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Sample records for systemic al amyloidosis

  1. AL Amyloidosis

    PubMed Central

    2012-01-01

    Definition of the disease AL amyloidosis results from extra-cellular deposition of fibril-forming monoclonal immunoglobulin (Ig) light chains (LC) (most commonly of lambda isotype) usually secreted by a small plasma cell clone. Most patients have evidence of isolated monoclonal gammopathy or smoldering myeloma, and the occurrence of AL amyloidosis in patients with symptomatic multiple myeloma or other B-cell lymphoproliferative disorders is unusual. The key event in the development of AL amyloidosis is the change in the secondary or tertiary structure of an abnormal monoclonal LC, which results in instable conformation. This conformational change is responsible for abnormal folding of the LC, rich in β leaves, which assemble into monomers that stack together to form amyloid fibrils. Epidemiology AL amyloidosis is the most common type of systemic amyloidois in developed countries with an estimated incidence of 9 cases/million inhabitant/year. The average age of diagnosed patients is 65 years and less than 10% of patients are under 50. Clinical description The clinical presentation is protean, because of the wide number of tissues or organs that may be affected. The most common presenting symptoms are asthenia and dyspnoea, which are poorly specific and may account for delayed diagnosis. Renal manifestations are the most frequent, affecting two thirds of patients at presentation. They are characterized by heavy proteinuria, with nephrotic syndrome and impaired renal function in half of the patients. Heart involvement, which is present at diagnosis in more than 50% of patients, leading to restrictive cardiopathy, is the most serious complication and engages prognosis. Diagnostic methods The diagnosis relies on pathological examination of an involved site showing Congo red-positive amyloid deposits, with typical apple-green birefringence under polarized light, that stain positive with an anti-LC antibody by immunohistochemistry and/or immunofluorescence. Due to the

  2. 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.

  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. Disease burden of systemic light-chain (AL) amyloidosis: a systematic literature review.

    PubMed

    Lin, Huamao Mark; Gao, Xin; Cooke, Catherine E; Berg, Deborah; Labotka, Richard; Faller, Douglas V; Seal, Brian; Hari, Parameswaran

    2017-02-21

    A systematic literature review on AL amyloidosis was conducted in order to understand the disease burden, and identify unmet medical needs and knowledge gaps. MEDLINE, EMBASE and COCHRANE databases were searched for English language studies published in the last 10 years using search terms that focused on the clinical, economic, and patient-reported outcome (PRO) aspects of AL amyloidosis. There was a low yield of articles in the economic and PRO categories and additional searches were conducted in clinical conference proceedings, and using Google and Google Scholar. After review, there were 65 articles included for data extraction. AL amyloidosis is a rare disorder without any FDA or EMA approved indications for drug therapy. Using off-label therapies, there is a high rate, 42-64%, of non-response or progression, and an associated high mortality. Toxicities during therapy are common with estimates of up to 30-40% of patients experiencing severity of grade 3 or higher. Patients with AL amyloidosis report severe psychological distress, anxiety and clinical depression. There is a deficiency in the literature on the economic costs associated with AL amyloidosis, and information on costs has been derived from studies that examined multiple myeloma or other disease or treatment components common to AL amyloidosis.

  6. Exceptional oral manifestations of amyloid light chain protein (AL) systemic amyloidosis.

    PubMed

    Elad, Sharon; Czerninski, Rakefet; Fischman, Stuart; Keshet, Na'ama; Drucker, Scott; Davidovich, Tzipporah; Goldschmidt, Neta

    2010-03-01

    Oral amyloidosis is usually presented in the tongue and is often associated with multiple myeloma. We present three patients with unusual oral manifestations of primary amyloidosis, which to the best of our knowledge have not been previously published. In two cases the oral manifestation was overt at the time of diagnosis and all cases ended in patient mortality. Since these oral manifestations can contribute to the diagnosis of systemic amyloidosis, clinicians should be made aware of them. Future research should assess the significance of oral manifestation as a prognostic indicator.

  7. Concomitant diagnosis of primary Sjögren's syndrome and systemic AL amyloidosis.

    PubMed

    Delèvaux, I; André, M; Amoura, Z; Kémény, J L; Piette, J C; Aumaître, O

    2001-07-01

    A 48 year old woman was referred to hospital for buccal discomfort. Physical examination showed a macroglossia and features of xerostomia. She was diagnosed as having primary Sjögren's syndrome according to the criteria proposed by the European Community study group in 1993. Furthermore, a lower lip salivary gland biopsy showed amyloid deposits that were also seen in the stomach and in the bone marrow. Echocardiography was consistent with cardiac amyloidosis. Serum immunofixation identified a monoclonal IgGlambda. As far as is known, this is the first report of systemic primary amyloidosis associated with Sjögren's syndrome. The relation between these two disorders is discussed.

  8. 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

  9. Amyloidosis

    MedlinePlus

    ... your tissues as amyloid, interfering with normal function. AA amyloidosis mostly affects your kidneys but occasionally your ... infectious or inflammatory disease increases your risk of AA amyloidosis. Family history. Some types of amyloidosis are ...

  10. 75 FR 65279 - Schedule for Rating Disabilities; AL Amyloidosis (Primary Amyloidosis)

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-10-22

    ... presumptive service connection based on herbicide exposure for this disease. The intended effects are to... adding AL amyloidosis to the list of diseases associated with exposure to certain herbicide...

  11. Acquired factor X deficiency developed four years after autologous transplantation in a patient with multiple myeloma associated with systemic AL amyloidosis.

    PubMed

    Takemura, Tomonari; Fukatsu, Yusuke; Nagata, Yasuyuki; Asahina, Aya; Yokota, Daisuke; Hirano, Isao; Yagyu, Tomohiro; Ono, Takaaki; Katsumi, Akira; Ohnishi, Kazunori

    2014-05-01

    We describe a case of acquired factor X deficiency after high-dose melphalan with autologous stem cell transplantation (HDM/ASCT) for multiple myeloma (MM) with systemic AL amyloidosis. A 68-year-old woman with renal amyloidosis was diagnosed as having MM in 2007. She achieved a partial response after VAD (vincristine, adriamycin, dexamethasone) therapy and HDM/ASCT. In December 2011, coagulation tests revealed a prolonged prothrombin time (PT) of 17.6 sec and she was administered vitamin K. In January 2012, she received low anterior resection with colostomy for rectal cancer. She received fresh frozen plasma (FFP) infusion but the perioperative bleeding tendency persisted. In February 2012, she was referred from surgery for colostomy closure. She showed no progression of MM and had prolonged PT, corrected by mixing with normal plasma. Factor X activity was markedly decreased. She was diagnosed as having an acquired factor X deficiency and was given FFP infusion for colostomy closure. Although acquired factor X deficiency after HDM/ASCT for MM with systemic AL amyloidosis is rare, we should be aware of the possibility of this disease in MM patients with a bleeding tendency.

