ({approx} 30,19 and 18 displacements per atom in Ge, Si and <span class="hlt">Al</span> respectively). In the case of <span class="hlt">Al</span> -- 2.3 at. % Ge alloy specimens irradiated at 10 and 50 K a crystalline-to-amorphous (c-to-a) transition is observed at {Phi}{sub c} {approx} 2.4 {times} 10{sup 23} cm{sup {minus}2} (24 and 14 dpa in Ge and <span class="hlt">Al</span> respectively). The temperature dependence of {Phi}{sub c} is also studied for a Ge/<span class="hlt">Al</span> bilayer specimen. The value of {Phi}{sub c} is a constant for T < {approx} 160, and then it increases rapidly with increasing T; {Phi}{sub c} becomes immeasurably large at a critical temperature of {approx} 190 K. The results can be understood in terms of an electron-irradiation-induced recoil-implantation mechanism.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://ntrs.nasa.gov/search.jsp?R=19810054114&hterms=free+energy&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D30%26Ntt%3Dfree%2Benergy','NASA-TRS'); return false;" href="http://ntrs.nasa.gov/search.jsp?R=19810054114&hterms=free+energy&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D30%26Ntt%3Dfree%2Benergy"><span id="translatedtitle">The MgO-<span class="hlt">Al</span>2O3-SiO2 <span class="hlt">system</span> - Free energy of pyrope and <span class="hlt">Al</span>2O3-enstatite. [in earth mantle formation</span></a></p> <p><a target="_blank" href="http://ntrs.nasa.gov/search.jsp">NASA Technical Reports Server (NTRS)</a></p> <p>Saxena, S. K.</p> <p>1981-01-01</p> <p>The model of fictive ideal components is used to determine Gibbs free energies of formation of pyrope and <span class="hlt">Al</span>2O3-enstatite from the experimental data on coexisting garnet and orthopyroxene and orthopyroxene and spinel in the temperature range 1200-1600 K. It is noted that <span class="hlt">Al</span>2O3 forms an ideal solution with MgSiO3. These thermochemical data are found to be consistent with the <span class="hlt">Al</span>2O3 isopleths that could be drawn using most recent experimental data and with the reversed experimental data on the garnet-spinel field boundary.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016MMTB...47.2433D','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016MMTB...47.2433D"><span id="translatedtitle">Study on Viscosity of the La2O3-SiO2-<span class="hlt">Al</span>2O3 Slag <span class="hlt">System</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Deng, Yong-chun; Wu, Sheng-li; Jiang, Yin-ju; Jia, Su-qi</p> <p>2016-08-01</p> <p>The viscosities and free-running temperatures of slag in a La2O3-SiO2-<span class="hlt">Al</span>2O3 slag <span class="hlt">system</span> were measured using an internal rotating cylinder method. For different La2O3 mass contents (45, 50, and 55 pct) in the La2O3-SiO2-<span class="hlt">Al</span>2O3 ternary slag, the slag viscosity and free-running temperature decreased with a decrease in SiO2 content and an increase in <span class="hlt">Al</span>2O3 content, and decreased with an increase in La2O3 content. Minor components B2O3, FeO, and MnO could decrease the viscosity and free-running temperature of La2O3-SiO2-<span class="hlt">Al</span>2O3 ternary slag, especially FeO, and a small amount of FeO and B2O3 had an additive effect on slag viscosity and free-running temperature reduction.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/pages/biblio/1321965-structural-ordering-solid-liquid-interfaces-al-sm-system-molecular-dynamics-study','SCIGOV-DOEP'); return false;" href="http://www.osti.gov/pages/biblio/1321965-structural-ordering-solid-liquid-interfaces-al-sm-system-molecular-dynamics-study"><span id="translatedtitle">Structural ordering at solid-liquid interfaces in <span class="hlt">Al</span>-Sm <span class="hlt">system</span>: A molecular-dynamics study</span></a></p> <p><a target="_blank" href="http://www.osti.gov/pages">DOE PAGESBeta</a></p> <p>Sun, Yang; Zhang, Feng; Ye, Zhuo; Ding, Zejun; Mendelev, Mikhail I.; Kramer, Matthew J.; Wang, Cai -Zhuang; Ho, Kai -Ming</p> <p>2016-07-12</p> <p>The structural ordering at solid-liquid interfaces far from equilibrium is studied with molecular dynamics simulations for the <span class="hlt">Al</span>-Sm <span class="hlt">system</span>. Using the van-Hove self-correlation function as the criterion to identify attachment/detachment events that occur at the interface, we are able to determine the time-dependent interface position, and characterize the detailed interfacial structure ordering surrounding the attached atoms. For the interface between an undercooled <span class="hlt">Al</span>90Sm10 liquid and a metastable cubic structure, the solid induces the crystalline order of the cubic phase in the liquid layers, promoting the continuous growth of the crystal phase. When the same liquid is put in contact withmore » f.c.c. <span class="hlt">Al</span>, Sm from the liquid can still attach to the solid interface despite its insolubility in the <span class="hlt">Al</span> lattice. Non-f.c.c. order is revealed surrounding the attached Sm atoms. Lastly, we show that the local structure ordering at interface is highly correlated to solid packing and liquid ordering.« less</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/22907449','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/22907449"><span id="translatedtitle">Catalytic oxidation with <span class="hlt">Al</span>-Ce-Fe-PILC as a post-treatment <span class="hlt">system</span> for coffee wet processing wastewater.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Sanabria, Nancy R; Peralta, Yury M; Montañez, Mardelly K; Rodríguez-Valencia, Nelson; Molina, Rafael; Moreno, Sonia</p> <p>2012-01-01</p> <p>The effluent from the anaerobic biological treatment of coffee wet processing wastewater (CWPW) contains a non-biodegradable compound that must be treated before it is discharged into a water source. In this paper, the wet hydrogen peroxide catalytic oxidation (WHPCO) process using <span class="hlt">Al</span>-Ce-Fe-PILC catalysts was researched as a post-treatment <span class="hlt">system</span> for CWPW and tested in a semi-batch reactor at atmospheric pressure and 25 °C. The <span class="hlt">Al</span>-Ce-Fe-PILC achieved a high conversion rate of total phenolic compounds (70%) and mineralization to CO(2) (50%) after 5 h reaction time. The chemical oxygen demand (COD) of coffee processing wastewater after wet hydrogen peroxide catalytic oxidation was reduced in 66%. The combination of the two treatment methods, biological (developed by Cenicafé) and catalytic oxidation with <span class="hlt">Al</span>-Ce-Fe-PILC, achieved a 97% reduction of COD in CWPW. Therefore, the WHPCO using <span class="hlt">Al</span>-Ce-Fe-PILC catalysts is a viable alternative for the post-treatment of coffee processing wastewater.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2015JNuM..457...72O','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2015JNuM..457...72O"><span id="translatedtitle">Phase equilibria investigations and thermodynamic modeling of the <span class="hlt">system</span> Bi2O3-<span class="hlt">Al</span>2O3</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Oudich, F.; David, N.; Mathieu, S.; Vilasi, M.</p> <p>2015-02-01</p> <p>The <span class="hlt">system</span> Bi2O3-<span class="hlt">Al</span>2O3 has been experimentally investigated above 600 °C by DTA, XRD and EPMA under air and low oxygen pressure. Only two compounds were found to exist in equilibrium, which are Bi2<span class="hlt">Al</span>4O9(1Bi2O3:2<span class="hlt">Al</span>2O3) and Bi25<span class="hlt">Al</span>O39(25:1). The latter exhibits a sillenite structure and does not contain pentavalent bismuth. A peritectoid decomposition of (25:1) and a peritectic melting of (1:2) occur at 775 °C and 1075 °C respectively, while an eutectic transformation was observed at 815 °C for 97 mol% Bi2O3. On the basis of the results obtained within the present work as well as experimental data provided from literature, a thermodynamic modeling where the liquid phase is described by the two-sublattice ionic liquid model was performed according to the Calphad approach. The resulting thermodynamic optimization yielded good agreement with experimental results in the investigated region.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/22345239','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/22345239"><span id="translatedtitle">Phase and structural behavior of Sm<span class="hlt">Al</span>O{sub 3}–RAlO{sub 3} (R = Eu, Gd) <span class="hlt">systems</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Ohon, N.; Vasylechko, L.; Prots, Yu.; Schmidt, M.</p> <p>2014-02-01</p> <p>Highlights: • Continuous solid solutions exist in the Sm<span class="hlt">Al</span>O{sub 3}–RAlO{sub 3} (R = Eu, Gd) <span class="hlt">systems</span>. • Lattice parameter crossover was found in solid solutions Sm{sub 1−x}R{sub x}<span class="hlt">Al</span>O{sub 3} (R = Eu, Gd). • Thermally induced lattice crossovers occur in Sm{sub 0.9}R{sub 0.1}<span class="hlt">Al</span>O{sub 3} at elevated temperatures. • First-order structural phase transition Pbnm↔R3{sup ¯}c was found in Sm{sub 1−x}R{sub x}<span class="hlt">Al</span>O{sub 3} (R = Eu, Gd). • Phase diagram of the <span class="hlt">systems</span> Sm<span class="hlt">Al</span>O{sub 3}–Eu<span class="hlt">Al</span>O{sub 3} and Sm<span class="hlt">Al</span>O{sub 3}–Gd<span class="hlt">Al</span>O{sub 3} has been constructed. - Abstract: Phase and structural behavior in the Sm<span class="hlt">Al</span>O{sub 3}–RAlO{sub 3} (R = Eu, Gd) <span class="hlt">systems</span> has been studied in a whole concentration range by means of laboratory X-ray diffraction, in situ synchrotron powder diffraction and differential thermal analysis techniques. Continuous solid solutions with orthorhombic perovskite structure have been found in both <span class="hlt">systems</span>. Peculiarity of the solid solutions of Sm{sub 1−x}Eu{sub x}<span class="hlt">Al</span>O{sub 3} and Sm{sub 1−x}Gd{sub x}<span class="hlt">Al</span>O{sub 3} is the existence of two lattice parameter crossovers in each <span class="hlt">system</span> occurred at x{sub Eu} = 0.07 and 0.62 and at x{sub Gd} = 0.04 and 0.33, respectively. The temperature induced lattice crossovers in the Sm{sub 0.9}Eu{sub 0.1}<span class="hlt">Al</span>O{sub 3} and Sm{sub 0.9}Gd{sub 0.1}<span class="hlt">Al</span>O{sub 3} samples have been found at 387 and 922 K and at 501 and 894 K. First-order reversible structural phase transformations Pbnm↔R3{sup ¯}c have been detected in both <span class="hlt">systems</span> at the elevated temperatures. The temperatures of these transitions increase linearly with the decreasing of the samarium content. Phase diagrams of the pseudo-binary <span class="hlt">systems</span> Sm<span class="hlt">Al</span>O{sub 3}–Eu<span class="hlt">Al</span>O{sub 3} and Sm<span class="hlt">Al</span>O{sub 3}–Gd<span class="hlt">Al</span>O{sub 3} have been constructed.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/7209323','DOE-PATENT-XML'); return false;" href="http://www.osti.gov/scitech/biblio/7209323"><span id="translatedtitle">High-transition-temperature superconductors in the Nb-<span class="hlt">Al</span>-Ge <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/doepatents">DOEpatents</a></p> <p>Giorgi, A.L.; Szklarz, E.G.</p> <p>1972-09-26</p> <p>The patent describes superconducting materials of the nominal composition Nb(x)<span class="hlt">Al</span>(y)Ge(l-y), where x is in the range of 1.9 to 2.8 and y is in the range of 0.5 to 0.9, having transition temperatures in the 19 -20K. range which are readily produced by annealing arc-melted compositions, or cold-pressed, heat-treated compositions at moderate temperatures for reasonably long times (about 50 hours).</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016PhRvB..94i4111G','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016PhRvB..94i4111G"><span id="translatedtitle">Phase and structural stability in Ni-<span class="hlt">Al</span> <span class="hlt">systems</span> from first principles</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Goiri, Jon Gabriel; Van der Ven, Anton</p> <p>2016-09-01</p> <p>We report on a comprehensive first-principles study of phase stability in the Ni-<span class="hlt">Al</span> binary, both at zero Kelvin and at finite temperature. First-principles density functional theory calculations of the energies of enumerated orderings on fcc and the sublattices of B2 not only predict the stability of known phases, but also reveal the stability of a family of ordered phases that combine features of L 12 and L 10 in different ratios to adjust their overall composition. The calculations also confirm the stability of vacancy ordered B2 derivatives that are stable in the <span class="hlt">Al</span>-rich half of the phase diagram. We introduce strain order parameters to systematically analyze instabilities with respect to the Bain path connecting the fcc and bcc lattices. Many unstable orderings on both fcc and bcc are predicted around compositions of xNi=0.625 , where a martensitic phase transformation is known to occur. Cluster expansion techniques together with Monte Carlo simulations were used to calculate a finite-temperature-composition phase diagram of the Ni-<span class="hlt">Al</span> binary. The calculated phase diagram together with an analysis of Bain instabilities reveals the importance of anharmonicity in determining the phase bounds between the B2 based β phase and the L 12 based γ' phase, as well as properties related to martensitic transformations that are observed upon quenching Ni-rich β .</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/1999PhDT.......256M','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/1999PhDT.......256M"><span id="translatedtitle">Phase equilibria and transformations in the Ti-<span class="hlt">Al</span>-Nb <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Mishurda, Joseph Constantine</p> <p></p> <p>The phase equilibria and transformations in the Nb-Ti-<span class="hlt">Al</span> <span class="hlt">system</span> in the vicinity of the Sigma phase field have been examined with respect to the Liquidus Surface (Phase 1), the Phase Equilibria and Equilibrium Transformations (Phase II), and the Phase Transformations, Mechanisms and Kinetics (Phase III). Eight alloy compositions were produced by arc melting. The alloys were characterized by differential thermal analysis, metallography, X-ray diffraction, scanning electron microscopy (BSEI), electron probe microanalysis and transmission electron microscopy. The liquidus examination shed new light on previous microstructural interpretations, opening up new possibilities for microstructural development and control of multiphase alloys. Differential thermal analysis has identified the existence of a beta to sigma + gamma transformation in an alloy where it was not previously thought to exist. The results differed from the calculated diagram by higher titanium solubility in the sigma and delta phases than predicted at lower temperatures and a lower solubility of alpha2 and gamma. The high temperature betao transforms to gamma + sigma in a eutectoid fashion resulting in a desirable lamellar structure of sigma and gamma. The existence of a new body centered tetragonal crystal structure ao = 5.11A and co 28.12A with a point group symmetry of P4/mmm, at 700°C was discovered. A plethel section was found for the sigma + beta two phase alloys. A betao + O + sigma three phase field passes through the alloys between 981 and 1000°C. The plethel section at the transformation has an eutectoid characteristic, however, the nature of the transformation changes to a peritectoid. At temperatures below 970°C, the first transformation to occur is the decomposition of the metastable betao phase to an intermediate metastable phase O'. Reasonable values for Q were obtained, applicable to the diffusion limited region of the TTT-curve. The microstructure evolution of the sigma + beta</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/servlets/purl/114567','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/servlets/purl/114567"><span id="translatedtitle">Analysis and design modifications for upgrade of storage ring bump pulse <span class="hlt">system</span> driving the injection bump magnets at the <span class="hlt">ALS</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Stover, G.D.