  12. Systemic Light Chain Amyloidosis Mimicking Rheumatic Disorders

    PubMed Central

    2016-01-01

    Secondary amyloidosis can complicate chronic inflammatory autoimmune diseases. However, the clinical findings of primary amyloidosis may mimic those of primary rheumatologic disorders. We present the case of a 53-year-old woman who presented with dystrophic nail changes, dry eyes, bilateral carpal tunnel syndrome, Raynaud's phenomenon, and high titer positive nucleolar pattern antinuclear antibody. She was initially misdiagnosed as having Undifferentiated Connective Tissue Disease (UCTD). On further workup, she was eventually diagnosed with lambda light chain systemic amyloidosis by abdominal fat pad biopsy. Her symptoms completely resolved after autologous stem cell transplantation. With this case, we would like to highlight the similarities in the clinical features between light chain amyloidosis and rheumatological disorders. We would also like to emphasize the importance of the prompt recognition of the clinical features of amyloidosis which are crucial to triggering appropriate diagnostic procedures, since early diagnosis is a key to improving outcomes in this disease with an otherwise poor prognosis. PMID:28042297

  13. Secondary systemic amyloidosis

    MedlinePlus

    ... Reactive arthritis Respiratory Rheumatoid arthritis Sjögren syndrome Systemic lupus erythematosus Review Date 5/3/2015 Updated by: Laura J. Martin, MD, MPH, ABIM Board Certified in Internal Medicine and Hospice and Palliative Medicine. Also reviewed by ...

  14. Primary systemic amyloidosis presenting as constrictive pericarditis.

    PubMed

    Singh, Vikas; Fishman, Joel E; Alfonso, Carlos E

    2011-01-01

    The most frequent presentation of cardiac amyloidosis is with endomyocardial deposition, and resultant restrictive cardiomyopathy. We present a case of primary systemic amyloidosis causing constrictive pericarditis (CP) and congestive heart failure without clinical evidence of endomyocardial deposition. A comprehensive evaluation by noninvasive and invasive studies facilitated the differentiation of CP from restrictive cardiomyopathy and the patient was effectively treated with pericardectomy. To our knowledge, this is the first documented case of primary systemic amyloidosis causing selective CP with successful antemortem diagnosis and treatment in a young man.

  15. Autoimmune disease leading to pulmonary AL amyloidosis and pulmonary hypertension

    PubMed Central

    Ellender, Claire M; McLean, Catriona; Williams, Trevor J; Snell, Gregory I; Whitford, Helen M

    2015-01-01

    A 33-year-old woman with past history of Sjögren's syndrome and systemic lupus erythematosus presented with dyspnea and syncope secondary to pulmonary hypertension. After progressive symptoms over 4 years, she received bilateral lung transplantation. Histopathology of the explanted lungs showed isolated pulmonary amyloid light-chain amyloidosis and pulmonary cysts. No evidence of systemic amyloidosis was found at the time of transplantation. Seven years post lung transplantation, she remains well with no evidence of systemic amyloidosis recurrence. PMID:26090118

  16. Nutritional status independently affects quality of life of patients with systemic immunoglobulin light-chain (AL) amyloidosis.

    PubMed

    Caccialanza, Riccardo; Palladini, Giovanni; Klersy, Catherine; Cereda, Emanuele; Bonardi, Chiara; Cameletti, Barbara; Montagna, Elisabetta; Russo, Paola; Foli, Andrea; Milani, Paolo; Lavatelli, Francesca; Merlini, Giampaolo

    2012-03-01

    Nutritional status is an independent prognostic factor in immunoglobulin light-chain amyloidosis (AL), but its influence on quality of life (QoL) is unknown. The aim of this cross-sectional study was to investigate the association between nutritional status and QoL in AL patients at diagnosis. One hundred and fifty consecutive patients with biopsy-proven AL were assessed for nutritional status by anthropometry [body mass index, unintentional weight loss (WL) in the previous 6 months and mid-arm muscle circumference (MAMC)], biochemistry (serum prealbumin), and semiquantitative food intake at referral. QoL was assessed by the Medical Outcomes Study 36-item Short Form General Health Survey. The composite physical component summary (PCS) and the mental component summary (MCS) for AL outpatients were 36.2 ± 10.1 and 44.9 ± 11.3, respectively (p < 0.001 for both vs the population norms of 50). In multivariate linear regression models adjusted for gender, age, Eastern Cooperative Oncology Group performance status, the number of organs involved, the severity of cardiac damage, C-reactive protein, energy intake, and WL, PCS was significantly lower for serum prealbumin <200 mg/L and MAMC <10th percentile (adjusted difference 3.8, 95% CI 0.18-7.5, p = 0.040 and 5.3, 95% CI 2.0-8.7, p = 0.002, respectively). MCS was decreased by 0.47 (95% CI 0.18-0.75, p = 0.002) for each kilogram of body weight lost in the previous 6 months. Nutritional status independently affects QoL in AL patients since diagnosis. Nutritional evaluation should be integral part of the clinical assessment of AL patients. Nutritional support intervention trials are warranted in such patients' population.

  17. Light chain (AL) amyloidosis: update on diagnosis and management

    PubMed Central

    2011-01-01

    Light chain (AL) amyloidosis is a plasma cell dyscrasia characterized by the pathologic production of fibrillar proteins comprised of monoclonal light chains which deposit in tissues and cause organ dysfunction. The diagnosis can be challenging, requiring a biopsy and often specialized testing to confirm the subtype of systemic disease. The goal of treatment is eradication of the monoclonal plasma cell population and suppression of the pathologic light chains which can result in organ improvement and extend patient survival. Standard treatment approaches include high dose melphalan (HDM) followed by autologous hematopoietic stem cell transplantation (SCT) or oral melphalan with dexamethasone (MDex). The use of novel agents (thalidomide, lenalidomide and bortezomib) alone and in combination with steroids and alkylating agents has shown efficacy and continues to be explored. A risk adapted approach to SCT followed by novel agents as consolidation reduces treatment related mortality with promising outcomes. Immunotherapeutic approaches targeting pathologic plasma cells and amyloid precursor proteins or fibrils are being developed. Referral of patients to specialized centers focusing on AL amyloidosis and conducting clinical trials is essential to improving patient outcomes. PMID:22100031

  18. 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

  19. Bendamustine-Induced Nephrogenic Diabetes Insipidus in a Patient With AL Amyloidosis.

    PubMed

    Uwumugambi, Nsabimana A; Sanchorawala, Vaishali; Shelton, Anthony C; Stern, Lauren; Gordon, Craig E

    2017-02-01

    Nephrogenic diabetes insipidus is a condition characterized by polyuria with dilute urine due to the inability of the principal cells of the renal collecting ducts to respond to antidiuretic hormone and concentrate urine. Nephrogenic diabetes insipidus can be drug induced, and several chemotherapeutic agents have been reported to cause it. Bendamustine is a traditional chemotherapeutic agent being studied for treatment for relapsed systemic AL amyloidosis. We report a case of a 59-year-old man with AL amyloidosis who developed partial nephrogenic diabetes insipidus after receiving bendamustine for treatment of AL amyloidosis. The nephrogenic diabetes insipidus responded well to sodium restriction, hydrochlorothiazide, and desmopressin treatment, allowing the patient to receive subsequent bendamustine cycles without polyuria. Nephrogenic diabetes insipidus resolved shortly after completion of bendamustine therapy.

  20. CVID Associated with Systemic Amyloidosis

    PubMed Central

    Esenboga, Saliha; Çagdas Ayvaz, Deniz; Saglam Ayhan, Arzu; Peynircioglu, Banu; Sanal, Ozden; Tezcan, Ilhan

    2015-01-01

    Common variable immunodeficiency (CVID) is a frequent primary immune deficiency (PID), which consists of a heterogeneous group of disorders and can present with recurrent infections, chronic diarrhea, autoimmunity, chronic pulmonary and gastrointestinal diseases, and malignancy. Secondary amyloidosis is an uncommon complication of CVID. We report an unusual case of a 27-year-old male patient who presented with recurrent sinopulmonary infections, chronic diarrhea, and hypogammaglobulinemia and was diagnosed with CVID. The patient was treated with intravenous immunoglobulin (IVIg) therapy once every 21 days and daily trimethoprim-sulfamethoxazole for prophylaxis. Two years after initial diagnosis, the patient was found to have progressive decline in IgG levels (as low as 200–300 mg/dL) despite regular Ig infusions. The laboratory tests revealed massive proteinuria and his kidney biopsy showed accumulation of AA type amyloid. We believe that the delay in the diagnosis of CVID and initiation of Ig replacement therapy caused chronic inflammation due to recurrent infections in our patient and this led to an uncommon and life-threatening complication, amyloidosis. Patients with CVID require regular follow-up for the control of infections and assessment of adequacy of Ig replacement therapy. Amyloidosis should be kept in the differential diagnosis when managing patients with CVID. PMID:26346511

  1. Life-saving implantable cardioverter defibrillator therapy in cardiac AL amyloidosis

    PubMed Central

    Patel, Ketna S; Hawkins, Philip N; Whelan, Carol J; Gillmore, Julian D

    2014-01-01

    Cardiac involvement is the main determinant of prognosis in systemic monoclonal immunoglobulin light chain (AL) amyloidosis. Ventricular arrhythmias and sudden cardiac death are not uncommon. The electrical events that precede sudden death, and their potential to be treated effectively, remain undefined. There are no European guidelines for the use of implantable cardioverter defibrillator (ICD) in amyloidosis. ICDs in general are not usually offered to patients with a life expectancy of less than 1 year. We describe a patient who presented with cardiac AL amyloidosis who underwent prophylactic ICD implantation for the prevention of sudden cardiac death during treatment with chemotherapy, in whom life-threatening ventricular arrhythmia was successfully terminated over a 3-year period. PMID:25535224

  2. Uninvolved immunoglobulins predicting hematological response in newly diagnosed AL amyloidosis.

    PubMed

    Muchtar, Eli; Magen, Hila; Itchaki, Gilad; Cohen, Amos; Rosenfeld, Ra'ama; Shochat, Tzippy; Kornowski, Ran; Iakobishvili, Zaza; Raanani, Pia

    2016-02-01

    Immunoparesis serves as a marker for elevated risk for progression in plasma cell proliferative disorders. However, the impact of immunoparesis in AL amyloidosis has not been addressed. Immunoparesis was defined qualitatively as any decrease below the low reference levels of the uninvolved immunoglobulins and quantitatively, as the relative difference between the uninvolved immunoglobulins and the lower reference values. Forty-one newly diagnosed AL amyloidosis patients were included. Sixty-six percent of patients had a suppression of the uninvolved immunoglobulins. The median relative difference of the uninvolved immunoglobulins was 18% above the low reference levels [range (-71%)-210%]. Ninety percent of the patients were treated with novel agents-based regimens, mostly bortezomib-containing regimens. Nineteen percent of the patients did not attain response to first line treatment. Patients with relative difference of uninvolved immunoglobulins below -25% of the low reference levels were less likely to respond to first line treatment compared to patients with a relative difference of -25% and above [odds ratio for no response vs. partial response and better 30 [(95% CI 4.1-222.2), P=0.0004]. Patients who failed first line treatment were successfully salvaged with lenalidomide-based treatment. Immunoparesis, if assessed quantitatively, may serve as a predictor of response in AL amyloidosis patients treated with bortezomib-containing regimens.

  3. Aggregation of Full-length Immunoglobulin Light Chains from Systemic Light Chain Amyloidosis (AL) Patients Is Remodeled by Epigallocatechin-3-gallate.

    PubMed

    Andrich, Kathrin; Hegenbart, Ute; Kimmich, Christoph; Kedia, Niraja; Bergen, H Robert; Schönland, Stefan; Wanker, Erich; Bieschke, Jan

    2017-02-10

    Intervention into amyloid deposition with anti-amyloid agents like the polyphenol epigallocatechin-3-gallate (EGCG) is emerging as an experimental secondary treatment strategy in systemic light chain amyloidosis (AL). In both AL and multiple myeloma (MM), soluble immunoglobulin light chains (LC) are produced by clonal plasma cells, but only in AL do they form amyloid deposits in vivo We investigated the amyloid formation of patient-derived LC and their susceptibility to EGCG in vitro to probe commonalities and systematic differences in their assembly mechanisms. We isolated nine LC from the urine of AL and MM patients. We quantified their thermodynamic stabilities and monitored their aggregation under physiological conditions by thioflavin T fluorescence, light scattering, SDS stability, and atomic force microscopy. LC from all patients formed amyloid-like aggregates, albeit with individually different kinetics. LC existed as dimers, ∼50% of which were linked by disulfide bridges. Our results suggest that cleavage into LC monomers is required for efficient amyloid formation. The kinetics of AL LC displayed a transition point in concentration dependence, which MM LC lacked. The lack of concentration dependence of MM LC aggregation kinetics suggests that conformational change of the light chain is rate-limiting for these proteins. Aggregation kinetics displayed two distinct phases, which corresponded to the formation of oligomers and amyloid fibrils, respectively. EGCG specifically inhibited the second aggregation phase and induced the formation of SDS-stable, non-amyloid LC aggregates. Our data suggest that EGCG intervention does not depend on the individual LC sequence and is similar to the mechanism observed for amyloid-β and α-synuclein.

  4. 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.

  5. 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

  6. Long-term outcomes of primary systemic light chain (AL) amyloidosis in patients treated upfront with bortezomib or lenalidomide and the importance of risk adapted strategies.