</p> <p>1995-04-01</p> <p>A fast (4.0 ms half period) resonant discharge pulse <span class="hlt">system</span>, using SCRs, was designed and constructed to drive the injection bump magnet <span class="hlt">system</span> at the Advanced Light Source (<span class="hlt">ALS</span>). The commissioning process revealed a high frequency resonance (T = 800 NS) superimposed on the driver discharge wave form. In addition, the peak amplitude of the magnet load recovery current exceeded design specifications. A SPICE analysis confirmed the suspected mechanisms for the parasitic ringing and the excessive load current {open_quotes}undershoot{close_quotes}. This paper will address the subsequent analysis, measurements, and modifications carried out during the maintenance shutdown in June 1993.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/cgi-bin/nph-data_query?bibcode=2012PhLB..710..426P&link_type=ABSTRACT','NASAADS'); return false;" href="http://adsabs.harvard.edu/cgi-bin/nph-data_query?bibcode=2012PhLB..710..426P&link_type=ABSTRACT"><span id="translatedtitle">Nuclear rainbow in the 16O + 27<span class="hlt">AL</span> <span class="hlt">system</span>: The role of couplings at energies far above the barrier</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Pereira, D.; Linares, R.; Oliveira, J. R. B.; Lubian, J.; Chamon, L. C.; Gomes, P. R. S.; Cunsolo, A.; Cappuzzello, F.; Cavallaro, M.; Carbone, D.; Foti, A.</p> <p>2012-04-01</p> <p>High precision elastic and inelastic angular distributions have been measured for the 16O + 27<span class="hlt">Al</span> <span class="hlt">system</span> at a beam energy of 100 MeV. The data analysis confirms a rainbow formation as already predicted by parameter-free Coupled Channel calculations. It also helps to reveal the crucial role of inelastic couplings in the rainbow formation for heavier <span class="hlt">systems</span> even at energies far above the Coulomb barrier. This feature, well known in atomic/molecular scattering, is experimentally studied for the first time in Nuclear Physics.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2015AIPA....5h7149A','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2015AIPA....5h7149A"><span id="translatedtitle">Electrical properties of GaAs metal-oxide-semiconductor structure comprising <span class="hlt">Al</span>2O3 gate oxide and <span class="hlt">Al</span>N passivation layer fabricated in situ using a metal-organic vapor deposition/atomic layer deposition hybrid <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Aoki, Takeshi; Fukuhara, Noboru; Osada, Takenori; Sazawa, Hiroyuki; Hata, Masahiko; Inoue, Takayuki</p> <p>2015-08-01</p> <p>This paper presents a compressive study on the fabrication and optimization of GaAs metal-oxide-semiconductor (MOS) structures comprising a <span class="hlt">Al</span>2O3 gate oxide, deposited via atomic layer deposition (ALD), with an <span class="hlt">Al</span>N interfacial passivation layer prepared in situ via metal-organic chemical vapor deposition (MOCVD). The established protocol afforded self-limiting growth of <span class="hlt">Al</span>2O3 in the atmospheric MOCVD reactor. Consequently, this enabled successive growth of MOCVD-formed <span class="hlt">Al</span>N and ALD-formed <span class="hlt">Al</span>2O3 layers on the GaAs substrate. The effects of <span class="hlt">Al</span>N thickness, post-deposition anneal (PDA) conditions, and crystal orientation of the GaAs substrate on the electrical properties of the resulting MOS capacitors were investigated. Thin <span class="hlt">Al</span>N passivation layers afforded incorporation of optimum amounts of nitrogen, leading to good capacitance-voltage (C-V) characteristics with reduced frequency dispersion. In contrast, excessively thick <span class="hlt">Al</span>N passivation layers degraded the interface, thereby increasing the interfacial density of states (Dit) near the midgap and reducing the conduction band offset. To further improve the interface with the thin <span class="hlt">Al</span>N passivation layers, the PDA conditions were optimized. Using wet nitrogen at 600 °C was effective to reduce Dit to below 2 × 1012 cm-2 eV-1. Using a (111)A substrate was also effective in reducing the frequency dispersion of accumulation capacitance, thus suggesting the suppression of traps in GaAs located near the dielectric/GaAs interface. The current findings suggest that using an atmosphere ALD process with in situ <span class="hlt">Al</span>N passivation using the current MOCVD <span class="hlt">system</span> could be an efficient solution to improving GaAs MOS interfaces.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2015PMM...116..544D','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2015PMM...116..544D"><span id="translatedtitle">Estimation of excess energies and activity coefficients for the penternary Ni-Cr-Co-<span class="hlt">Al</span>-Mo <span class="hlt">system</span> and its subsystems</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Dogan, A.; Arslan, H.; Dogan, T.</p> <p>2015-06-01</p> <p>Using different prediction methods, such as the General Solution Model of Kohler and Muggianu, the excess energy and activities of molybdenum for the sections of the phase diagram for the penternary Ni-Cr-Co-<span class="hlt">Al</span>-Mo <span class="hlt">system</span> with mole ratios xNi/ xMo = 1, xCr/ xMo = 1, xCo/ xMo = 1, and x<span class="hlt">Al</span>/ xMo = r = 0.5 and 1, were thermodynamically investigated at a temperature of 2000 K, whereas the excess energy and activities of Bi for the section corresponding to the ternary Bi-Ga-Sb <span class="hlt">system</span> with mole ratio xGa/ xSb = 1/9 were thermodynamically investigated at a temperature of 1073 K. In the case of r = 0.5 and 1 in the alloys Ni-Cr-Co-<span class="hlt">Al</span>-Mo, a positive deviation in the activity coefficient was revealed, as molybdenum content increased. Moreover, in the calculations performed in Chou's GSM model, the obtained values for excess Gibbs energies are negative in the whole concentration range of bismuth at 1073 K and exhibit the minimum of about -2.2 kJ/mol at the mole ratio xGa/ xSb = 1/9 in the alloy Bi-Ga-Sb.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2015JTST...24..953E','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2015JTST...24..953E"><span id="translatedtitle">Corrosion of NiCoCr<span class="hlt">Al</span>Y Coatings and TBC <span class="hlt">Systems</span> Subjected to Water Vapor and Sodium Sulfate</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Eriksson, Robert; Yuan, Kang; Li, Xin-Hai; Lin Peng, Ru</p> <p>2015-08-01</p> <p>Thermal barrier coating (TBC) <span class="hlt">systems</span> are commonly used in gas turbines for protection against high-temperature degradation. Penetration of the ceramic top coat by corrosive species may cause corrosion damage on the underlying NiCoCr<span class="hlt">Al</span>Y bond coat and cause failure of the TBC <span class="hlt">system</span>. In the current study, four oxidation/corrosion conditions were tried: (i) lab air, (ii) water vapor, (iii) sodium sulfate deposited on the specimens, and (iv) water vapor + sodium sulfate. The test was done at 750 °C in a cyclic test rig with 48 h cycles. The corrosion damage was studied on NiCoCr<span class="hlt">Al</span>Y-coated specimens, thin APS TBC specimens, and thick APS TBC specimens. Water vapor was found to have very minor influence on the oxidation, while sodium sulfate increased the TGO thickness both for NiCoCr<span class="hlt">Al</span>Y specimens and TBC-coated specimens; the influence of the TBC thickness was found to be very small. Sodium sulfate promoted thicker TGO; more Cr-rich TGO; the formation of Y oxides, and internally, Y sulfides; pore formation in the coating as well as in the substrate; and the formation of a Cr-depleted zone in the substrate.</p> </li> </ol> <div class="pull-right"> <ul class="pagination"> <li><a href="#" onclick='return showDiv("page_1");'>«</a></li> <li><a href="#" onclick='return showDiv("page_20");'>20</a></li> <li><a href="#" onclick='return showDiv("page_21");'>21</a></li> <li class="active"><span>22</span></li> <li><a href="#" onclick='return showDiv("page_23");'>23</a></li> <li><a href="#" onclick='return showDiv("page_24");'>24</a></li> <li><a href="#" onclick='return showDiv("page_25");'>»</a></li> </ul> </div> </div><!-- col-sm-12 --> </div><!-- row --> </div><!-- page_22 --> <div id="page_23" class="hiddenDiv"> <div class="row"> <div class="col-sm-12"> <div class="pull-right"> <ul class="pagination"> <li><a href="#" onclick='return showDiv("page_1");'>«</a></li> <li><a href="#" onclick='return showDiv("page_21");'>21</a></li> <li><a href="#" onclick='return showDiv("page_22");'>22</a></li> <li class="active"><span>23</span></li> <li><a href="#" onclick='return showDiv("page_24");'>24</a></li> <li><a href="#" onclick='return showDiv("page_25");'>25</a></li> <li><a href="#" onclick='return showDiv("page_25");'>»</a></li> </ul> </div> </div> </div> <div class="row"> <div class="col-sm-12"> <ol class="result-class" start="441"> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/1003913','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/1003913"><span id="translatedtitle">Cyclotron resonance at microwave frequencies in two-dimensional hole <span class="hlt">system</span> in <span class="hlt">Al</span>GaAs/GaAs quantum wells.</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Ong, N. P.; Bayrakci, S. P.; Lai, K. W.; Pan, Wei; Tsui, Daniel Chee; West, Ken W.; Pfeiffer, Loren N.</p> <p>2003-07-01</p> <p>Cyclotron resonance at the microwave frequency is used to measure the band edge mass (m{sub b}) in the two-dimensional hole (2DH) <span class="hlt">system</span>, confined in 30 nm quantum wells in the <span class="hlt">Al</span>{sub 0.1}Ga{sub 0.9}As/GaAs/<span class="hlt">Al</span>{sub 0.1}Ga{sub 0.9}As heterostructures. We find that for 2DH density p {le} 1.0 x 10{sup 10} cm{sup -2}, m{sub b} is nearly constant, {approx}0.35m{sub e}. It increases with increasing density, to {approx}0.5m{sub e} at p = 7.4 x 10{sup 10} cm{sup -2}.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2011SSSci..13.2124W','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2011SSSci..13.2124W"><span id="translatedtitle">Hydrothermal transformation of magadiite into ferrierite in <span class="hlt">Al</span> 2O 3-Na 2O-ethylenediamine-H 2O <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Wang, Yu; Yang, Yang; Cui, Miao; Sun, Jiangbo; Qi, Lin; Ji, Shouhua; Meng, Changgong</p> <p>2011-12-01</p> <p>This study investigated the transformation of magadiite into ferrierite in <span class="hlt">Al</span> 2O 3-Na 2O-ethylenediamine (EDA)-H 2O <span class="hlt">system</span>. The influence of various parameters such as reaction temperature, time, alkalinity, the reactant Na 2O/SiO 2 ratio and EDA/SiO 2 ratio were examined. Thermal and acid stability of the synthetic ferrierite are presented. Highly crystallized and pure ferrierite could be obtained from dispersion with the molar composition: 0.01 Na 2O: 0.005 <span class="hlt">Al</span> 2O 3: SiO 2: 30 H 2O: 20 EDA by heating at 433 K for 48 h. The structure of ferrierite was destroyed when the temperature rose above 873 K and the framework of the sample, stirred in 5 mol/L HCl for 3 h, is consistent with the untreated ferrierite.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/22273493','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/22273493"><span id="translatedtitle">Morphology and properties of a hybrid organic-inorganic <span class="hlt">system</span>: <span class="hlt">Al</span> nanoparticles embedded into CuPc thin film</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Molodtsova, O. V.; Babenkov, S. V.; Aristova, I. M.; Vilkov, O. V.; Aristov, V. Yu.</p> <p>2014-04-28</p> <p>The evolution of the morphology and the electronic structure of the hybrid organic-inorganic <span class="hlt">system</span> composed of aluminum nanoparticles (NPs) distributed in an organic semiconductor matrix—copper phthalocyanine (CuPc)—as a function of nominal aluminum content was studied by transmission electron microscopy and by photoemission spectroscopy methods. The aluminum atoms deposited onto the CuPc surface diffuse into the organic matrix and self-assemble to NPs in a well-defined manner with a narrow diameter distribution, which depends on the amount of aluminum that is evaporated onto the CuPc film. We find clear evidence of a charge transfer from <span class="hlt">Al</span> to CuPc and we have been able to determine the lattice sites where <span class="hlt">Al</span> ions sit. The finally at high coverage about 64 Å the formation of metallic aluminum overlayer on CuPc thin film takes place.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/cgi-bin/nph-data_query?bibcode=2010JNuM..397....1S&link_type=ABSTRACT','NASAADS'); return false;" href="http://adsabs.harvard.edu/cgi-bin/nph-data_query?bibcode=2010JNuM..397....1S&link_type=ABSTRACT"><span id="translatedtitle">Ab-initio calculations and phase diagram assessments of An-<span class="hlt">Al</span> <span class="hlt">systems</span> (An = U, Np, Pu)</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Sedmidubský, D.; Konings, R. J. M.; Souček, P.</p> <p>2010-02-01</p> <p>The enthalpies of formation of binary intermetallic compounds An<span class="hlt">Al</span> n(n=2,3,4, An=U,Np,Pu) were assessed from first principle calculations of total energies performed using full potential APW + lo technique within density functional theory ( WIEN2k). The substantial contribution to entropies, S298°, arising from lattice vibrations was calculated by direct method within harmonic crystal approximation ( Phonon software + VASP for obtaining Hellmann-Feynman forces). The electronic heat capacity and the corresponding contribution to entropy were estimated from the density of states at Fermi level obtained from electronic structure calculations. The phase diagrams of the relevant <span class="hlt">systems</span> An-<span class="hlt">Al</span> were calculated based on the thermodynamic data assessed from ab-initio calculations, known equilibrium and calorimetry data by employing the FactSage program.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/servlets/purl/532645','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/servlets/purl/532645"><span id="translatedtitle">VXI based multibunch detector and QPSK modulator for the PEP-II/<span class="hlt">ALS</span>/DA{Phi}NE longitudinal feedback <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Young, A.; Fox, J.; Teytelman, D.</p> <p>1997-04-01</p> <p>The PEP-II/<span class="hlt">ALS</span>/DA{Phi}NE feedback <span class="hlt">systems</span> are complex <span class="hlt">systems</span> implemented using analog, digital and microwave circuits. The VXI hardware implementation for the Front-end and Back-end analog processing modules is presented. The Front-end module produces a baseband beam phase signal from pickups using a microwave tone burst generator. The Back-end VXI module generates an AM/QPSK modulated signal from a baseband correction signal computed in a digital signal processor. These components are implemented in VXI packages that allow a wide spectrum of <span class="hlt">system</span> functions including a 120 MHz bandwidth rms detector, reference phase servo, woofer link to the RF control <span class="hlt">system</span>, standard VXI status/control, and user defined registers. The details of the design and implementation of the VXI modules including performance characteristics are presented.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/24010694','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/24010694"><span id="translatedtitle">[Review of evidence of thalidomide and lenalidomide in different hematological diseases: chronic lymphocytic leukemia, primary <span class="hlt">amyloidosis</span>, myelofibrosis and syndrome myelodysplastic].</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Jiménez Lozano, I; Juárez Jiménez, J C</p> <p>2013-01-01</p> <p>Lenalidomide is an immunomodulatory drug approved by the AEMPS and the EMA, in combination with dexamethasone, for the treatment of multiple myeloma in adult patients who have received at least one prior therapy. Moreover, it has recently been approved for the treatment of patients with transfusion-dependent anaemia due to low- or intermediate-1-risk myelodysplastic syndromes associated with an isolated deletion 5q cytogenetic abnormality when other therapeutic options are insufficient or inadequate. It has also shown to be active in other hematologic and no hematologic diseases. Growing evidence of its use entails a challenge when situating the drug in a cost-effective way to treat these diseases. On this article we review the available evidence on the use of lenalidomide in the second line treatment of patients with chronic lymphocytic leukemia, primary <span class="hlt">amyloidosis</span> and primary myelofibrosis, and in the first line treatment of patients with myelodysplastic syndrome, and also the evidence of other immunomodulators. Different clinical practice guidelines and scientific evidence portals consider lenalidomide a valid alternative in the first-line treatment of patients with myelodysplastic syndromes, specially those with the deletion of 5q, and in second line for patients with chronic lymphocytic leukemia. However, the available evidence of lenalidomide in the treatment of patients with primary <span class="hlt">amyloidosis</span> and primary myelofibrosis is limited, ant thus is not considered as the first choice treatment. In any case, the treatment of choice should consider the safety profile in each patient, the previous treatments that has received and the own therapeutic protocols of each center.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4225459','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4225459"><span id="translatedtitle">Physician user satisfaction with an electronic medical records <span class="hlt">system</span> in primary healthcare centres in <span class="hlt">Al</span> Ain: a qualitative study</span></a></p> <p><a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pmc">PubMed Central</a></p> <p>Al Alawi, Shamma; Al Dhaheri, Aysha; Al Baloushi, Durra; Al Dhaheri, Mouza; Prinsloo, Engela A M</p> <p>2014-01-01</p> <p>Objectives To explore physician satisfaction with an electronic medical records (EMR) <span class="hlt">system</span>, to identify and explore the main limitations of the <span class="hlt">system</span> and finally to submit recommendations to address these limitations. Design A descriptive qualitative study that entailed three focus group interviews was performed among physicians using open-ended questions. The interviews were audiotaped, documented and transcribed verbatim. The themes were explored and analysed in different categories. Setting The study was conducted in primary healthcare centres (PHC) in <span class="hlt">Al</span> Ain, United Arab Emirates (UAE). Participants A total of 23 physicians, all using the same EMR <span class="hlt">system</span>, attended one of three focus groups held in PHC in <span class="hlt">Al</span> Ain Medical District. Each focus group consisted of 7–9 physicians working in PHC as family medicine specialists, residents or general practitioners. Primary outcome measure Physician satisfaction with the EMR <span class="hlt">system</span>. Results Key themes emerged and were categorised as physician-dependent, patient-related and <span class="hlt">system</span>-related factors. In general, physicians were satisfied with the EMR <span class="hlt">system</span> in spite of initial difficulties with implementation. Most participants identified that the long time required to do the documentation affected their practice and patient communication. Many physicians expressed satisfaction with the orders and results of laboratory and radiology functions and they emphasised that this was the strongest point in the EMR. They were also satisfied with the electronic prescription function, stating that it reduced errors and saved time. Conclusions Physicians are satisfied with the EMR and have a positive perception regarding the application of the <span class="hlt">system</span>. Several themes emerged during this study that need to be considered to enhance the EMR <span class="hlt">system</span>. Further studies need to be conducted among other healthcare practitioners and patients to explore their attitude and perception about the EMR. PMID:25377010</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.alsa.org/','NIH-MEDLINEPLUS'); return false;" href="http://www.alsa.org/"><span id="translatedtitle"><span class="hlt">ALS</span> Association</span></a></p> <p><a target="_blank" href="http://medlineplus.gov/">MedlinePlus</a></p> <p></p> <p></p> <p>... toward a world without <span class="hlt">ALS</span>! Walk to Defeat ALS® Walk to Defeat ALS® draws people of all ... We need your help. I Will Advocate National <span class="hlt">ALS</span> Registry The National <span class="hlt">ALS</span> Registry is a congressionally ...</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016JMiMi..26h4007B','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016JMiMi..26h4007B"><span id="translatedtitle">Characterization of a smartphone size haptic rendering <span class="hlt">system</span> based on thin-film <span class="hlt">Al</span>N actuators on glass substrates</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Bernard, F.; Casset, F.; Danel, J. S.; Chappaz, C.; Basrour, S.</p> <p>2016-08-01</p> <p>This paper presents for the first time the characterization of a smartphone-size haptic rendering <span class="hlt">system</span> based on the friction modulation effect. According to previous work and finite element modeling, the homogeneous flexural modes are needed to get the haptic feedback effect. The device studied consists of a thin film <span class="hlt">Al</span>N transducers deposited on an 110  ×  65 mm2 glass substrate. The transducer’s localization on the glass plate allows a transparent central area of 90  ×  49 mm2. Electrical and mechanical parameters of the <span class="hlt">system</span> are extracted from measurement. From this extraction, the electrical impedance matching reduced the applied voltage to 17.5 V AC and the power consumption to 1.53 W at the resonance frequency of the vibrating <span class="hlt">system</span> to reach the haptic rendering specification. Transient characterizations of the actuation highlight a delay under the dynamic tactile detection. The characterization of the <span class="hlt">Al</span>N transducers used as sensors, including the noise rejection, the delay or the output charge amplitude allows detections with high accuracy of any variation due to external influences. Those specifications are the first step to a low-power-consumption feedback-looped <span class="hlt">system</span>.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.ncbi.nlm.nih.gov/pubmed/23671077','PUBMED'); return false;" href="http://www.ncbi.nlm.nih.gov/pubmed/23671077"><span id="translatedtitle">182Hf-182W age dating of a 26<span class="hlt">Al</span>-poor inclusion and implications for the origin of short-lived radioisotopes in the early Solar <span class="hlt">System</span>.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Holst, Jesper C; Olsen, Mia B; Paton, Chad; Nagashima, Kazuhide; Schiller, Martin; Wielandt, Daniel; Larsen, Kirsten K; Connelly, James N; Jørgensen, Jes K; Krot, Alexander N; Nordlund, Ake; Bizzarro, Martin</p> <p>2013-05-28</p> <p>Refractory inclusions [calcium-aluminum-rich inclusions, (CAIs)] represent the oldest Solar <span class="hlt">System</span> solids and provide information regarding the formation of the Sun and its protoplanetary disk. CAIs contain evidence of now extinct short-lived radioisotopes (e.g., (26)<span class="hlt">Al</span>, (41)Ca, and (182)Hf) synthesized in one or multiple stars and added to the protosolar molecular cloud before or during its collapse. Understanding how and when short-lived radioisotopes were added to the Solar <span class="hlt">System</span> is necessary to assess their validity as chronometers and constrain the birthplace of the Sun. Whereas most CAIs formed with the canonical abundance of (26)<span class="hlt">Al</span> corresponding to (26)<span class="hlt">Al</span>/(27)<span class="hlt">Al</span> of ∼5 × 10(-5), rare CAIs with fractionation and unidentified nuclear isotope effects (FUN CAIs) record nucleosynthetic isotopic heterogeneity and (26)<span class="hlt">Al</span>/(27)<span class="hlt">Al</span> of <5 × 10(-6), possibly reflecting their formation before canonical CAIs. Thus, FUN CAIs may provide a unique window into the earliest Solar <span class="hlt">System</span>, including the origin of short-lived radioisotopes. However, their chronology is unknown. Using the (182)Hf-(182)W chronometer, we show that a FUN CAI recording a condensation origin from a solar gas formed coevally with canonical CAIs, but with (26)<span class="hlt">Al</span>/(27)<span class="hlt">Al</span> of ∼3 × 10(-6). The decoupling between (182)Hf and (26)<span class="hlt">Al</span> requires distinct stellar origins: steady-state galactic stellar nucleosynthesis for (182)Hf and late-stage contamination of the protosolar molecular cloud by a massive star(s) for (26)<span class="hlt">Al</span>. Admixing of stellar-derived (26)<span class="hlt">Al</span> to the protoplanetary disk occurred during the epoch of CAI formation and, therefore, the (26)<span class="hlt">Al</span>-(26)Mg systematics of CAIs cannot be used to define their formation interval. In contrast, our results support (182)Hf homogeneity and chronological significance of the (182)Hf-(182)W clock. PMID:23671077</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/23671077','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/23671077"><span id="translatedtitle">182Hf-182W age dating of a 26<span class="hlt">Al</span>-poor inclusion and implications for the origin of short-lived radioisotopes in the early Solar <span class="hlt">System</span>.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Holst, Jesper C; Olsen, Mia B; Paton, Chad; Nagashima, Kazuhide; Schiller, Martin; Wielandt, Daniel; Larsen, Kirsten K; Connelly, James N; Jørgensen, Jes K; Krot, Alexander N; Nordlund, Ake; Bizzarro, Martin</p> <p>2013-05-28</p> <p>Refractory inclusions [calcium-aluminum-rich inclusions, (CAIs)] represent the oldest Solar <span class="hlt">System</span> solids and provide information regarding the formation of the Sun and its protoplanetary disk. CAIs contain evidence of now extinct short-lived radioisotopes (e.g., (26)<span class="hlt">Al</span>, (41)Ca, and (182)Hf) synthesized in one or multiple stars and added to the protosolar molecular cloud before or during its collapse. Understanding how and when short-lived radioisotopes were added to the Solar <span class="hlt">System</span> is necessary to assess their validity as chronometers and constrain the birthplace of the Sun. Whereas most CAIs formed with the canonical abundance of (26)<span class="hlt">Al</span> corresponding to (26)<span class="hlt">Al</span>/(27)<span class="hlt">Al</span> of ∼5 × 10(-5), rare CAIs with fractionation and unidentified nuclear isotope effects (FUN CAIs) record nucleosynthetic isotopic heterogeneity and (26)<span class="hlt">Al</span>/(27)<span class="hlt">Al</span> of <5 × 10(-6), possibly reflecting their formation before canonical CAIs. Thus, FUN CAIs may provide a unique window into the earliest Solar <span class="hlt">System</span>, including the origin of short-lived radioisotopes. However, their chronology is unknown. Using the (182)Hf-(182)W chronometer, we show that a FUN CAI recording a condensation origin from a solar gas formed coevally with canonical CAIs, but with (26)<span class="hlt">Al</span>/(27)<span class="hlt">Al</span> of ∼3 × 10(-6). The decoupling between (182)Hf and (26)<span class="hlt">Al</span> requires distinct stellar origins: steady-state galactic stellar nucleosynthesis for (182)Hf and late-stage contamination of the protosolar molecular cloud by a massive star(s) for (26)<span class="hlt">Al</span>. Admixing of stellar-derived (26)<span class="hlt">Al</span> to the protoplanetary disk occurred during the epoch of CAI formation and, therefore, the (26)<span class="hlt">Al</span>-(26)Mg systematics of CAIs cannot be used to define their formation interval. In contrast, our results support (182)Hf homogeneity and chronological significance of the (182)Hf-(182)W clock.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/24041987','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/24041987"><span id="translatedtitle">Regulation of <span class="hlt">system</span> x(c)- in the SOD1-G93A mouse model of <span class="hlt">ALS</span>.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Albano, Rebecca; Liu, XiaoQian; Lobner, Doug</p> <p>2013-12-01</p> <p>The cystine/glutamate antiporter (<span class="hlt">system</span> xc-) is critical for the generation of the antioxidant glutathione by transporting cystine into the cell. At the same time, <span class="hlt">system</span> xc- also releases glutamate, which can potentially lead to excitotoxicity. The dual actions of <span class="hlt">system</span> xc- make it of great interest in any disease, like amyotrophic lateral sclerosis (<span class="hlt">ALS</span>), in which there is evidence of the involvement of both oxidative stress and excitotoxicity. The present study investigated the regulation of <span class="hlt">system</span> xc- in the spinal cord of the SOD1-G93A transgenic mouse model of <span class="hlt">ALS</span>. In acute spinal cord slices of 70day old SOD1-G93A transgenic mice cystine uptake by <span class="hlt">system</span> xc- was significantly increased compared to age matched non-transgenic mice; but it was not significantly different at 55, 100, or 130days. The 70day old SOD1-G93A transgenic mice also showed significantly increased glutamate release in the presence of cystine. d-Aspartate uptake through excitatory amino acid transporters (EAATs), the main mechanism by which glutamate is cleared from the extracellular space, was also examined. In spinal cord slices of 70day old SOD1-G93A mice no change in d-aspartate uptake was found. Together, these findings suggest that at 70days of age, SOD1-G93A transgenic mice have increased <span class="hlt">system</span> xc- activity, but no change in EAAT function. These results raise the possibility that excitotoxicity in the SOD1-G93A transgenic mouse, at least at early time points, may be due to increased <span class="hlt">system</span> xc- activity and not decreased EAAT function.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/1996PhRvB..5416555M','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/1996PhRvB..5416555M"><span id="translatedtitle">Electronic structure of normal, inverse, and partially inverse spinels in the Mg<span class="hlt">Al</span>2O4 <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Mo, Shang-Di; Ching, W. Y.</p> <p>1996-12-01</p> <p>The electronic structure of normal, inverse, and partially inverse spinels in the Mg<span class="hlt">Al</span>2O4 <span class="hlt">system</span> are studied by means of first-principles calculations. For the normal spinel, the calculated ground-state properties are in good agreement with experimental data. A local-density-approximation band gap of 5.80 eV is obtained. For the inverse and partially inverse spinels, in which up to eight Mg atoms in a tetrahedral coordination are interchanged with eight of the 16 <span class="hlt">Al</span> atoms in octahedral coordination, the atomic positions are relaxed by realistic interatomic pair potentials. Based on the relaxed models, the electronic structure and their dependence on the inversion parameter λ are studied. The total lattice energy increases as λ increases with a change of slope at λ=4/16. It is found that the general features in the density of states (DOS) in these spinels are quite similar with subtle differences in the peak structures between normal and inverse spinels. The smallest band gap of 4.84 eV is found at λ=4/16. The orbital decomposition of the partial DOS of <span class="hlt">Al</span> and Mg in different coordination environments is fully analyzed. These results are discussed in the context of an order-disorder phenomenon associated with a cation site interchange, and their implications on spectroscopic detections.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2012LPI....43.2255K','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2012LPI....43.2255K"><span id="translatedtitle">Heterogeneous Distribution of ^2^6<span class="hlt">Al</span> at the Birth of the Solar <span class="hlt">System</span>: Evidence from Corundum-Bearing Refractory Inclusions</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Krot, A. N.; Makide, K.; Nagashima, K.; Huss, G. R.; Hellebrand, E.; Petaev, M. I.</p> <p>2012-03-01</p> <p>Corundum-bearing CAIs recorded heterogeneous distribution of ^2^6<span class="hlt">Al</span> at the birth of the solar <span class="hlt">system</span>. We suggest that ^2^6<span class="hlt">Al</span> was injected into the protosolar molecular cloud core by a wind from a massive star and was later homogenized through the disk.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/servlets/purl/5673494','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/servlets/purl/5673494"><span id="translatedtitle">Atomic mechanisms of. gamma. ' precipitate plate growth in the <span class="hlt">Al</span>-Ag <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Howe, J.M.