    PubMed

    Kastritis, Efstathios; Roussou, Maria; Gavriatopoulou, Maria; Migkou, Magdalini; Kalapanida, Despina; Pamboucas, Constantinos; Kaldara, Elisavet; Ntalianis, Argyrios; Psimenou, Erasmia; Toumanidis, Savvas T; Tasidou, Anna; Terpos, Evangelos; Dimopoulos, Meletios A

    2015-04-01

    Bortezomib and lenalidomide are increasingly used in patients with AL amyloidosis, but long term data on their use as primary therapy in AL amyloidosis are lacking while early mortality remains significant. Thus, we analyzed the long term outcomes of 85 consecutive unselected patients, which received primary therapy with bortezomib or lenalidomide and we prospectively evaluated a risk adapted strategy based on bortezomib/dexamethasone to reduce early mortality. Twenty-six patients received full-dose bortezomib/dexamethasone, 36 patients lenalidomide with oral cyclophosphamide and low-dose dexamethasone and 23 patients received bortezomib/dexamethasone at a dose and schedule adjusted to the risk of early death. On intent to treat, 67% of patients achieved a hematologic response (24% hemCRs) and 34% an organ response; both were more frequent with bortezomib. An early death occurred in 20%: in 36% of those treated with full-dose bortezomib/dexamethasone, in 22% of lenalidomide-treated patients but only in 4.5% of patients treated with risk-adapted bortezomib/dexamethasone. Activity of full vs. adjusted dose bortezomib/dexamethasone was similar; twice weekly vs. weekly administration of bortezomib also had similar activity. After a median follow up of 57 months, median survival is 47 months and is similar for patients treated with bortezomib vs. lenalidomide-based regimens. However, risk adjusted-bortezomib/dexamethasone was associated with improved 1-year survival vs. full-dose bortezomib/dexamethasone or lenalidomide-based therapy (81% vs. 56% vs. 53%, respectively). In conclusion, risk-adapted bortezomib/dexamethasone may reduce early mortality and preserve activity while long term follow up indicates that remissions obtained with lenalidomide or bortezomib may be durable, even without consolidation with alkylators.

  7. Simultaneous presentation of kappa-restricted chronic lymphocytic leukemia and lambda light chain AL amyloidosis.

    PubMed

    von Keudell, Gottfried; Sanchorawala, Vaishali; O'Hara, Carl; C Seldin, David; Sloan, J Mark

    2014-06-01

    We report on a 58-year-old man who presented with simultaneous kappa-restricted chronic lymphocytic leukemia (CLL) and a lambda-restricted plasma cell dyscrasia causing AL amyloidosis involving the kidney and GI tract. While monoclonal immunoglobulins occasionally produced by CLL has previously been implicated in AL amyloidosis, this is the first case of AL amyloidosis resulting from a distinct plasma cell dyscrasia that is not clonally related to the concurrent CLL. Appropriate treatment depended on detailed pathologic diagnosis of both disease processes.

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

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-02-08

    ... herbicide exposure for this disease. The intended effects are to provide consistency in disability ratings... presumptive service connection based on herbicide exposure for this disease. A final rule regarding that... amyloidosis to the list of diseases associated with exposure to certain herbicide agents. For these...

  10. Doxycycline reduces fibril formation in a transgenic mouse model of AL amyloidosis

    PubMed Central

    Ward, Jennifer Ellis; Ren, Ruiyi; Toraldo, Gianluca; SooHoo, Pam; Guan, Jian; O'Hara, Carl; Jasuja, Ravi; Trinkaus-Randall, Vickery; Liao, Ronglih; Connors, Lawreen H.

    2011-01-01

    Systemic AL amyloidosis results from the aggregation of an amyloidogenic immunoglobulin (Ig) light chain (LC) usually produced by a plasma cell clone in the bone marrow. AL is the most rapidly fatal of the systemic amyloidoses, as amyloid fibrils can rapidly accumulate in tissues including the heart, kidneys, autonomic or peripheral nervous systems, gastrointestinal tract, and liver. Chemotherapy is used to eradicate the cellular source of the amyloidogenic precursor. Currently, there are no therapies that target the process of LC aggregation, fibril formation, or organ damage. We developed transgenic mice expressing an amyloidogenic λ6 LC using the cytomegalovirus (CMV) promoter to circumvent the disruption of B cell development by premature expression of recombined LC. The CMV-λ6 transgenic mice develop neurologic dysfunction and Congophilic amyloid deposits in the stomach. Amyloid deposition was inhibited in vivo by the antibiotic doxycycline. In vitro studies demonstrated that doxycycline directly disrupted the formation of recombinant LC fibrils. Furthermore, treatment of ex vivo LC amyloid fibrils with doxycycline reduced the number of intact fibrils and led to the formation of large disordered aggregates. The CMV-λ6 transgenic model replicates the process of AL amyloidosis and is useful for testing the antifibril potential of orally available agents. PMID:21998211

  11. [Systemic amyloidosis associated with IgD-λ multiple myeloma].

    PubMed

    Nagamachi, Yasuhiro; Yamauchi, Naofumi; Muramatsu, Hirohito; Inomata, Hidetoshi; Nozawa, Eri; Koyama, Ryuzo; Ihara, Koji; Nishisato, Takuji; Yamada, Hideyuki; Yano, Tomohiro; Kikuchi, Shohei; Hirako, Tasuku; Kitaoka, Keisuke; Ono, Kaoru; Ihara, Hideyuki; Kato, Junji

    2011-12-01

    We describe here a case of systemic amyloidosis associated with IgD multiple myeloma. A 59-year-old man was admitted to our hospital in April 2009, because of macroglossia and swelling in both wrists and fingers. He had difficulty moving his limbs and was aware of peripheral neuropathy. Skin biopsy revealed extensive deposition of amyloidosis, which was positive by Congo red staining. Laboratory findings were as follows: serum electrophoresis revealed IgD λ monoclonal protein, and Bence-Jones protein was detected. Monoclonal IgD protein had a concentration of 727 mg/dl, and a bone marrow aspiration revealed 49.6% of plasma cells. These findings led to a diagnosis of IgD multiple myeloma with systemic amyloidosis. The patient was treated with MP (melphalan and methylprednisolone), high-dose dexamethasone and VAD therapy (vincristine, adriamycin and dexamethasone), but systemic amyloidosis progressed, and his general condition deteriorated. Coexistence of IgD multiple myeloma and systemic amyloidosis is rare, and accumulation of case reports is needed to gain a better understanding of this condition.

  12. 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.

  13. Evaluation of systemic amyloidosis by scintigraphy with sup 123 I-labeled serum amyloid P component

    SciTech Connect

    Hawkins, P.N.; Lavender, J.P.; Pepys, M.B. )

    1990-08-23

    In systemic amyloidosis the distribution and progression of disease have been difficult to monitor, because they can be demonstrated only by biopsy. Serum amyloid P component (SAP) is a normal circulating plasma protein that is deposited on amyloid fibrils because of its specific binding affinity for them. We investigated whether labeled SAP could be used to locate amyloid deposits. Purified human SAP labeled with iodine-123 was given intravenously to 50 patients with biopsy-proved systemic amyloidosis--25 with the AL (primary) type and 25 with the AA (secondary) type--and to 26 control patients with disease and 10 healthy subjects. Whole-body images and regional views were obtained after 24 hours and read in a blinded fashion. In the patients with amyloidosis the 123I-SAP was localized rapidly and specifically in amyloid deposits. The scintigraphic images obtained were characteristic and appeared to identify the extent of amyloid deposition in all 50 patients. There was no uptake of the 123I-SAP by the control patients and the healthy subjects. In all patients with AA amyloidosis the spleen was affected, whereas the scans showed uptake in the heart, skin, carpal region, and bone marrow only in patients with the AL type. Positive images were seen in six patients in whom biopsies had been negative or unsuccessful; in all six, amyloid was subsequently found on biopsy or at autopsy. Progressive amyloid deposition was observed in 9 of 11 patients studied serially. Scintigraphy after the injection of 123I-SAP can be used for diagnosing, locating, and monitoring the extent of systemic amyloidosis.

  14. [New advances in the subtyping of systemic amyloidosis].