</p> <p>1985-03-01</p> <p>Energy-dispersive x-ray spectroscopy results that the precipitates have the composition Ag/sub 2/<span class="hlt">Al</span>. High-resolution electron microscopy of ..gamma..' precipitates in both <110>//<1120> and <111>//<0001> orientations shows that all interfaces of the precipitate are largely coherent with the matrix and are faceted along low-energy (111) and (110) matrix plans, due to the influence of surface and elastic strain energies on the transformation. Further comparison between experimental and calculated high-resolution images of the precipitate/matrix interface and of Shockley partial dislocation ledges on the precipitate faces demonstrates that both thickening and lengthening of ..gamma..' precipitate plates occurs by the passage of the Shockley partial dislocations along alternate (111) matrix planes by a terrace-ledge-kink mechanism. These images and electron diffraction information also indicate that the ..gamma..' precipitates are ordered, where the A-planes in the precipitate contain nearly pure Ag and the B-planes have the composition <span class="hlt">Al</span>/sub 2/Ag, and that the limiting reaction in the growth process is the substitutional diffusion of Ag cross kinks in the Shockley partial dislocations, which terminate in the Ag-rich A-planes. The terraces between ledges are atomically flat and ledges are uniformly stepped-down from the centers to the edges of isolated precipitates. Convergent-beam electron diffraction (CBED) analyses of ..gamma..' precipitates indicate that they have the space group P6/sub 3//mmc. Effect of specimen thickness on symmetry determinations by CBED was also examined for an ..cap alpha..-titanium sample. Results show that the symmetries observed in CBED patterns from thin specimens may be due to the limited thickness of the specimen, rather than to the actual space group of the material.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/26939035','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/26939035"><span id="translatedtitle">New amide-chloride phases in the Li-<span class="hlt">Al</span>-N-H-Cl <span class="hlt">system</span>: formation and hydrogen storage behaviour.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Fernández Albanesi, L; Garroni, S; Enzo, S; Gennari, F C</p> <p>2016-04-01</p> <p>New amide-chloride phases were successfully synthesized by mechanical milling of the LiNH2-<span class="hlt">Al</span>Cl3 mixture at a molar ratio of 1 : 0.11 and further heating at 150 °C under argon (0.1 MPa) or under hydrogen pressure (0.7 MPa). Powder X-ray diffraction measurements as a function of milling time increase revealed that the milling of the LiNH2-0.11<span class="hlt">Al</span>Cl3 mixture results in the formation of a FCC solid solution with an excess of LiNH2. Subsequent heating of the LiNH2-0.11<span class="hlt">Al</span>Cl3 sample ball milled for 5 hours at 150 °C under argon or under hydrogen induces the appearance of an amide-chloride phase isostructural with cubic Li4(NH2)3Cl. This Li-<span class="hlt">Al</span>-N-H-Cl phase transforms progressively into the trigonal phase after prolonged heating at 300 °C under hydrogen pressure. The thermal behaviour of the amide-chloride without and with LiH addition displays dissimilar decomposition pathways. The decomposition of amide-chloride alone involves the formation of ammonia and hydrogen from 120 to 300 °C. Conversely, the amide-chloride material in the presence of LiH only releases hydrogen avoiding the emission of ammonia. The resultant material is able to be rehydrogenated under moderate conditions (300 °C, 0.7 MPa H2), providing a new reversible hydrogen storage <span class="hlt">system</span>.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://hdl.handle.net/2060/19890015352','NASA-TRS'); return false;" href="http://hdl.handle.net/2060/19890015352"><span id="translatedtitle">A three solar cell <span class="hlt">system</span> based on a self-supporting, transparent <span class="hlt">Al</span>GaAs top solar cell</span></a></p> <p><a target="_blank" href="http://ntrs.nasa.gov/search.jsp">NASA Technical Reports Server (NTRS)</a></p> <p>Negley, Gerald H.; Rhoads, Sandra L.; Terranova, Nancy E.; Mcneely, James B.; Barnett, Allen M.</p> <p>1989-01-01</p> <p>Development of a three solar cell stack can lead to practical efficiencies greater than 30 percent (1x,AM0). A theoretical efficiency limitation of 43.7 percent at AM0 and one sun is predicted by this model. Including expected losses, a practical <span class="hlt">system</span> efficiency of 36.8 percent is anticipated. These calculations are based on a 1.93eV/1.43eV/0.89eV energy band gap combination. <span class="hlt">Al</span>GaAs/GaAs/GaInAsP materials can be used with a six-terminal wiring configuration. The key issues for multijunction solar cells are the top and middle solar cell performance and the sub-bandgap transparency. AstroPower has developed a technique to fabricate <span class="hlt">Al</span>GaAs solar cells on rugged, self-supporting, transparent <span class="hlt">Al</span>GaAs substrates. Top solar cell efficiencies greater than 11 percent AM0 have been achieved. State-of-the-art GaAs or InP devices will be used for the middle solar cell. GaInAsP will be used to fabricate the bottom solar cell. This material is lattice-matched to InP and offers a wide range of bandgaps for optimization of the three solar cell stack. Liquid phase epitaxy is being used to grow the quaternary material. Initial solar cells have shown open-circuit voltages of 462 mV for a bandgap of 0.92eV. Design rules for the multijunction three solar cell stack are discussed. The progress in the development of the self-supporting <span class="hlt">Al</span>GaAs top solar cell and the GaInAsP bottom solar cell is presented.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=3670341','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=3670341"><span id="translatedtitle">182Hf–182W age dating of a 26<span class="hlt">Al</span>-poor inclusion and implications for the origin of short-lived radioisotopes in the early Solar <span class="hlt">System</span></span></a></p> <p><a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pmc">PubMed Central</a></p> <p>Holst, Jesper C.; Olsen, Mia B.; Paton, Chad; Nagashima, Kazuhide; Schiller, Martin; Wielandt, Daniel; Larsen, Kirsten K.; Connelly, James N.; Jørgensen, Jes K.; Krot, Alexander N.; Nordlund, Åke; Bizzarro, Martin</p> <p>2013-01-01</p> <p>Refractory inclusions [calcium–aluminum-rich inclusions, (CAIs)] represent the oldest Solar <span class="hlt">System</span> solids and provide information regarding the formation of the Sun and its protoplanetary disk. CAIs contain evidence of now extinct short-lived radioisotopes (e.g., 26<span class="hlt">Al</span>, 41Ca, and 182Hf) synthesized in one or multiple stars and added to the protosolar molecular cloud before or during its collapse. Understanding how and when short-lived radioisotopes were added to the Solar <span class="hlt">System</span> is necessary to assess their validity as chronometers and constrain the birthplace of the Sun. Whereas most CAIs formed with the canonical abundance of 26<span class="hlt">Al</span> corresponding to 26<span class="hlt">Al</span>/27<span class="hlt">Al</span> of ∼5 × 10−5, rare CAIs with fractionation and unidentified nuclear isotope effects (FUN CAIs) record nucleosynthetic isotopic heterogeneity and 26<span class="hlt">Al</span>/27<span class="hlt">Al</span> of <5 × 10−6, possibly reflecting their formation before canonical CAIs. Thus, FUN CAIs may provide a unique window into the earliest Solar <span class="hlt">System</span>, including the origin of short-lived radioisotopes. However, their chronology is unknown. Using the 182Hf–182W chronometer, we show that a FUN CAI recording a condensation origin from a solar gas formed coevally with canonical CAIs, but with 26<span class="hlt">Al</span>/27<span class="hlt">Al</span> of ∼3 × 10−6. The decoupling between 182Hf and 26<span class="hlt">Al</span> requires distinct stellar origins: steady-state galactic stellar nucleosynthesis for 182Hf and late-stage contamination of the protosolar molecular cloud by a massive star(s) for 26<span class="hlt">Al</span>. Admixing of stellar-derived 26<span class="hlt">Al</span> to the protoplanetary disk occurred during the epoch of CAI formation and, therefore, the 26Al–26Mg systematics of CAIs cannot be used to define their formation interval. In contrast, our results support 182Hf homogeneity and chronological significance of the 182Hf–182W clock. PMID:23671077</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://pubs.er.usgs.gov/publication/70016664','USGSPUBS'); return false;" href="http://pubs.er.usgs.gov/publication/70016664"><span id="translatedtitle">Revised values for the thermodynamic properties of boehmite, <span class="hlt">Al</span>O(OH) , and related species and phases in the <span class="hlt">system</span> <span class="hlt">Al</span>-H-O</span></a></p> <p><a target="_blank" href="http://pubs.er.usgs.gov/pubs/index.jsp?view=adv">USGS Publications Warehouse</a></p> <p>Hemingway, B.S.; Robie, R.A.; Apps, J.A.</p> <p>1991-01-01</p> <p>Heat capacity measurements are reported for a well-characterized boehmite that differ significantly from results reported earlier by Shomate and Cook (1946) for a monohydrate of alumina. It is suggested that the earlier measurements were made on a sample that was a mixture of phases and that use of that heat-capacity and derived thermodynamic data be discontinued. The entropy of boehmite derived in this study is 37.19 ?? 0.10 J/(mol.K) at 298.15 K. Based on our value for the entropy and accepting the recommended Gibbs free energy for <span class="hlt">Al</span>(OH)-4, the Gibbs free energy and enthalpy of formation of boehmite are calculated to be -918.4 ?? 2.1 and -996.4 ?? 2.2 kJ/mol, respectively, from solubility data for boehmite. The Gibbs energy for boehmite is unchanged from that given by Hemingway et <span class="hlt">al</span>. (1978). -from Authors</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2011JaJAP..50a5803Z','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2011JaJAP..50a5803Z"><span id="translatedtitle">Super Smooth Modification of <span class="hlt">Al</span>2O3 Ceramic Substrate by High Temperature Glaze of CaO-<span class="hlt">Al</span>2O3-SiO2 <span class="hlt">System</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Zhang, Jihua; Zhen, Shanxue; Yang, Lijun; Lou, Feizhi; Chen, Hongwei; Yang, Chuanren</p> <p>2011-01-01</p> <p>The rough surface of ceramic substrate is an obstacle for the scale down of line-width for thin film passive integrated devices (PID). In this paper, a modification method for <span class="hlt">Al</span>2O3 ceramic substrate with super smooth in surface was proposed. Coating a layer of CaO-<span class="hlt">Al</span>2O3-SiO2 (CAS) glass was performed to flat the rough surface of alumina substrate by sol-gel method. It was found that addition of 0.06% V2O5 can inhibit the recrystallization of the glaze. The root-mean-square (RMS) roughness of the glazed substrates reached a surprising flatness as small as 0.5 nm, and its melting temperature is higher than 1300 °C. This substrate with super flatness and high temperature endurance may be promising for high performance thin film devices.</p> </li> </ol> <div class="pull-right"> <ul class="pagination"> <li><a href="#" onclick='return showDiv("page_1");'>«</a></li> <li><a href="#" onclick='return showDiv("page_21");'>21</a></li> <li><a href="#" onclick='return showDiv("page_22");'>22</a></li> <li class="active"><span>23</span></li> <li><a href="#" onclick='return showDiv("page_24");'>24</a></li> <li><a href="#" onclick='return showDiv("page_25");'>25</a></li> <li><a href="#" onclick='return showDiv("page_25");'>»</a></li> </ul> </div> </div><!-- col-sm-12 --> </div><!-- row --> </div><!-- page_23 --> <div id="page_24" class="hiddenDiv"> <div class="row"> <div class="col-sm-12"> <div class="pull-right"> <ul class="pagination"> <li><a href="#" onclick='return showDiv("page_1");'>«</a></li> <li><a href="#" onclick='return showDiv("page_21");'>21</a></li> <li><a href="#" onclick='return showDiv("page_22");'>22</a></li> <li><a href="#" onclick='return showDiv("page_23");'>23</a></li> <li class="active"><span>24</span></li> <li><a href="#" onclick='return showDiv("page_25");'>25</a></li> <li><a href="#" onclick='return showDiv("page_25");'>»</a></li> </ul> </div> </div> </div> <div class="row"> <div class="col-sm-12"> <ol class="result-class" start="461"> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2012MS%26E...36a2028R','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2012MS%26E...36a2028R"><span id="translatedtitle">The Influence of impact on Composite Armour <span class="hlt">System</span> Kevlar-29/polyester-<span class="hlt">Al</span>2O3</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Ramadhan, A. A.; Abu Talib, A. R.; Mohd Rafie, A. S.; Zahari, R.</p> <p>2012-09-01</p> <p>An experimental investigation of high velocity impact responses of composite laminated plates using a helium gas gun has been presented in this paper. The aim of this study was to develop the novel composite structure that meets the specific requirements of ballistic resistance which used for body protections, vehicles and other applications. Thus the high velocity impact tests were performed on composite Kevlar-29 fiber/polyester resin with alumina powder (<span class="hlt">Al</span>2O3). The impact test was conducted by using a cylindrical steel projectile of 7.62mm diameter at a velocity range of 160-400 m/s. The results (shown in this work) are in terms of varying plate thickness and the amount of energy absorbed by the laminated plates meanwhile we obtained that the 12mm thickness of composite plate suitable for impact loading up to 200m/s impact velocity. Therefore this composite structure (it is used to reduce the amount of Kevlar) considered most economical armoure products. We used the ANSYS AUTODYN 3D- v.12 software for our simulations. The results have been obtained a4.1% maximum errors with experimental work of energy absorption.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/5689128','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/5689128"><span id="translatedtitle">Simultaneous precipitation of orthophosphate in activated sludge <span class="hlt">systems</span> with <span class="hlt">Al</span>(III)</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Gates, D.D.</p> <p>1991-01-01</p> <p>This dissertation examines the simultaneous precipitation of soluble orthophosphate (SOP) when alum is dosed to an activated sludge aeration basin. The results of batch and continuous flow experimental studies were used to develop a model of this process. This research identified three regions of chemical phosphorus removal, in each of which a different SOP removal mechanism appeared to apply: Region 1 extends to SOP residual concentrations as low as 1.0 mg P/1. In this region the stoichiometric precipitation of <span class="hlt">Al</span>{sub 0.91}H{sub 2}PO{sub 4} (OH){sub 1.73(s)} is the predominate phosphate removal mechanism. Region 2 includes SOP residual concentrations in the range 0.1-1.0 mg P/1. Phosphate removal in this region is described on the basis of the adsorption of SOP on to aluminum hydroxide solid surfaces. Region 3 includes SOP residual concentrations as low as 0.02 mg P/1. The minimum SOP phosphate concentration that can be reached in this region is controlled by the presence of both aluminum-hydroxyphosphate and aluminum hydroxide solids.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/1997SPIE.3172..724M','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/1997SPIE.3172..724M"><span id="translatedtitle">Spreading of the combustion wave in SiO2-<span class="hlt">Al</span> <span class="hlt">systems</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Maltsev, Vladimir M.; Gafiyatulina, Galina P.; Tavrov, Alexander V.</p> <p>1997-11-01</p> <p>The process of self-propagating high-temperature synthesis (SHS) has been applied as the new technology for production of construction materials and especial refractories with enhanced strength- and fire-resisting properties. These materials are aimed to be used as synthetic mullits lining refractories in heat units. In the visible light range, the following characteristics of SHS process have been investigated: the localization and the temperature of initiating of SHS process,the propagation of SHS wave on surface- or in volume, velocities of spreading of combustion wave, the number of SHS reaction stages. The visualization with a video-recording of the combustion wave of the SHS process has been performed inside the muffle furnace. The video has been processed with computer card of frame grabber and has been analyzed in multivideo mode, where each frame had been captured in fixed time interval. Thus, several mixtures of SiO2-<span class="hlt">Al</span> have been studied by variation of: the SiO2 particle size,the stoichiometric coefficient, by the substitution of the SiO2 to ashes and kaolin, and by the adding of supplementary components like Fe2O3. The SHS reactions are processed by the preliminary heating to the temperature of 650-860 degrees C. The local thermal self initiating of the SHS process and its propagation in the volume of a sample have been visualized. The multistage SHS reaction has been identified.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016JSSCh.237...48C','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016JSSCh.237...48C"><span id="translatedtitle">MAX phase - Alumina composites via elemental and exchange reactions in the Tin+1ACn <span class="hlt">systems</span> (A=<span class="hlt">Al</span>, Si, Ga, Ge, In and Sn)</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Cuskelly, Dylan; Richards, Erin; Kisi, Erich</p> <p>2016-05-01</p> <p>Extension of the aluminothermal exchange reaction synthesis of Mn+1AXn phases to <span class="hlt">systems</span> where the element 'A' is not the reducing agent was investigated in <span class="hlt">systems</span> TiO2-A-<span class="hlt">Al</span>-C for A=<span class="hlt">Al</span>, Si, Ga, Ge, In and Sn as well as Cr2O3-Ga-<span class="hlt">Al</span>-C. MAX phase-<span class="hlt">Al</span>2O3 composites were made in all <span class="hlt">systems</span> except those with A=Ga or In. The effectiveness of conversion to MAX phases was generally in the range 63-96% without optimisation of starting ratios. Optimisation in the Ti-Si-C <span class="hlt">system</span> gave a MAX phase component with >98% Ti3SiC2.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/24906288','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/24906288"><span id="translatedtitle">Multiple <span class="hlt">systemic</span> transplantations of human amniotic mesenchymal stem cells exert therapeutic effects in an <span class="hlt">ALS</span> mouse model.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Sun, Haitao; Hou, Zongliu; Yang, Huaqiang; Meng, Mingyao; Li, Peng; Zou, Qingjian; Yang, Lujun; Chen, Yuxin; Chai, Huihui; Zhong, Huilin; Yang, Zara Zhuyun; Zhao, Jing; Lai, Liangxue; Jiang, Xiaodan; Xiao, Zhicheng</p> <p>2014-09-01</p> <p>Amyotrophic lateral sclerosis (<span class="hlt">ALS</span>) is an adult-onset progressive neurodegenerative disease involving degeneration of motor neurons in the central nervous <span class="hlt">system</span>. Stem cell treatment is a potential therapy for this fatal disorder. The human amniotic membrane (HAM), an extremely rich and easily accessible tissue, has been proposed as an attractive material in cellular therapy and regenerative medicine because of its advantageous characteristics. In the present study, we evaluate the long-term effects of a cellular treatment by intravenous administration of human amniotic mesenchymal stem cells (hAMSCs) derived from HAM into a hSOD1(G93A) mouse model. The mice received <span class="hlt">systemic</span> administration of hAMSCs or phosphate-buffered saline (PBS) at the onset, progression and symptomatic stages of the disease. hAMSCs were detected in the spinal cord at the final stage of the disease, in the form of isolates or clusters and were negative for β-tubulin III and GFAP. Compared with the treatment with PBS, multiple hAMSC transplantations significantly retarded disease progression, extended survival, improved motor function, prevented motor neuron loss and decreased neuroinflammation in mice. These findings demonstrate that hAMSC transplantation is a promising cellular treatment for <span class="hlt">ALS</span>.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/1983ZNatA..38..811D','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/1983ZNatA..38..811D"><span id="translatedtitle">Notizen: Raman-Spektroskopische Untersuchungen der <span class="hlt">Systeme</span> ASCl3/Cl2-<span class="hlt">Al</span>Cl3 und AsCl3 /Cl2-GaCl3/ Raman Spectra of the <span class="hlt">Systems</span> AsCl3/Cl2—<span class="hlt">Al</span>Cl3 and AsCl3/Cl2-GaCl3</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Demircan, B.; Brockner, W.</p> <p>1983-07-01</p> <p>In the <span class="hlt">systems</span> investigated only the compounds As<span class="hlt">Al</span>Cl8 and AsGaCl8 g are found. Raman spectra were recorded for the solid and molten compounds and for mixtures with excess ASCl3 + CL2 and <span class="hlt">Al</span>Cl3 and GaCl3 , respectively. The observed frequencies are assigned to tetrahedral AsCl4+, <span class="hlt">Al</span>Cl4- and GaCl4- units. Melting of AsCl4<span class="hlt">Al</span>Cl 4 and AsCl4GaCl4 causes a decomposition into AsCl3 , Cl2 and <span class="hlt">Al</span>Cl3 and GaCl3 , respectively.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://hdl.handle.net/2060/19960054113','NASA-TRS'); return false;" href="http://hdl.handle.net/2060/19960054113"><span id="translatedtitle">InGa<span class="hlt">Al</span>AsPN: A Materials <span class="hlt">System</span> for Silicon Based Optoelectronics and Heterostructure Device Technologies</span></a></p> <p><a target="_blank" href="http://ntrs.nasa.gov/search.jsp">NASA Technical Reports Server (NTRS)</a></p> <p>Broekaert, T. P. E.; Tang, S.; Wallace, R. M.; Beam, E. A., III; Duncan, W. M.; Kao, Y. -C.; Liu, H. -Y.</p> <p>1995-01-01</p> <p>A new material <span class="hlt">system</span> is proposed for silicon based opto-electronic and heterostructure devices; the silicon lattice matched compositions of the (In,Ga,<span class="hlt">Al</span>)-(As,P)N 3-5 compounds. In this nitride alloy material <span class="hlt">system</span>, the bandgap is expected to be direct at the silicon lattice matched compositions with a bandgap range most likely to be in the infrared to visible. At lattice constants ranging between those of silicon carbide and silicon, a wider bandgap range is expected to be available and the high quality material obtained through lattice matching could enable applications such as monolithic color displays, high efficiency multi-junction solar cells, opto-electronic integrated circuits for fiber communications, and the transfer of existing 3-5 technology to silicon.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://ntrs.nasa.gov/search.jsp?R=19840031712&hterms=business+Green&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D40%26Ntt%3Dbusiness%2BGreen','NASA-TRS'); return false;" href="http://ntrs.nasa.gov/search.jsp?R=19840031712&hterms=business+Green&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D40%26Ntt%3Dbusiness%2BGreen"><span id="translatedtitle">Theoretical study of the <span class="hlt">Al</span>O blue-green (B2Sigma + - X2Sigma +) band <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://ntrs.nasa.gov/search.jsp">NASA Technical Reports Server (NTRS)</a></p> <p>Partridge, H.; Langhoff, S. R.; Lengsfield, B. H., III; Liu, B.</p> <p>1983-01-01</p> <p>Two independent, extensive theoretical calculations are reported for the relative band strengths of the <span class="hlt">Al</span>O (B2Sigma + - X2Sigma +) blue-green <span class="hlt">system</span> and for the radiative lifetimes of the lowest few vibrational levels of the B2Sigma(+) state. The theoretical lifetimes, which include a small (less than -.5 percent) contribution from bound-bound transitions into the A2Pi state, are in excellent agreement with laser fluorescence studies. The theoretical lifetimes increase monotonically and very slowly with increasing vibrational quantum number. The relative band strengths for the blue-green <span class="hlt">system</span> derived from the two theoretical calculations are in excellent agreement, but differ systematically from the relative band strengths of Linton and Nicholls (1969). The present results suggest that their self-absorption corrections are not large enough, resulting in relative intensities that are too large, especially for the weak bands with r centroids less than 1.5 A.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4200032','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4200032"><span id="translatedtitle">Development of a biocompatible nanodelivery <span class="hlt">system</span> for tuberculosis drugs based on isoniazid-Mg/<span class="hlt">Al</span> layered double hydroxide</span></a></p> <p><a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pmc">PubMed Central</a></p> <p>Saifullah, Bullo; Arulselvan, Palanisamy; El Zowalaty, Mohamed Ezzat; Fakurazi, Sharida; Webster, Thomas J; Geilich, Benjamin M; Hussein, Mohd Zobir</p> <p>2014-01-01</p> <p>The primary challenge in finding a treatment for tuberculosis (TB) is patient non-compliance to treatment due to long treatment duration, high dosing frequency, and adverse effects of anti-TB drugs. This study reports on the development of a nanodelivery <span class="hlt">system</span> that intercalates the anti-TB drug isoniazid into Mg/<span class="hlt">Al</span> layered double hydroxides (LDHs). Isoniazid was found to be released in a sustained manner from the novel nanodelivery <span class="hlt">system</span> in humans in simulated phosphate buffer solutions at pH 4.8 and pH 7.4. The nanodelivery formulation was highly biocompatible compared to free isoniazid against human normal lung and 3T3 mouse fibroblast cells. The formulation was active against Mycobacterium tuberculosis and gram-positive bacteria and gram-negative bacteria. Thus results show significant promise for the further study of these nanocomposites for the treatment of TB. PMID:25336952</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://ntrs.nasa.gov/search.jsp?R=19840044028&hterms=Mn&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D70%26Ntt%3DMn','NASA-TRS'); return false;" href="http://ntrs.nasa.gov/search.jsp?R=19840044028&hterms=Mn&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D70%26Ntt%3DMn"><span id="translatedtitle">Carbides in iron-rich Fe-Mn-Cr-Mo-<span class="hlt">Al</span>-Si-C <span class="hlt">systems</span></span></a></p> <p><a target="_blank" href="http://ntrs.nasa.gov/search.jsp">NASA Technical Reports Server (NTRS)</a></p> <p>Lemkey, F. D.; Gupta, H.; Nowotny, H.; Wayne, S. F.</p> <p>1984-01-01</p> <p>The optimization of high carbon iron-base superalloy properties with duplex microstructure gamma + M7C3 carbide requires analysis in the context of a seven-component <span class="hlt">system</span>. Data are first provided here for the Fe-Mn-Cr-Mo-C quinary <span class="hlt">system</span>, at 30 at. pct carbon. A characterization of competing carbides, according to a pseudoternary phase diagram at 35 wt pct iron, is made from isothermal sections. It is noted that while M7C3 and M3C carbides' occurrences are respectively favored at the Cr and Mn corners, the M2C carbide and molybdenum cementite are predominant with increasing amounts of Mo. Lattice parameters are reported for the various carbides.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/441495','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/441495"><span id="translatedtitle">Durability of Ti-6<span class="hlt">Al</span>-4V/LaRC-PETI-5 adhesive bonded <span class="hlt">system</span> for HSCT applications</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Parvatareddy, H.; Pasricha, A.; Dillard, D.A.; Dillard, J.G.</p> <p>1996-12-31</p> <p>Structural adhesive joints are being widely used and studied as alternatives to conventional fasteners in the aerospace, automotive, and other industries. Adhesive bonding offers advantages such as lower weight and lower manufacturing costs. Furthermore, high performance adhesives which are currently being synthesized (e.g. epoxies, phenolics, acrylics, thermoplastic polyimides) offer other useful properties such as higher modulus, higher toughness, and stability at high temperatures. In the present study, the durability of the Ti-6<span class="hlt">Al</span>-4V/LaRC PETI-5 adhesive bonded <span class="hlt">system</span> is being evaluated utilizing double cantilever beam (DCB) fracture specimens. These DCB tests have been used extensively to study adhesive joints. The current study is part of a comprehensive study to develop a durable material <span class="hlt">system</span> for application in the proposed mach 2.4 high speed civil transport (HSCT) aircraft. According to the design criteria, the material <span class="hlt">system</span> to be used on the aircraft should be durable for over 60,000 hours of flight encountering temperatures during flight in the range of 177{degrees}C. Physical aging and chemical aging of the adhesive material are some of the important issues which have to be evaluated and taken into consideration for predicting the bond durability. In order to simulate the service environment conditions of the HSCT, the Ti-6<span class="hlt">Al</span>-4V/LaRC PETI-5 bonds were aged in one of three temperatures; 150, 177, and 204{degrees}C, at one of three different environments; atmospheric air, and reduced air pressures of 2 psi air (13.8 KPa) and 0.2 psi air (1.38 KPa).</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/cgi-bin/nph-data_query?bibcode=2013JAP...113k3510H&link_type=ABSTRACT','NASAADS'); return false;" href="http://adsabs.harvard.edu/cgi-bin/nph-data_query?bibcode=2013JAP...113k3510H&link_type=ABSTRACT"><span id="translatedtitle">Alloying-related trends from first principles: An application to the Ti-<span class="hlt">Al</span>-X-N <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Holec, David; Zhou, Liangcai; Rachbauer, Richard; Mayrhofer, Paul H.</p> <p>2013-03-01</p> <p>Tailoring and improving material properties by alloying is a long-known and used concept. Recent research has demonstrated the potential of ab initio calculations in understanding the material properties at the nanoscale. Here, we present a systematic overview of alloying trends when early transition metals (Y, Zr, Nb, Hf, and Ta) are added in the Ti1-xAlxN <span class="hlt">system</span>, routinely used as a protective hard coating. The alloy lattice parameters tend to be larger than the corresponding linearised Vegard's estimation, with the largest deviation more than 2.5% obtained for Y0.5<span class="hlt">Al</span>0.5N. The chemical strengthening is most pronounced for Ta and Nb, although also causing smallest elastic distortions of the lattice due to their atomic radii being comparable with Ti and <span class="hlt">Al</span>. This is further supported by the analysis of the electronic density of states. Finally, mixing enthalpy as a measure of the driving force for decomposition into the stable constituents is enhanced by adding Y, Zr, and Nb, suggesting that the onset of spinodal decomposition will appear in these cases for lower thermal loads than for Hf and Ta alloyed Ti1-xAlxN.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://medlineplus.gov/ency/article/002189.htm','NIH-MEDLINEPLUS'); return false;" href="https://medlineplus.gov/ency/article/002189.htm"><span id="translatedtitle"><span class="hlt">ALS</span> - resources</span></a></p> <p><a target="_blank" href="http://medlineplus.gov/">MedlinePlus</a></p> <p></p> <p></p> <p>Resources - <span class="hlt">ALS</span> ... The following organizations are good resources for information on amyotrophic lateral sclerosis : Muscular Dystrophy Association -- mda.org/disease/amyotrophic-lateral-sclerosis National Amyotrophic Lateral Sclerosis (<span class="hlt">ALS</span>) Registry -- ...