    PubMed

    Sun, Wei-Yi; Li, Jian

    2014-02-01

    Amyloidosis is a heterogeneous group of diseases caused by deposition of misfolded proteins, which usually leads to organ dysfunction. Accurate typing of amyloid deposits is of paramount importance because organ involvements and disease prognosis differ widely among different subtypes, and its treatments are type specific. Correct identification of amyloidogenic protein is crucial to proper treatment. Traditional antibody-based diagnostic methods such as immunohistochemistry and immunofluorescence are helpful in amyloid typing, but limitations of those approaches including antibody availability and serum protein contamination impair sensitivity and specificity of diagnosis. Sometimes misdiagnosis can lead to catastrophic therapeutic outcome. Genetic testing is important to confirm the diagnosis of hereditary amyloidosis. Nowadays proteomic analysis has been used as an advanced strategy for amyloid typing and the gold-standard today is laser microdissection followed by mass spectrometry (LMD/MS), which can identify causal protein without additional clinical information. Furthermore, LMD/MS is performed on formalin-fixed paraffin-embedded (FFPE) specimens, thus large scale retrospective studies based on archival material can be conducted. In recent studies, LMD/MS has been proven superior to traditional methods without the drawbacks mentioned above. This proteomic approach provides guarantee of appropriate clinical management and probability of new insights into the mechanism of amyloidosis.In this article the new advances of studies on subtyping of systemic amyloidosis are reviewed.

  15. [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.

  16. Renal Outcomes in Patients with AL Amyloidosis: Prognostic Factors, Renal response and the Impact of Therapy.

    PubMed

    Kastritis, Efstathios; Gavriatopoulou, Maria; Roussou, Maria; Migkou, Magdalini; Fotiou, Despina; Ziogas, Dimitrios C; Kanellias, Nikos; Eleutherakis-Papaiakovou, Evangelos; Panagiotidis, Ioannis; Giannouli, Stavroula; Psimenou, Erasmia; Marinaki, Smaragdi; Apostolou, Theofanis; Gakiopoulou, Hariklia; Tasidou, Anna; Papassotiriou, Ioannis; Terpos, Evangelos; Dimopoulos, Meletios A

    2017-03-28

    A staging system for patients with renal AL amyloidosis, based on eGFR (<50 ml/min/1.73 m(2) ) and proteinuria (≥5gr/day) at diagnosis, as well as criteria for renal progression (≥25% eGFR reduction) and response (≥30% reduction of proteinuria without renal progression) were recently proposed. We validated these criteria in a cohort of 125 patients with renal AL amyloidosis, mostly treated with bortezomib or lenalidomide. We confirmed the prognostic value of the renal staging system but also identified the limitations of renal progression criteria which are based only on eGFR reduction. We identified the ratio of 24h proteinuria to eGFR as a sensitive marker of renal risk which also accounts for changes in both proteinuria and eGFR: 24h proteinuria/eGFR ratio <30 (in mg/ml/min/1.73 m(2) ) was associated with a 2-year progression to dialysis rate of 0% compared to 9% for a ratio of 31-99 and 35% for a ratio ≥100 (p<0.001). In landmark analysis, patients who achieved a reduction of this ratio by at least 25% or ≤100 (if initially >100) at 3 months had a 2-year progression to dialysis of 0% vs 24% for patients who either did not reduce to or still had a ratio >100 (p=0.001); similar results were obtained by applying the same criteria at 6 months; thus, the evaluation of treatment effect on renal function may be identified early. Furthermore, primary bortezomib-based therapy was more effective than lenalidomide-based therapy, in terms of renal outcomes, especially in patients at intermediate renal risk, but without affecting overall survival. This article is protected by copyright. All rights reserved.

  17. Assessment of renal response with urinary exosomes in patients with AL amyloidosis: A proof of concept.

    PubMed

    Ramirez-Alvarado, Marina; Barnidge, David R; Murray, David L; Dispenzieri, Angela; Marin-Argany, Marta; Dick, Christopher J; Cooper, Shawna A; Nasr, Samih H; Ward, Christopher J; Dasari, Surendra; Jiménez-Zepeda, Víctor H; Leung, Nelson

    2017-03-10

    Immunoglobulin light chain (AL) amyloidosis is a fatal complication of B-cell proliferation secondary to deposition of amyloid fibrils in various organs. Urinary exosomes (UEX) are the smallest of the microvesicles excreted in the urine. Previously, we found UEX of patients with AL amyloidosis contained immunoglobulin light chain (LC) oligomers that patients with multiple myeloma did not have. To further explore the role of the LC oligomers, UEX was isolated from an AL amyloidosis patient with progressive renal disease despite achieving a complete response. LC oligomers were identified. Mass spectrometry (MS) of the UEX and serum identified two monoclonal lambda LCs. Proteomics of the trypsin digested amyloid fragments in the kidney by laser microdissection and MS analysis identified a λ6 LC. The cDNA from plasma cell clone was from the IGLV- 6-57 family and it matched the amino acid sequences of the amyloid peptides. The predicted mass of the peptide product of the cDNA matched the mass of one of the two LCs identified in the UEX and serum. UEX combined with MS were able to identify 2 monoclonal lambda LCs that current clinical methods could not. It also identified the amyloidogenic LC which holds potential for response assessment in the future. This article is protected by copyright. All rights reserved.

  18. Myocardial infarction with "clean coronaries" caused by amyloid light-chain AL amyloidosis: a case report and literature review.

    PubMed

    Tsai, Stephanie B; Seldin, David C; Wu, Hao; O'Hara, Carl; Ruberg, Frederick L; Sanchorawala, Vaishali

    2011-09-01

    In AL (amyloid light-chain) amyloidosis, the greatest risk of death occurs in patients with cardiac involvement, who typically develop diastolic dysfunction and then systolic heart failure, with predisposition to arrhythmias and sudden death. Here, we present an alternate variation of cardiac amyloidosis. This patient had recent non-obstructive coronary angiography, yet suffered a fatal myocardial infarction shortly after stem cell collection and mobilization in preparation for treatment with high-dose melphalan and autologous stem cell transplantation (HDM/SCT). On autopsy, widespread deposition of amyloid was found in the small vessels of the heart with evidence of associated acute infarction. While the typical presentation of cardiac amyloidosis is an infiltrating restrictive cardiomyopathy, this case report and literature review illustrate that ischemic small vessel amyloidosis may also occur. Small vessel coronary disease and associated myocardial ischemia should be considered in patients with AL amyloidosis with angina, as its presence may increase treatment-related complications. Contemporary testing should aim to detect both forms of cardiac amyloidosis, which may impact management and prognosis.

  19. 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.

  20. Primary Systemic Amyloidosis and High Levels of Angiotensin-Converting Enzyme: Two Case Reports

    PubMed Central

    Praena-Segovia, J.; Sanchez-Gastaldo, A.; Bernabeu-Wittel, M.; Ocete-Pérez, R.; Ávila-Polo, R.; Martino, M. L.