</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4844960','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4844960"><span id="translatedtitle">Mechanism of stress-driven composition evolution during hetero-epitaxy in a ternary <span class="hlt">Al</span>GaN <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pmc">PubMed Central</a></p> <p>He, Chenguang; Qin, Zhixin; Xu, Fujun; Zhang, Lisheng; Wang, Jiaming; Hou, Mengjun; Zhang, Shan; Wang, Xinqiang; Ge, Weikun; Shen, Bo</p> <p>2016-01-01</p> <p>Two <span class="hlt">Al</span>GaN samples with different strain were designed to investigate mechanism of stress-driven composition evolution. It is discovered that <span class="hlt">Al</span>GaN grown on <span class="hlt">Al</span>N or (<span class="hlt">Al</span>N/GaN superlattices (SLs))/GaN both consist of two distinct regions with different compositions: transition region and uniform region, which is attributed to the compositional pulling effect. The formation of the transition region is due to the partial stress release caused by the generation of misfit dislocations near the hetero-interface. And the <span class="hlt">Al</span> composition in the uniform region depends on the magnitude of residual strain. The difference in relaxation degree is 80.5% for the <span class="hlt">Al</span>GaN epilayers grown on different underlayers, leading to a large <span class="hlt">Al</span> composition difference of 22%. The evolutionary process of <span class="hlt">Al</span> composition along [0001] direction was investigated in detail. PMID:27112969</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016ApSS..388..503D','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016ApSS..388..503D"><span id="translatedtitle">Microstructure and property of diamond-like carbon films with <span class="hlt">Al</span> and Cr co-doping deposited using a hybrid beams <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Dai, Wei; Liu, Jingmao; Geng, Dongsen; Guo, Peng; Zheng, Jun; Wang, Qimin</p> <p>2016-12-01</p> <p>DLC films with weak carbide former <span class="hlt">Al</span> and carbide former Cr co-doping (<span class="hlt">Al</span>:Cr-DLC) were deposited by a hybrid beams <span class="hlt">system</span> comprising an anode-layer linear ion beam source (LIS) and high power impulse magnetron sputtering using a gas mixture of C2H2 and Ar as the precursor. The doped <span class="hlt">Al</span> and Cr contents were controlled via adjusting the C2H2 fraction in the gas mixture. The composition, microstructure, compressive stress, mechanical properties and tribological behaviors of the <span class="hlt">Al</span>:Cr-DLC films were researched carefully using X-ray photoelectron spectroscopy, transmission electron microscopy, Raman spectroscopy, stress-tester, nanoindentation and ball-on-plate tribometer as function of the C2H2 fraction. The results show that the <span class="hlt">Al</span> and Cr contents in the films increased continuously as the C2H2 fraction decreased. The doped Cr atoms preferred to bond with the carbon while the <span class="hlt">Al</span> atoms mainly existed in metallic state. Structure modulation with alternate multilayer consisted of <span class="hlt">Al</span>-poor DLC layer and <span class="hlt">Al</span>-rich DLC layer was found in the films. Those periodic <span class="hlt">Al</span>-rich DLC layers can effectively release the residual stress of the films. On the other hand, the formation of the carbide component due to Cr incorporation can help to increase the film hardness. Accordingly, the residual stress of the DLC films can be reduced without sacrificing the film hardness though co-doping <span class="hlt">Al</span> and Cr atoms. Furthermore, it was found that the periodic <span class="hlt">Al</span>-rich layer can greatly improve the elastic resilience of the DLC films and thus decreases the film friction coefficient and wear rate significantly. However, the existence of the carbide component would cause abrasive wear and thus deteriorate the wear performance of the films.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://ntrs.nasa.gov/search.jsp?R=20040043716&hterms=ALS&qs=N%3D0%26Ntk%3DAll%26Ntx%3Dmode%2Bmatchall%26Ntt%3DALS','NASA-TRS'); return false;" href="http://ntrs.nasa.gov/search.jsp?R=20040043716&hterms=ALS&qs=N%3D0%26Ntk%3DAll%26Ntx%3Dmode%2Bmatchall%26Ntt%3DALS"><span id="translatedtitle">Using <span class="hlt">System</span> Mass (SM), Equivalent Mass (EM), Equivalent <span class="hlt">System</span> Mass (ESM) or Life Cycle Mass (LCM) in Advanced Life Support (<span class="hlt">ALS</span>) Reporting</span></a></p> <p><a target="_blank" href="http://ntrs.nasa.gov/search.jsp">NASA Technical Reports Server (NTRS)</a></p> <p>Jones, Harry</p> <p>2003-01-01</p> <p>The Advanced Life Support (<span class="hlt">ALS</span>) has used a single number, Equivalent <span class="hlt">System</span> Mass (ESM), for both reporting progress and technology selection. ESM is the launch mass required to provide a space <span class="hlt">system</span>. ESM indicates launch cost. ESM alone is inadequate for technology selection, which should include other metrics such as Technology Readiness Level (TRL) and Life Cycle Cost (LCC) and also consider perfom.arxe 2nd risk. ESM has proven difficult to implement as a reporting metric, partly because it includes non-mass technology selection factors. Since it will not be used exclusively for technology selection, a new reporting metric can be made easier to compute and explain. <span class="hlt">Systems</span> design trades-off performance, cost, and risk, but a risk weighted cost/benefit metric would be too complex to report. Since life support has fixed requirements, different <span class="hlt">systems</span> usually have roughly equal performance. Risk is important since failure can harm the crew, but it is difficult to treat simply. Cost is not easy to estimate, but preliminary space <span class="hlt">system</span> cost estimates are usually based on mass, which is better estimated than cost. Amass-based cost estimate, similar to ESM, would be a good single reporting metric. The paper defines and compares four mass-based cost estimates, Equivalent Mass (EM), Equivalent <span class="hlt">System</span> Mass (ESM), Life Cycle Mass (LCM), and <span class="hlt">System</span> Mass (SM). EM is traditional in life support and includes mass, volume, power, cooling and logistics. ESM is the specifically defined <span class="hlt">ALS</span> metric, which adds crew time and possibly other cost factors to EM. LCM is a new metric, a mass-based estimate of LCC measured in mass units. SM includes only the factors of EM that are originally measured in mass, the hardware and logistics mass. All four mass-based metrics usually give similar comparisons. SM is by far the simplest to compute and easiest to explain.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016JCrGr.448....1Y','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016JCrGr.448....1Y"><span id="translatedtitle">Colony structure in Ce-doped <span class="hlt">Al</span>2O3/YAG eutectic <span class="hlt">systems</span> grown by vertical Bridgman technique</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Yamada, Seiya; Yoshimura, Masafumi; Sakata, Shin-ichi; Taishi, Toshinori; Hoshikawa, Keigo</p> <p>2016-08-01</p> <p>We investigated the influence of growth rate and Ce concentration on colony structure variation in <span class="hlt">Al</span>2O3/YAG:Ce eutectic <span class="hlt">systems</span>. The distance between boundary zones in the colony structure decreased with increases in either growth rate or Ce concentration. The eutectic spacing in the coarse microstructure in the boundary zone decreased with increasing growth rate but increased with increasing Ce concentration. We conclude that the colony structure is formed by cellular growth driven by constitutional supercooling with an interface instability due to Ce atom accumulation, so that the distance between boundary zones depends on both the growth rate and Ce concentration, and the coarse microstructure in the boundary zone depends on the solidification rate perpendicular to the growth interface at the cell bottom of the microscopic growth interface shape in the cellular growth.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/26965404','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/26965404"><span id="translatedtitle">Landfill site selection using geographic information <span class="hlt">system</span> and analytical hierarchy process: A case study <span class="hlt">Al</span>-Hillah Qadhaa, Babylon, Iraq.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Chabuk, Ali; Al-Ansari, Nadhir; Hussain, Hussain Musa; Knutsson, Sven; Pusch, Roland</p> <p>2016-05-01</p> <p><span class="hlt">Al</span>-Hillah Qadhaa is located in the central part of Iraq. It covers an area of 908 km(2) with a total population of 856,804 inhabitants. This Qadhaa is the capital of Babylon Governorate. Presently, no landfill site exists in that area based on scientific site selection criteria. For this reason, an attempt has been carried out to find the best locations for landfills. A total of 15 variables were considered in this process (groundwater depth, rivers, soil types, agricultural land use, land use, elevation, slope, gas pipelines, oil pipelines, power lines, roads, railways, urban centres, villages and archaeological sites) using a geographic information <span class="hlt">system</span>. In addition, an analytical hierarchy process was used to identify the weight for each variable. Two suitable candidate landfill sites were determined that fulfil the requirements with an area of 9.153 km(2) and 8.204 km(2) These sites can accommodate solid waste till 2030.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/22261797','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/22261797"><span id="translatedtitle">Electron heating due to microwave photoexcitation in the high mobility GaAs/<span class="hlt">Al</span>GaAs two dimensional electron <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Ramanayaka, A. N.; Mani, R. G.; Wegscheider, W.</p> <p>2013-12-04</p> <p>We extract the electron temperature in the microwave photo-excited high mobility GaAs/<span class="hlt">Al</span>GaAs two dimensional electron <span class="hlt">system</span> (2DES) by studying the influence of microwave radiation on the amplitude of Shubnikov-de Haas oscillations (SdHOs) in a regime where the cyclotron frequency, ω{sub c}, and the microwave angular frequency, ω, satisfy 2ω ≤ ω{sub c} ≤ 3.5ω The results indicate that increasing the incident microwave power has a weak effect on the amplitude of the SdHOs and therefore the electron temperature, in comparison to the influence of modest temperature changes on the dark-specimen SdH effect. The results indicate negligible electron heating under modest microwave photo-excitation, in good agreement with theoretical predictions.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2014EGUGA..1611097L','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2014EGUGA..1611097L"><span id="translatedtitle">Offshore fault <span class="hlt">system</span> in the <span class="hlt">Al</span> Hoceima region from new high-resolution bathymetric and seismic reflection data</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Lafosse, Manfred; d'Acremont, Elia; Rabaute, Alain; Mercier de l'Epinay, Bernard; Gorini, Christian; André Gutscher, Marc; Poort, Jeffrey; Ammar, Abdellah; Tahayt, Abdelilah; Leroy, Pascal; Smit, Jeroen; Do Couto, Damien; Cancouët, Romain; Prunier, Christophe; Ercilla, Gemma</p> <p>2014-05-01</p> <p>The <span class="hlt">Al</span>-Hoceima Region (Morocco) is the one of the most active seismic area of the western Mediterranean Sea. Detailed surveys in a shallow water environment are required to identify the connecting onshore-offshore active structures and to propose a tectonic framework. We use combined high-resolution seismic reflection and swath-bathymetry data from the Marlboro-2 cruise, which took place in 2012 off the coast of <span class="hlt">Al</span> Hoceima, to detail the fault <span class="hlt">system</span> through the Nekor basin, between the Trougout Fault and the Boussekour Agdal fault. The Boussekour-Agdal fault is a N026 oriented fault, dipping east and affecting the plio-quaternary sequence offshore and the internal units of the oriental Rif onshore. The fault trace shows a vertical offset of 6.5 m on the high-resolution swath bathymetry close to the shoreline, while the northern prolongation of the fault is buried. The Bokkoya fault (Calvert et <span class="hlt">al</span>. 1997) is a N029 oriented fault dipping east. The vertical offset at the seafloor is 13m. This fault affects sedimentary structures above a paleo-terrace at -105mbsl, probably related to the last sea-level fall. The onshore-offshore N-S oriented Trougout fault corresponds to the eastern boundary between the plio-quaternary Nekor basin and the volcano-clastic deposits of Ras Tarf. This fault produces a vertical offset of 2.3m at the sea-floor. These three major fault zones limit two basins: the Nekor basin between the Bokkoya and the Trougout faults, and a depression between the Boussekour-Agdal and the Bokkoya Faults. The quaternary deposits are syn-tectonic. In the Nekor basin secondary normal faults are oriented N150, shift the sea-floor and affect the Messinian unconformity. Successive positions of a paleo-canyon (seen in the seismic lines) show a migration of the subsidence from east to west inside the Nekor basin. Faults affecting the Messinian unconformity control this subsidence. Between the Boussekour-Agdal and the Bokkoya faults, the thickness and the geometry of</p> </li> </ol> <div class="pull-right"> <ul class="pagination"> <li><a href="#" onclick='return showDiv("page_1");'>«</a></li> <li><a href="#" onclick='return showDiv("page_21");'>21</a></li> <li><a href="#" onclick='return showDiv("page_22");'>22</a></li> <li><a href="#" onclick='return showDiv("page_23");'>23</a></li> <li class="active"><span>24</span></li> <li><a href="#" onclick='return showDiv("page_25");'>25</a></li> <li><a href="#" onclick='return showDiv("page_25");'>»</a></li> </ul> </div> </div><!-- col-sm-12 --> </div><!-- row --> </div><!-- page_24 --> <div id="page_25" class="hiddenDiv"> <div class="row"> <div class="col-sm-12"> <div class="pull-right"> <ul class="pagination"> <li><a href="#" onclick='return showDiv("page_1");'>«</a></li> <li><a href="#" onclick='return showDiv("page_21");'>21</a></li> <li><a href="#" onclick='return showDiv("page_22");'>22</a></li> <li><a href="#" onclick='return showDiv("page_23");'>23</a></li> <li><a href="#" onclick='return showDiv("page_24");'>24</a></li> <li class="active"><span>25</span></li> <li><a href="#" onclick='return showDiv("page_25");'>»</a></li> </ul> </div> </div> </div> <div class="row"> <div class="col-sm-12"> <ol class="result-class" start="481"> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/servlets/purl/10139691','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/servlets/purl/10139691"><span id="translatedtitle">Phase relationships and stability of the {mu}- and {zeta}-phases in the Ag-<span class="hlt">Al</span>-X (X=Zn, Ga, Ge) <span class="hlt">systems</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Paruchuri, M.R.; Massalaski, T.B.</p> <p>1993-09-01</p> <p>Details of phase relationships in three ternary <span class="hlt">systems</span>, Ag-<span class="hlt">Al</span>-X (X=Zn, Ga, Ge) near the Ag-rich corner at 400C are presented. Metallography, X-ray diffraction, scanning electron microscopy and X-ray spectroscopy have been used to establish the respective isothermal sections at 400C. In each case, the {mu}-phase and the {zeta}-phase of the Ag-<span class="hlt">Al</span> binary <span class="hlt">system</span> extend into the ternary <span class="hlt">system</span> and terminate at a three-phase region involving the Ag-rich primary solid solution ({alpha}{sub 1}-phase) in the Ag-<span class="hlt">Al</span>-Zn and Ag-<span class="hlt">Al</span>-Ga <span class="hlt">systems</span>, and the Ge-rich primary solid solution ({alpha}{sub 2}-phase) in the Ag-<span class="hlt">Al</span>-Ge ternary <span class="hlt">system</span>. The stability ranges of the {mu} and {zeta}-phases follow approximately constant electron concentration lines. The solid solubilities of Zn, Ga and Ge in the {mu}-phase are relatively small, compared with those in the {zeta}-phase (up to 18 at. %). No ternary phase appears to exist in the Ag-rich portions studied in the three ternary <span class="hlt">systems</span>.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016GeCoA.187...41M','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016GeCoA.187...41M"><span id="translatedtitle">Internally consistent thermodynamic data for aqueous species in the <span class="hlt">system</span> Na-K-<span class="hlt">Al</span>-Si-O-H-Cl</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Miron, George D.; Wagner, Thomas; Kulik, Dmitrii A.; Heinrich, Christoph A.