    2013-01-01

    Infiltrative heart diseases are caused by a heterogeneous group of disorders; amyloidosis and sarcoidosis are two frequent causes of myocardial infiltration, which differ in clinical and biological outcome and treatment issues. The presence of high levels of angiotensin-converting enzyme (ACE) in a patient with infiltrative heart disease may increase suspicion of sarcoidosis. Nevertheless, no mention about increased ACE levels in extracerebral primary systemic amyloidosis is available. We present two cases of primary systemic amyloidosis, which are cardiac involvement and elevated ACE levels. PMID:24826302

  1. Caloric restriction reduces the systemic progression of mouse AApoAII amyloidosis

    PubMed Central

    Ding, Xin; Yang, Mu; Xu, Zhe; Miyahara, Hiroki; Mori, Masayuki; Higuchi, Keiichi

    2017-01-01

    In mouse senile amyloidosis, apolipoprotein (Apo) A-II is deposited extracellularly in many organs in the form of amyloid fibrils (AApoAII). Reduction of caloric intake, known as caloric restriction (CR), slows the progress of senescence and age-related disorders in mice. In this study, we intravenously injected 1 μg of isolated AApoAII fibrils into R1.P1-Apoa2c mice to induce experimental amyloidosis and investigated the effects of CR for the next 16 weeks. In the CR group, AApoAII amyloid deposits in the liver, tongue, small intestine and skin were significantly reduced compared to those of the ad libitum feeding group. CR treatment led to obvious reduction in body weight, improvement in glucose metabolism and reduction in the plasma concentration of ApoA-II. Our molecular biological analyses of the liver suggested that CR treatment might improve the symptoms of inflammation, the unfolded protein response induced by amyloid deposits and oxidative stress. Furthermore, we suggest that CR treatment might improve mitochondrial functions via the sirtuin 1-peroxisome proliferator-activated receptor γ coactivator 1α (SIRT1-PGC-1α) pathway. We suggest that CR is a promising approach for treating the onset and/or progression of amyloidosis, especially for systemic amyloidosis such as senile AApoAII amyloidosis. Our analysis of CR treatment for amyloidosis should provide useful information for determining the cause of amyloidosis and developing effective preventive treatments. PMID:28225824

  2. Induction of cerebral beta-amyloidosis: intracerebral versus systemic Abeta inoculation.

    PubMed

    Eisele, Yvonne S; Bolmont, Tristan; Heikenwalder, Mathias; Langer, Franziska; Jacobson, Laura H; Yan, Zheng-Xin; Roth, Klaus; Aguzzi, Adriano; Staufenbiel, Matthias; Walker, Lary C; Jucker, Mathias

    2009-08-04

    Despite the importance of the aberrant polymerization of Abeta in the early pathogenic cascade of Alzheimer's disease, little is known about the induction of Abeta aggregation in vivo. Here we show that induction of cerebral beta-amyloidosis can be achieved in many different brain areas of APP23 transgenic mice through the injection of dilute Abeta-containing brain extracts. Once the amyloidogenic process has been exogenously induced, the nature of the induced Abeta-deposition is determined by the brain region of the host. Because these observations are reminiscent of a prion-like mechanism, we then investigated whether cerebral beta-amyloidosis also can be induced by peripheral and systemic inoculations or by the intracerebral implantation of stainless steel wires previously coated with minute amounts of Abeta-containing brain extract. Results reveal that oral, intravenous, intraocular, and intranasal inoculations yielded no detectable induction of cerebral beta-amyloidosis in APP23 transgenic mice. In contrast, transmission of cerebral beta-amyloidosis through the Abeta-contaminated steel wires was demonstrated. Notably, plasma sterilization, but not boiling of the wires before implantation, prevented the induction of beta-amyloidosis. Our results suggest that minute amounts of Abeta-containing brain material in direct contact with the CNS can induce cerebral beta-amyloidosis, but that systemic cellular mechanisms of prion uptake and transport to the CNS may not apply to Abeta.

  3. T1 mapping and survival in systemic light-chain amyloidosis

    PubMed Central

    Banypersad, Sanjay M.; Fontana, Marianna; Maestrini, Viviana; Sado, Daniel M.; Captur, Gabriella; Petrie, Aviva; Piechnik, Stefan K.; Whelan, Carol J.; Herrey, Anna S.; Gillmore, Julian D.; Lachmann, Helen J.; Wechalekar, Ashutosh D.; Hawkins, Philip N.; Moon, James C.

    2015-01-01

    Aims To assess the prognostic value of myocardial pre-contrast T1 and extracellular volume (ECV) in systemic amyloid light-chain (AL) amyloidosis using cardiovascular magnetic resonance (CMR) T1 mapping. Methods and results One hundred patients underwent CMR and T1 mapping pre- and post-contrast. Myocardial ECV was calculated at contrast equilibrium (ECVi) and 15 min post-bolus (ECVb). Fifty-four healthy volunteers served as controls. Patients were followed up for a median duration of 23 months and survival analyses were performed. Mean ECVi was raised in amyloid (0.44 ± 0.12) as was ECVb (mean 0.44 ± 0.12) compared with healthy volunteers (0.25 ± 0.02), P < 0.001. Native pre-contrast T1 was raised in amyloid (mean 1080 ± 87 ms vs. 954 ± 34 ms, P < 0.001). All three correlated with pre-test probability of cardiac involvement, cardiac biomarkers, and systolic and diastolic dysfunction. During follow-up, 25 deaths occurred. An ECVi of >0.45 carried a hazard ratio (HR) for death of 3.84 [95% confidence interval (CI): 1.53–9.61], P = 0.004 and pre-contrast T1 of >1044 ms = HR 5.39 (95% CI: 1.24–23.4), P = 0.02. Extracellular volume after primed infusion and ECVb performed similarly. Isolated post-contrast T1 was non-predictive. In Cox regression models, ECVi was independently predictive of mortality (HR = 4.41, 95% CI: 1.35–14.4) after adjusting for E:E′, ejection fraction, diastolic dysfunction grade, and NT-proBNP. Conclusion Myocardial ECV (bolus or infusion technique) and pre-contrast T1 are biomarkers for cardiac AL amyloid and they predict mortality in systemic amyloidosis. PMID:25411195

  4. Mice with Alopecia, Osteoporosis, and Systemic Amyloidosis Due to Mutation in Zdhhc13, a Gene Coding for Palmitoyl Acyltransferase

    PubMed Central

    Hsiao, Ya-Wen; Huang, Hong-Wen; Kao, Hsiao-Jung; Liu, Kai-Ming; Shen, Li-Fen; Song, I-wen; Tu, Chen-Pei D.; Wu, Jer-Yuarn; Kikuchi, Tateki; Justice, Monica J.; Yen, Jeffrey J. Y.; Chen, Yuan-Tsong

    2010-01-01

    Protein palmitoylation has emerged as an important mechanism for regulating protein trafficking, stability, and protein–protein interactions; however, its relevance to disease processes is not clear. Using a genome-wide, phenotype driven N-ethyl-N-nitrosourea–mediated mutagenesis screen, we identified mice with failure to thrive, shortened life span, skin and hair abnormalities including alopecia, severe osteoporosis, and systemic amyloidosis (both AA and AL amyloids depositions). Whole-genome homozygosity mapping with 295 SNP markers and fine mapping with an additional 50 SNPs localized the disease gene to chromosome 7 between 53.9 and 56.3 Mb. A nonsense mutation (c.1273A>T) was located in exon 12 of the Zdhhc13 gene (Zinc finger, DHHC domain containing 13), a gene coding for palmitoyl transferase. The mutation predicted a truncated protein (R425X), and real-time PCR showed markedly reduced Zdhhc13 mRNA. A second gene trap allele of Zdhhc13 has the same phenotypes, suggesting that this is a loss of function allele. This is the first report that palmitoyl transferase deficiency causes a severe phenotype, and it establishes a direct link between protein palmitoylation and regulation of diverse physiologic functions where its absence can result in profound disease pathology. This mouse model can be used to investigate mechanisms where improper palmitoylation leads to disease processes and to understand molecular mechanisms underlying human alopecia, osteoporosis, and amyloidosis and many other neurodegenerative diseases caused by protein misfolding and amyloidosis. PMID:20548961

  5. 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.