</p> <p>2016-08-01</p> <p>A large amount of critically evaluated experimental data on mineral solubility, covering the entire Na-K-<span class="hlt">Al</span>-Si-O-H-Cl <span class="hlt">system</span> over wide ranges in temperature and pressure, was used to simultaneously refine the standard state Gibbs energies of aqueous ions and complexes in the framework of the revised Helgeson-Kirkham-Flowers equation of state. The thermodynamic properties of the solubility-controlling minerals were adopted from the internally consistent dataset of Holland and Powell (2002; Thermocalc dataset ds55). The global optimization of Gibbs energies of aqueous species, performed with the GEMSFITS code (Miron et <span class="hlt">al</span>., 2015), was set up in such a way that the association equilibria for ion pairs and complexes, independently derived from conductance and potentiometric data, are always maintained. This was achieved by introducing reaction constraints into the parameter optimization that adjust Gibbs energies of complexes by their respective Gibbs energy effects of reaction, whenever the Gibbs energies of reactant species (ions) are changed. The optimized thermodynamic dataset is reported with confidence intervals for all parameters evaluated by Monte Carlo trial calculations. The new thermodynamic dataset is shown to reproduce all available fluid-mineral phase equilibria and mineral solubility data with good accuracy and precision over wide ranges in temperature (25-800 °C), pressure (1 bar to 5 kbar) and composition (salt concentrations up to 5 molal). The global data optimization process adopted in this study can be readily repeated any time when extensions to new chemical elements and species are needed, when new experimental data become available, or when a different aqueous activity model or equation of state should be used. This work serves as a proof of concept that our optimization strategy is feasible and successful in generating a thermodynamic dataset reproducing all fluid-mineral and aqueous speciation equilibria in the Na-K-<span class="hlt">Al</span>-Si-O-H-Cl <span class="hlt">system</span> within</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/5300162','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/5300162"><span id="translatedtitle">Value of positive myocardial technetium-99m-pyrophosphate scintigraphy in the noninvasive diagnosis of cardiac <span class="hlt">amyloidosis</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Wizenberg, T.A.; Muz, J.; Sohn, Y.H.; Samlowski, W.; Weissler, A.M.</p> <p>1982-04-01</p> <p>Ten consecutive patients with tissue-proven <span class="hlt">amyloidosis</span>, seven of whom presented with congestive heart failure, were found to exhibit intense diffuse uptake of technetium-99m-pyrophosphate (Tc-99m-PYP) on cardiac radionuclide imaging. The patients exhibited echocardiographic and systolic time interval abnormalities suggesting combined restrictive and congestive cardiomyopathic changes. On M-mode echocardiograms, there was symmetrically increased thickness of the interventricular septum and left ventricular (LV) posterior wall in diastole (10 of 10), decreased fractional shortening of the LV minor axis diameter in systole (eight of nine), and decreased percent thickening of the LV minor axis diameter in systole (eight of nine) and LV posterior wall (10 of 10) in systole. Three patients demonstrated enlarged LV end-diastolic diameter. All 10 patients had abnormal PEP/LVET and eight had shortened LVETI. When combined with noninvasive tests of LV performance, positive myocardial pyrophosphate (PYP) scanning provides a new and useful adjunct in the diagnosis of amyloid heart disease.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4940161','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4940161"><span id="translatedtitle">Time-resolved studies define the nature of toxic IAPP intermediates, providing insight for anti-<span class="hlt">amyloidosis</span> therapeutics</span></a></p> <p><a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pmc">PubMed Central</a></p> <p>Abedini, Andisheh; Plesner, Annette; Cao, Ping; Ridgway, Zachary; Zhang, Jinghua; Tu, Ling-Hsien; Middleton, Chris T; Chao, Brian; Sartori, Daniel J; Meng, Fanling; Wang, Hui; Wong, Amy G; Zanni, Martin T; Verchere, C Bruce; Raleigh, Daniel P; Schmidt, Ann Marie</p> <p>2016-01-01</p> <p>Islet <span class="hlt">amyloidosis</span> by IAPP contributes to pancreatic β-cell death in diabetes, but the nature of toxic IAPP species remains elusive. Using concurrent time-resolved biophysical and biological measurements, we define the toxic species produced during IAPP amyloid formation and link their properties to induction of rat INS-1 β-cell and murine islet toxicity. These globally flexible, low order oligomers upregulate pro-inflammatory markers and induce reactive oxygen species. They do not bind 1-anilnonaphthalene-8-sulphonic acid and lack extensive β-sheet structure. Aromatic interactions modulate, but are not required for toxicity. Not all IAPP oligomers are toxic; toxicity depends on their partially structured conformational states. Some anti-amyloid agents paradoxically prolong cytotoxicity by prolonging the lifetime of the toxic species. The data highlight the distinguishing properties of toxic IAPP oligomers and the common features that they share with toxic species reported for other amyloidogenic polypeptides, providing information for rational drug design to treat IAPP induced β-cell death. DOI: http://dx.doi.org/10.7554/eLife.12977.001 PMID:27213520</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.ncbi.nlm.nih.gov/pubmed/27251043','PUBMED'); return false;" href="http://www.ncbi.nlm.nih.gov/pubmed/27251043"><span id="translatedtitle">Protective Effects of Forskolin on Behavioral Deficits and Neuropathological Changes in a Mouse Model of Cerebral <span class="hlt">Amyloidosis</span>.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Owona, Brice Ayissi; Zug, Caroline; Schluesener, Hermann J; Zhang, Zhi-Yuan</p> <p>2016-07-01</p> <p>The production of amyloid-β peptides in the brains of patients with Alzheimer disease (AD) may contribute to memory loss and impairments in social behavior. Here, an efficient adenylate cyclase activator, forskolin, was orally administered by gavage (100 mg/kg body weight) to 5-month-old transgenic APP/PS1 mice, which serve as an animal model of cerebral <span class="hlt">amyloidosis</span>. Analyses of nest construction, sociability, and immunohistochemical features were used to determine the effects of forskolin treatment. After a relatively short term of treatment (10 days), forskolin-treated transgenic mice showed restored nest construction ability (p < 0.05) and their sociability (p < 0.01). There was a reduction of Aβ plaque deposition in the cortex and in the hippocampus. Furthermore, expression of transforming growth factor β, glial fibrillary acidic protein, and Iba-1 in the cortex was reduced in the forskolin-treated group, suggesting regulation of the inflammatory response mediated by activated microglia and astrocytes in the brains of the APP/PS1 mice (p < 0.01). Taken together, these findings suggest that forskolin shows neuroprotective effects in APP/PS1 Tg mice and may be a promising drug in the treatment of patients with AD. PMID:27251043</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://hdl.handle.net/2060/20040045268','NASA-TRS'); return false;" href="http://hdl.handle.net/2060/20040045268"><span id="translatedtitle">Using Technology Readiness Level (TRL), Life Cycle Cost (LCC), and Other Metrics to Supplement Equivalent <span class="hlt">System</span> Mass (ESM) in Advanced Life Support (<span class="hlt">ALS</span>)</span></a></p> <p><a target="_blank" href="http://ntrs.nasa.gov/search.jsp">NASA Technical Reports Server (NTRS)</a></p> <p>Jones, Harry</p> <p>2003-01-01</p> <p>The <span class="hlt">ALS</span> project plan goals are reducing cost, improving performance, and achieving flight readiness. <span class="hlt">ALS</span> selects projects to advance the mission readiness of low cost, high performance technologies. The role of metrics is to help select good projects and report progress. The Equivalent Mass (EM) of a <span class="hlt">system</span> is the sum of the estimated mass of the hardware, of its required materials and spares, and of the pressurized volume, power supply, and cooling <span class="hlt">system</span> needed to support the hardware in space. EM is the total payload launch mass needed to provide and support a <span class="hlt">system</span>. EM is directly proportional to the launch cost.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/5354413','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/5354413"><span id="translatedtitle">Aluminum fluxing salts: a critical review of the chemistry and structures of alkali aluminum halides. Information circular/1986. [<span class="hlt">System</span> NaCl-KCl-<span class="hlt">Al</span>Cl/sub 3/-NaF-KF-<span class="hlt">Al</span>F/sub 3/</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Sorrell, C.A.; Groetsch, J.G.; Soboroff, D.M.</p> <p>1986-01-01</p> <p>The publication reviews the structural characteristics of crystalline phases and phase equilibria data for the <span class="hlt">system</span> NaCl-KCl-<span class="hlt">Al</span>Cl/sub 3/-NaF-KF-<span class="hlt">Al</span>F/sub 3/, which encompasses a large number of molten salt fluxes currently used in aluminum recycling. Its purpose is to provide guidelines for research into the relationships between molten salt compositions and their physical properties, notably vapor pressures, densities, surface tensions, and viscosities, knowledge of which is essential to minimizing fluxing efficiencies and metal recovery and minimizing hazardous emissions and disposal problems. In addition, the report describes experimental determinations of sub-solidus compatibility relationships in the <span class="hlt">system</span>, which is of the quaternary reciprocal type, containing 12 different stable 4-phase assemblages in the solid state. The compatibility diagram serves to define the important compositional planes across which important changes in properties are likely to occur.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://ntrs.nasa.gov/search.jsp?R=19860029458&hterms=modulus+young&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D10%26Ntt%3Dmodulus%2Byoung','NASA-TRS'); return false;" href="http://ntrs.nasa.gov/search.jsp?R=19860029458&hterms=modulus+young&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D10%26Ntt%3Dmodulus%2Byoung"><span id="translatedtitle">Modulus measurements in ordered Co-<span class="hlt">Al</span>, Fe-<span class="hlt">Al</span>, and Ni-<span class="hlt">Al</span> alloys</span></a></p> <p><a target="_blank" href="http://ntrs.nasa.gov/search.jsp">NASA Technical Reports Server (NTRS)</a></p> <p>Harmouche, M. R.; Wolfenden, A.</p> <p>1985-01-01</p> <p>The composition and/or temperature dependence of the dynamic Young's modulus for the ordered B2 Co-<span class="hlt">Al</span>, Fe-<span class="hlt">Al</span>, and Ni-<span class="hlt">Al</span> aluminides has been investigated using the piezoelectric ultrasonic composite oscillator technique (PUCOT). The modulus has been measured in the composition interval 48.49 to 52.58 at. pct Co, 50.87 to 60.2 at. pct Fe, and 49.22 to 55.95 at. pct Ni for Co-<span class="hlt">Al</span>, Fe-<span class="hlt">Al</span>, and Ni-<span class="hlt">Al</span>, respectively. The measured values for Co-<span class="hlt">Al</span> are in the temperature interval 300 to 1300 K, while those for the other <span class="hlt">systems</span> are for ambient temperature only. The data points show that Co-<span class="hlt">Al</span> is stiffer than Fe-<span class="hlt">Al</span>, which is stiffer than Ni-<span class="hlt">Al</span>. The data points for Fe-<span class="hlt">Al</span> and Ni-<span class="hlt">Al</span> are slightly higher than those reported in the literature.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://hdl.handle.net/2060/19800023979','NASA-TRS'); return false;" href="http://hdl.handle.net/2060/19800023979"><span id="translatedtitle">Performance of two-layer thermal barrier <span class="hlt">systems</span> on directionally solidified Ni-<span class="hlt">Al</span>-Mo and comparative effects of alloy thermal expansion on <span class="hlt">system</span> life</span></a></p> <p><a target="_blank" href="http://ntrs.nasa.gov/search.jsp">NASA Technical Reports Server (NTRS)</a></p> <p>Stecura, S.</p> <p>1980-01-01</p> <p>A promising two-layer thermal barrier coating <span class="hlt">system</span> (TBS), Ni-16.4Cr-5.1A1-0.15Y/ZrO2-6.1Y2O3 (all in weight percent), was identified for directionally solidified Ni-<span class="hlt">Al</span>-Mo (gamma/gamma' alpha). In cyclic furnace tests at 1095 C this <span class="hlt">system</span> on gamma/gamma' alpha was better than Ni-16. 4Cr-5.1<span class="hlt">Al</span>-0.15Y/ZrO2-7.8Y2O3 by about 50 percent. In natural gas - oxygen torch rig tests at 1250 C the ZrO2-6.1Y2O3 coating was better than the ZrO2-7.8Y2O3 coating by 95 percent, on MAR-M509 substrates and by 60 percent on gamma/gamma' alpha substrates. Decreasing the coefficient of thermal expansion of the substrate material from 17-18x10 to the -6 power/C (MAR-M200 + Hf and MAR-M509) to 11x10 to the -6 power/C (gamma/gamma' alpha) also resulted in improved TBS life. For example, in natural gas - oxygen torch rig tests at 1250 C, the life of Ni-16.4Cr-5.1<span class="hlt">Al</span>-0.15Y/ZrO26.1Y2O3 was about 30 percent better on gamma/gamma' alpha than on MAR-M509 substrates. Thus compositional changes in the bond and thermal barrier coatings were shown to have a greater effect on TBS life than does the coefficient of thermal expansion.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/5304067','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/5304067"><span id="translatedtitle">The effect of pressure on the combustion synthesis of a functionally-graded material: TiB[sub 2]-<span class="hlt">Al</span>[sub 2]O[sub 3]-<span class="hlt">Al</span> ceramic-metal composite <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Feng, H.J.; Moore, J.J. . Dept. of Metallurgical and Materials Engineering)</p> <p>1993-10-01</p> <p>This article describes some recent research on the synthesis of functionally-graded materials (FGM) using combustion synthesis or self-propagating high-temperature synthesis (SHS). A model ceramic-metal SHS <span class="hlt">system</span> was investigated. The effects of combustion mode, reactant stoichiometry (x<span class="hlt">Al</span>), green density, and applied loads on the stability of the SHS reaction and density of the FGM product are described.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2015MMTA...46..726A','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2015MMTA...46..726A"><span id="translatedtitle">Kinetic Study of the Competitive Growth Between θ-<span class="hlt">Al</span>2O3 and α-<span class="hlt">Al</span>2O3 During the Early Stages of Oxidation of β-(Ni,Pt)<span class="hlt">Al</span> Bond Coat <span class="hlt">Systems</span>: Effects of Low Oxygen Partial Pressure and Temperature</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Alvarado-Orozco, J. M.; Morales-Estrella, R.; Boldrick, M. S.; Trapaga-Martinez, G.; Gleeson, B.; Munoz-Saldana, J.</p> <p>2015-02-01</p> <p>An oxidation study of β-(Ni,Pt)<span class="hlt">Al</span> commercial bond coat <span class="hlt">systems</span> was carried by means of TGA analysis during isothermal treatments at temperatures from 1273 K to 1423 K (1000 °C to 1150 °C). The effect of oxygen partial pressure on their oxidation kinetics was studied and complemented by photo-stimulated luminescence spectroscopy and SEM. Pre-oxidation treatments performed on as-aluminized samples at 10-5 atm O2 did not accelerate the θ-<span class="hlt">Al</span>2O3 → α-<span class="hlt">Al</span>2O3 transformation, even after 5 hours of oxidation relative to samples oxidized in 0.21 atm O2, with the exception of the sample treated at 1273 K (1000 °C), where apparently α-<span class="hlt">Al</span>2O3 nucleation started earlier for the sample treated at a low pO2.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2007NIMPB.259..625H','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2007NIMPB.259..625H"><span id="translatedtitle">Measurement of 26<span class="hlt">Al</span> in Antarctic ice with the MALT-AMS <span class="hlt">system</span> at the University of Tokyo</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Horiuchi, Kazuho; Matsuzaki, Hiroyuki; Ohta, Aoi; Shibata, Yasuyuki; Motoyama, Hideaki</p> <p>2007-06-01</p> <p>We have attempted to determine the 26<span class="hlt">Al</span> concentration of Antarctic ice sampled from the vicinity of the Dome Fuji Research Station using accelerator mass spectrometry (AMS) at MALT (MicroAnalysis Laboratory, Tandem accelerator) of the University of Tokyo. Because the expected concentration of 26<span class="hlt">Al</span> in ice is very low, our standard procedure for the AMS measurement was re-examined and refined. The observed 26<span class="hlt">Al</span> concentration ranged between 160 and 210 atoms g-1. The averaged value of the 26<span class="hlt">Al</span>/10Be ratio from two samples was 1.75 ± 0.19 × 10-3, which agrees well with recently reported values for the meteoric 26<span class="hlt">Al</span>/10Be ratio from Antarctic ice and air filter residues. This result implies the possibility of future 26<span class="hlt">Al</span>/10Be dating of old Antarctic ice.