  6. Hereditary amyloidosis

    MedlinePlus

    ... MA. Amyloidosis. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine . 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 188. Seldin DC, Skinner M. Amyloidosis. In: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell ...

  7. A reappraisal of immunoglobulin variable gene primers and its impact on assessing clonal relationships between PB B cells and BM plasma cells in AL amyloidosis.

    PubMed

    Katoh, Nagaaki; Poshusta, Tanya L; Manske, Michelle K; Dispenzieri, Angela; Gertz, Morie A; Abraham, Roshini S; Ramirez-Alvarado, Marina

    2011-12-01

    Monoclonal tumor plasma cells as well as non-terminally differentiated B cells having a clonal relationship to the tumor cells have been detected in the peripheral blood (PB) of some multiple myeloma (MM) patients but rarely in light chain (primary systemic) amyloidosis (AL) patients. Previously, our group found these peripheral clonotypic B cells in three AL patients. Here, we report detailed analysis of a larger cohort of AL patients to validate the prior findings and to investigate the effect of this cell population on clinical outcome. Fourteen AL patients were selected from a clinical prospective trial, and the relationship between immunoglobulin light chain variable gene (V(L)) representation in PB B cells and the clonal population in the bone marrow (BM) was investigated. A clonal relationship was not detected, and the present study provides important insights into the disparity with the earlier data, including clinical history of the patients and methodological analysis.

  8. Echocardiographic diagnosis of systemic AA amyloidosis presenting with acute liver failure.

    PubMed

    Morelli, Sergio; Bonfiglio, Daniele; Galasso, Laura

    2010-08-01

    We report the case of a 73-year-old man admitted to our hospital for acute hepatic failure. Antemortem diagnosis of systemic AA amyloidosis was made because of typical electrocardiographic and echocardiographic findings, in the absence of the classic clinical picture of kidney involvement.

  9. Neuropathy and efficacy of once weekly subcutaneous bortezomib in multiple myeloma and light chain (AL) amyloidosis

    PubMed Central

    Sidana, Surbhi; Narkhede, Mayur; Elson, Paul; Hastings, Debbie; Faiman, Beth; Valent, Jason; Samaras, Christy; Hamilton, Kimberly; Liu, Hien K.; Smith, Mitchell R.; Reu, Frederic J.

    2017-01-01

    Introduction Randomized studies have shown that bortezomib (BTZ) can be given weekly via intravenous (IV) route or twice weekly via subcutaneous (SC) route with lower neuropathy risk and no loss of anti-myeloma efficacy compared to original standard IV twice weekly schedule. Weekly SC should therefore yield the best therapeutic index and is widely used but has not been compared to established administration schedules in the context of a clinical trial. Methods Comprehensive electronic medical record review was done for disease control and neuropathy symptoms of 344 consecutive patients who received their first BTZ-containing regimen for myeloma or AL amyloidosis before or after we changed to SC weekly in December 2010. Univariate and multivariable analyses were carried out that adjusted for age, underlying disease, concurrently used anticancer agents, underlying conditions predisposing to neuropathy, and number of prior regimens compared SC weekly to other schedules. Results Fifty-three patients received BTZ SC weekly, 17 SC twice weekly, 127 IV weekly and 147 IV twice weekly. Risk for neuropathy of any grade was higher with other schedules compared to SC weekly (44.3% vs. 26.9%, p = 0.001) while response rate was similar (72.1% vs. 76.6%, respectively, p = 0.15). Multivariable analyses upheld higher neuropathy risk (Odds ratio 2.45, 95% CI 1.26–4.76, p = 0.008) while the likelihood of not achieving a response (= partial response or better) was comparable (Odds ratio 1.25, 95% CI 0.58–2.71, p = 0.56) for other schedules compared to SC weekly, respectively. Lower neuropathy risk translated into longer treatment duration when BTZ was started SC weekly (p = 0.001). Conclusions Weekly SC BTZ has activity comparable to other schedules and causes low rates of neuropathy. PMID:28278302

  10. 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. PMID:27828646

  11. Extracorporeal Membrane Oxygenation as Bridge-to-Decision in Acute Heart Failure due to Systemic Light-Chain Amyloidosis

    PubMed Central

    Silva, Jennifer Mancio; Fontes-Carvalho, Ricardo; Valente, Dília; Almeida, Cristiana; Cruz, António José; Tente, David; Coelho, Henrique; Oliveira, Marco; Albuquerque, Aníbal; Ribeiro, Vasco Gama

    2015-01-01

    Patient: Female, 58 Final Diagnosis: Acute hear failure Symptoms: Dispnoea • edema • fatigue Medication: — Clinical Procedure: Bone marrow biopsy • endomyocardial biopsy • abdominal subcutaneous fat biopsy under ECMO support Specialty: Cardiology Objective: Rare disease Background: Cardiac amyloidosis results from the amyloid deposition in heart tissue, either in the context of a systemic disease or as a localized form. Several pro-amyloid proteins can produce amyloid deposits in the heart. Each of these amyloidoses has characteristic clinical (cardiac and extracardiac) features, and a specific diagnosis and treatment. Case Report: A 58-year-old woman who presented with acute heart failure and echocardiographic findings strongly suggestive of infiltrative cardiomyopathy needed percutaneous veno-arterial extracorporeal membrane oxygenation (ECMO) as bridge-to-decision. Amyloid deposition was found on endomyocardial and bone marrow biopsies. Bone marrow plasma cell infiltrate with acute renal lesion and hypercalcemia confirmed the diagnosis of multiple myeloma-associated systemic light-chain amyloidosis (AL). Refractory shock with multi-organic failure syndrome persisted and no improvements in left ventricular function and structure were seen. After extensive discussion by a multidisciplinary team, and with the patients’ family, she was not considered eligible for high-dose chemotherapy and/or autologous stem cell transplantation, heart transplantation, or sequential heart with autologous stem cell transplantation. The patient died a few hours after ECMO withdrawal. During the 14 days of ECMO support no major bleeding or thrombotic complications occurred. Conclusions: The clinician must consider a diagnosis of cardiac amyloidosis in patients with heart failure, a restrictive type of cardiomyopathy with ventricular hypertrophy in the absence of valve abnormalities, or uncontrolled arterial hypertension. Although developments in chemotherapy have greatly