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.osti.gov/scitech/biblio/20637112','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/biblio/20637112"><span id="translatedtitle">A positron study of the defect structures in the D0{sub 3} and B2 phases in the Fe-<span class="hlt">Al</span> <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Diego, N. de . E-mail: nievesd@fis.ucm.es; Plazaola, F.; Jimenez, J.A.; Rio, J. del</p> <p>2005-01-03</p> <p>The positron annihilation technique was used to identify the nature of the vacancy-type defects in the D0{sub 3} and B2 phases of the Fe-<span class="hlt">Al</span> <span class="hlt">system</span>. Seven alloys with <span class="hlt">Al</span> concentrations in the range 22.7-48 at.% <span class="hlt">Al</span> and with different thermal treatments were examined. Positron lifetime calculations for the expected defects in the two phases were also performed in order to facilitate the defect identification. In the B2 phase, two types of defects were identified: a thermal complex formed by a Fe-divacancy and an <span class="hlt">Al</span> antisite, and a Fe-vacancy. No constitutional vacancies were found in the D0{sub 3} phase.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2012GeCoA..77..415B','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2012GeCoA..77..415B"><span id="translatedtitle">26<span class="hlt">Al</span>- 26Mg deficit dating ultramafic meteorites and silicate planetesimal differentiation in the early Solar <span class="hlt">System</span>?</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Baker, Joel A.; Schiller, Martin; Bizzarro, Martin</p> <p>2012-01-01</p> <p>Meteorites with significantly sub-chondritic <span class="hlt">Al</span>/Mg that formed in the first 2 million years of the Solar <span class="hlt">System</span> should be characterised by deficits in the abundance of 26Mg (δ26Mg∗) due to the absence of in-growth of 26Mg from the decay of short-lived 26<span class="hlt">Al</span> (t1/2 = 0.73 Myr). However, these 26Mg deficits will be small (δ26Mg∗ >-0.037‰) even for material that formed at the same time as the Solar System’s oldest solids - calcium-aluminium-rich inclusions - and thus measurement of these deficits is analytically challenging. Here, we report on a search for 26Mg deficits in three types of ultramafic meteorites (pallasites, ureilites and aubrites) by multiple-collector inductively coupled plasma mass spectrometry. A range of analytical tests were carried out including analysis of: (1) a range of synthetic Mg solution standards; (2) Mg gravimetrically doped with a high purity 26Mg spike; (3) Mg cuts collected sequentially from cation exchange separation columns with fractionated stable Mg isotope compositions; (4) Mg separated from samples that was bracketed by analyses of both DSM-3 and Mg separated from a natural olivine sample subjected to the same chemical processing as the samples. These tests confirm it is possible to resolve differences in δ26Mg∗ from the terrestrial materials that are ⩽0.005‰. However, if Mg yields from chemical separation are low or an inappropriate equilibrium-isotopically fractionated standard is used this will generate analytical artefacts on δ26Mg∗ when this is calculated with the kinetic/exponential mass fractionation law as is the case when correcting for instrumental mass bias during mass spectrometric analysis. Olivine from four different main group pallasites and four bulk ureilites have small deficits in the abundance of 26Mg with δ26MgDSM-3∗=-0.0120±0.0018‰ and δ26MgDSM-3∗=-0.0062±0.0023‰, respectively, relative to terrestrial olivine (δ26MgDSM-3∗=+0.0029±0.0028‰). Six aubrites have δ26MgDSM-3</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/25852389','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/25852389"><span id="translatedtitle">Investigation on the passivated Si/<span class="hlt">Al</span>2O3 interface fabricated by non-vacuum spatial atomic layer deposition <span class="hlt">system</span>.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Lien, Shui-Yang; Yang, Chih-Hsiang; Wu, Kuei-Ching; Kung, Chung-Yuan</p> <p>2015-01-01</p> <p>Currently, aluminum oxide stacked with silicon nitride (<span class="hlt">Al</span>2O3/SiNx:H) is a promising rear passivation material for high-efficiency P-type passivated emitter and rear cell (PERC). It has been indicated that atomic layer deposition <span class="hlt">system</span> (ALD) is much more suitable to prepare high-quality <span class="hlt">Al</span>2O3 films than plasma-enhanced chemical vapor deposition <span class="hlt">system</span> and other process techniques. In this study, an ultrafast, non-vacuum spatial ALD with the deposition rate of around 10 nm/min, developed by our group, is hired to deposit <span class="hlt">Al</span>2O3 films. Upon post-annealing for the <span class="hlt">Al</span>2O3 films, the unwanted delamination, regarded as blisters, was found by an optical microscope. This may lead to a worse contact within the Si/<span class="hlt">Al</span>2O3 interface, deteriorating the passivation quality. Thin stoichiometric silicon dioxide films prepared on the Si surface prior to <span class="hlt">Al</span>2O3 fabrication effectively reduce a considerable amount of blisters. The residual blisters can be further out-gassed when the <span class="hlt">Al</span>2O3 films are thinned to 8 nm and annealed above 650°C. Eventually, the entire PERC with the improved triple-layer SiO2/<span class="hlt">Al</span>2O3/SiNx:H stacked passivation film has an obvious gain in open-circuit voltage (V oc) and short-circuit current (J sc) because of the increased minority carrier lifetime and internal rear-side reflectance, respectively. The electrical performance of the optimized PERC with the V oc of 0.647 V, J sc of 38.2 mA/cm(2), fill factor of 0.776, and the efficiency of 19.18% can be achieved.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.ncbi.nlm.nih.gov/pubmed/21600538','PUBMED'); return false;" href="http://www.ncbi.nlm.nih.gov/pubmed/21600538"><span id="translatedtitle">Relation of clinical, echocardiographic and electrocardiographic features of cardiac <span class="hlt">amyloidosis</span> to the presence of the transthyretin V122I allele in older African-American men.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Jacobson, Daniel; Tagoe, Clement; Schwartzbard, Arthur; Shah, Alan; Koziol, James; Buxbaum, Joel</p> <p>2011-08-01</p> <p>Previous studies have shown that 3% to 4% of African Americans carry an amyloidogenic allele of the human serum protein transthyretin (TTR V122I). The allele appears to have an absolute anatomic risk for cardiac amyloid deposition after 65 years of age. In this study, a case-control comparison was performed of clinical, echocardiographic, and electrocardiographic characteristics of 23 age at risk carriers of the amyloidogenic allele and 46 age-, gender-, and ethnically matched noncarriers being evaluated for cardiac disease using standard clinical testing. The 2 groups were matched for blood pressure and the cardiac ejection fraction. None of the subjects had a prestudy diagnosis of cardiac <span class="hlt">amyloidosis</span>. Carriers of the amyloidogenic allele were found to have statistically significant increases in the occurrence of many of the echocardiographic features of cardiac <span class="hlt">amyloidosis</span> relative to the noncarriers and a higher frequency of congestive heart failure and atrial fibrillation. The observations suggest that TTR V122I represents a substantial risk for clinically significant cardiac <span class="hlt">amyloidosis</span> in elderly African American men, behaving as an age-dependent autosomal dominant disease-associated allele. The diagnosis is difficult to make but can be suspected in African Americans aged >60 years on the basis of age, echocardiographic evidence of diastolic dysfunction, and interventricular septal thickening, even in the absence of more recently available sophisticated echocardiographic techniques for evaluating long-axis function and cardiac magnetic resonance imaging. Positive results for the amyloidogenic TTR V122I allele support the diagnosis and define the origin of the disease, which can be confirmed by endomyocardial biopsy. PMID:21600538</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/cgi-bin/nph-data_query?bibcode=2015PhyC..519..168N&link_type=ABSTRACT','NASAADS'); return false;" href="http://adsabs.harvard.edu/cgi-bin/nph-data_query?bibcode=2015PhyC..519..168N&link_type=ABSTRACT"><span id="translatedtitle">Computational materials design of attractive Fermion <span class="hlt">system</span> with large negative effective Ueff in the hole-doped Delafossite of Cu<span class="hlt">Al</span>O2, Ag<span class="hlt">Al</span>O2 and Au<span class="hlt">Al</span>O2: Charge-excitation induced Ueff < 0</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Nakanishi, A.; Fukushima, T.; Uede, H.; Katayama-Yoshida, H.</p> <p>2015-12-01</p> <p>On the basis of general design rules for negative effective U(Ueff) <span class="hlt">systems</span> by controlling purely-electronic and attractive Fermion mechanisms, we perform computational materials design (CMD®) for the negative Ueff <span class="hlt">system</span> in hole-doped two-dimensional (2D) Delafossite Cu<span class="hlt">Al</span>O2, Ag<span class="hlt">Al</span>O2 and Au<span class="hlt">Al</span>O2 by ab initio calculations with local density approximation (LDA) and self-interaction corrected-LDA (SIC-LDA). It is found that the large negative Ueff in the hole-doped attractive Fermion <span class="hlt">systems</span> for Cu<span class="hlt">Al</span>O2 (UeffLDA = - 4.53 eV and UeffSIC-LDA = - 4.20 eV), Ag<span class="hlt">Al</span>O2 (UeffLDA = - 4.88 eV and UeffSIC-LDA = - 4.55 eV) and Au<span class="hlt">Al</span>O2 (UeffLDA = - 4.14 eV and UeffSIC-LDA = - 3.55 eV). These values are 10 times larger than that in hole-doped three-dimensional (3D) CuFeS2 (Ueff = - 0.44 eV). For future calculations of Tc and phase diagram by quantum Monte Carlo simulations, we propose the negative Ueff Hubbard model with the anti-bonding single π-band model for Cu<span class="hlt">Al</span>O2, Ag<span class="hlt">Al</span>O2 and Au<span class="hlt">Al</span>O2 using the mapped parameters obtained from ab initio electronic structure calculations. Based on the theory of negative Ueff Hubbard model (Noziéres and Schmitt-Rink, 1985), we discuss |Ueff| dependence of superconducting critical temperature (Tc) in the 2D Delafossite of Cu<span class="hlt">Al</span>O2, Ag<span class="hlt">Al</span>O2 and Au<span class="hlt">Al</span>O2 and 3D Chalcopyrite of CuFeS2, which shows the interesting chemical trend, i.e., Tc increases exponentially (Tc ∝ exp [ - 1 / | Ueff | ]) in the weak coupling regime | Ueff(- 0.44 eV) | < W(∼ 2 eV) (where W is the band width of the negative Ueff Hubbard model) for the hole-doped CuFeS2, and then Tc goes through a maximum when | Ueff(- 4.88 eV , - 4.14 eV) | ∼ W(2.8 eV , 3.5 eV) for the hole-doped Ag<span class="hlt">Al</span>O2 and Au<span class="hlt">Al</span>O2, and finally Tc decreases with increasing |Ueff| in the strong coupling regime, where | Ueff(- 4.53 eV) | > W(1.7 eV) , for the hole-doped Cu<span class="hlt">Al</span>O2.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016NatSR...635172X','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016NatSR...635172X"><span id="translatedtitle">Misfit Strain Relaxation of Ferroelectric PbTiO3/La<span class="hlt">Al</span>O3 (111) Thin Film <span class="hlt">System</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Xu, Y. B.; Tang, Y. L.; Zhu, Y. L.; Liu, Y.; Li, S.; Zhang, S. R.; Ma, X. L.</p> <p>2016-10-01</p> <p>Ferroelectric thin films grown on high index substrates show unusual structural and switching dynamics due to their special strain states. Understanding the misfit relaxation behavior is crucial to facilitate the high index thin film growth with improved quality. In this paper, ferroelectric PbTiO3 thin films were grown on La<span class="hlt">Al</span>O3 (111) substrates by pulsed laser deposition technique. The microstructures were investigated by combinations of conventional and aberration-corrected transmission electron microscopy. Diffraction contrast analysis and high resolution imaging reveal that high density interfacial dislocations were distributed at the interfaces. These dislocations have mixed character with Burgers vectors of a <110> and line directions of <112>. The edge components of the dislocations, with the Burgers vectors parallel to the interface, accommodate the lattice mismatch and are the main contributor to the misfit relaxation of this <span class="hlt">system</span>. The formation mechanism of these dislocations is proposed and discussed to elucidate the novel mismatch relaxation behavior of <111> oriented perovskite films.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.ncbi.nlm.nih.gov/pubmed/26965404','PUBMED'); return false;" href="http://www.ncbi.nlm.nih.gov/pubmed/26965404"><span id="translatedtitle">Landfill site selection using geographic information <span class="hlt">system</span> and analytical hierarchy process: A case study <span class="hlt">Al</span>-Hillah Qadhaa, Babylon, Iraq.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Chabuk, Ali; Al-Ansari, Nadhir; Hussain, Hussain Musa; Knutsson, Sven; Pusch, Roland</p> <p>2016-05-01</p> <p><span class="hlt">Al</span>-Hillah Qadhaa is located in the central part of Iraq. It covers an area of 908 km(2) with a total population of 856,804 inhabitants. This Qadhaa is the capital of Babylon Governorate. Presently, no landfill site exists in that area based on scientific site selection criteria. For this reason, an attempt has been carried out to find the best locations for landfills. A total of 15 variables were considered in this process (groundwater depth, rivers, soil types, agricultural land use, land use, elevation, slope, gas pipelines, oil pipelines, power lines, roads, railways, urban centres, villages and archaeological sites) using a geographic information <span class="hlt">system</span>. In addition, an analytical hierarchy process was used to identify the weight for each variable. Two suitable candidate landfill sites were determined that fulfil the requirements with an area of 9.153 km(2) and 8.204 km(2) These sites can accommodate solid waste till 2030. PMID:26965404</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/cgi-bin/nph-data_query?bibcode=2014APS..MARA30009D&link_type=ABSTRACT','NASAADS'); return false;" href="http://adsabs.harvard.edu/cgi-bin/nph-data_query?bibcode=2014APS..MARA30009D&link_type=ABSTRACT"><span id="translatedtitle">Characterization of the plasmon mode of graphene/La<span class="hlt">Al</span>O3/SrTiO3 <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Dai, Weitao; Ryu, Sangwoo; Eom, Chang-Beom; Cen, Cheng</p> <p>2014-03-01</p> <p>Engineering graphene's properties in nanoscale with minimum material degradation is an outstanding challenge in graphene based technologies. Here we present a method targeting at on-demand tuning of 2D plasmon in graphene based on the integration of graphene and a novel complex oxide heterostructure. The recent development of complex oxides has raised the prospect for new classes of electronic devices. In particular, researchers have discovered a high-mobility two-dimensional electron gas forming at the interface between La<span class="hlt">Al</span>O3 (LAO) and SrTiO3 (STO). More interestingly, in samples with 3-unit-cell LAO film grown on STO substrate, a biased conducting atomic force microscope probe can locally and reversibly controls the interfacial metal-insulator transition. The close coupling of graphene with these programmable interfacial nanostructures in graphene/LAO/STO heterostructures presents numerous device opportunities. Samples with contacts addressing graphene and oxide interface separately are fabricated. Transport experiments are performed to study the carrier coupling in such hybrid bilayer conducting <span class="hlt">system</span>. We also report the investigation of plasmonic properties using a variable temperature near field scanning optical microscope.</p> </li> </ol> <div class="pull-right"> <ul class="pagination"> <li><a href="#" onclick='return showDiv("page_1");'>«</a></li> <li><a href="#" onclick='return showDiv("page_21");'>21</a></li> <li><a href="#" onclick='return showDiv("page_22");'>22</a></li> <li><a href="#" onclick='return showDiv("page_23");'>23</a></li> <li><a href="#" onclick='return showDiv("page_24");'>24</a></li> <li class="active"><span>25</span></li> <li><a href="#" onclick='return showDiv("page_25");'>»</a></li> </ul> </div> </div><!-- col-sm-12 --> </div><!-- row --> </div><!-- page_25 --> <center> <div class="footer-extlink text-muted"><small>Some links on this page may take you to non-federal websites. 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