  12. 2A4 binds soluble and insoluble light chain aggregates from AL amyloidosis patients and promotes clearance of amyloid deposits by phagocytosis †

    PubMed Central

    Renz, Mark; Torres, Ronald; Dolan, Philip J.; Tam, Stephen J.; Tapia, Jose R.; Li, Lauri; Salmans, Joshua R.; Barbour, Robin M.; Shughrue, Paul J.; Nijjar, Tarlochan; Schenk, Dale; Kinney, Gene G.; Zago, Wagner

    2016-01-01

    Abstract Amyloid light chain (AL) amyloidosis is characterized by misfolded light chain (LC) (amyloid) deposition in various peripheral organs, leading to progressive dysfunction and death. There are no regulatory agency–approved treatments for AL amyloidosis, and none of the available standard of care approaches directly targets the LC protein that constitutes the amyloid. NEOD001, currently in late-stage clinical trials, is a conformation-specific, anti-LC antibody designed to specifically target misfolded LC aggregates and promote phagocytic clearance of AL amyloid deposits. The present study demonstrated that the monoclonal antibody 2A4, the murine form of NEOD001, binds to patient-derived soluble and insoluble LC aggregates and induces phagocytic clearance of AL amyloid in vitro. 2A4 specifically labeled all 21 fresh-frozen organ samples studied, which were derived from 10 patients representing both κ and λ LC amyloidosis subtypes. 2A4 immunoreactivity largely overlapped with thioflavin T–positive labeling, and 2A4 bound both soluble and insoluble LC aggregates extracted from patient tissue. Finally, 2A4 induced macrophage engagement and phagocytic clearance of AL amyloid deposits in vitro. These findings provide further evidence that 2A4/NEOD001 can effectively clear and remove human AL-amyloid from tissue and further support the rationale for the evaluation of NEOD001 in patients with AL amyloidosis. PMID:27494229

  13. Kidney Involvement in Systemic Calcitonin Amyloidosis Associated With Medullary Thyroid Carcinoma.

    PubMed

    Koopman, Timco; Niedlich-den Herder, Cindy; Stegeman, Coen A; Links, Thera P; Bijzet, Johan; Hazenberg, Bouke P C; Diepstra, Arjan

    2017-04-01

    A 52-year-old woman with widely disseminated medullary thyroid carcinoma developed nephrotic syndrome and slowly decreasing kidney function. A kidney biopsy was performed to differentiate between malignancy-associated membranous glomerulopathy and tyrosine kinase inhibitor-induced focal segmental glomerulosclerosis. Surprisingly, the biopsy specimen revealed diffuse glomerular deposition of amyloid that was proved to be derived from the calcitonin hormone (Acal), produced by the medullary thyroid carcinoma. This amyloid was also present in an abdominal fat pad biopsy. Although local ACal deposition is a characteristic feature of medullary thyroid carcinoma, the systemic amyloidosis involving the kidney that is presented in this case report has not to our knowledge been described previously and may be the result of long-term high plasma calcitonin levels. Our case illustrates that systemic calcitonin amyloidosis should be considered in the differential diagnosis of proteinuria in patients with medullary thyroid carcinoma.

  14. Intestinal pseudo-obstruction associated with amyloidosis.

    PubMed

    Liapis, Konstantinos; Michelis, Fotios V; Delimpasi, Sosanna; Karmiris, Themistoklis

    2011-06-01

    Intestinal pseudo-obstruction is a condition characterised by clinical manifestations of mechanical obstruction of the intestine in the absence of any organic occlusion of the lumen. This syndrome has rarely been reported to complicate the course of systemic amyloidosis. We describe the case of a 64-year-old man who presented with the syndrome of small bowel pseudo-obstruction secondary to AL amyloid infiltration of the gastrointestinal tract. We comment on the pathophysiology and on the clinical importance of amyloidosis-associated intestinal pseudo-obstruction.

  15. 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

  16. Development of Renal Failure without Proteinuria in a Patient with Monoclonal Gammopathy of Undetermined Significance: An Unusual Presentation of AL Kappa Amyloidosis.

    PubMed

    Sun, Yijuan; Sandhu, Amarpreet; Gabaldon, Darlene; Danaraj, Jonathan; Servilla, Karen S; Tzamaloukas, Antonios H

    2012-01-01

    AL amyloidosis complicating monoclonal gammopathy of undetermined significance (MGUS) has usually a predominant glomerular deposition of lambda light chain. Heavy proteinuria is one of its cardinal manifestations. A 78-year-old man with a 9-year history of IgG kappa light-chain-MGUS and normal urine protein excretion developed severe renal failure. Serum levels of kappa light chain and serum IgG had been stable while proteinuria was absent throughout the nine-year period. For the first eight years, he had stable stage III chronic kidney disease attributed to bladder outlet obstruction secondary to prostatic malignancy. In the last year, he developed progressive serum creatinine elevation, without any increase in the serum or urine levels of paraproteins or any sign of malignancy. Renal ultrasound and furosemide renogram showed no evidence of urinary obstruction. Renal biopsy revealed AL amyloidosis, with reactivity exclusive for kappa light chains, affecting predominantly the vessels and the interstitium. Glomerular involvement was minimal. Melphalan and prednisone were initiated. However, renal function continues deteriorating. Deposition of AL kappa amyloidosis developing during the course of MGUS predominantly in the wall of the renal vessels and the renal interstitium, while the involvement of the glomeruli is minimal, leads to progressive renal failure and absence of proteinuria. Renal biopsy is required to detect both the presence and the sites of deposition of renal AL kappa light chain amyloidosis.

  17. Development of Renal Failure without Proteinuria in a Patient with Monoclonal Gammopathy of Undetermined Significance: An Unusual Presentation of AL Kappa Amyloidosis

    PubMed Central

    Sun, Yijuan; Sandhu, Amarpreet; Gabaldon, Darlene; Danaraj, Jonathan; Servilla, Karen S.; Tzamaloukas, Antonios H.

    2012-01-01

    AL amyloidosis complicating monoclonal gammopathy of undetermined significance (MGUS) has usually a predominant glomerular deposition of lambda light chain. Heavy proteinuria is one of its cardinal manifestations. A 78-year-old man with a 9-year history of IgG kappa light-chain-MGUS and normal urine protein excretion developed severe renal failure. Serum levels of kappa light chain and serum IgG had been stable while proteinuria was absent throughout the nine-year period. For the first eight years, he had stable stage III chronic kidney disease attributed to bladder outlet obstruction secondary to prostatic malignancy. In the last year, he developed progressive serum creatinine elevation, without any increase in the serum or urine levels of paraproteins or any sign of malignancy. Renal ultrasound and furosemide renogram showed no evidence of urinary obstruction. Renal biopsy revealed AL amyloidosis, with reactivity exclusive for kappa light chains, affecting predominantly the vessels and the interstitium. Glomerular involvement was minimal. Melphalan and prednisone were initiated. However, renal function continues deteriorating. Deposition of AL kappa amyloidosis developing during the course of MGUS predominantly in the wall of the renal vessels and the renal interstitium, while the involvement of the glomeruli is minimal, leads to progressive renal failure and absence of proteinuria. Renal biopsy is required to detect both the presence and the sites of deposition of renal AL kappa light chain amyloidosis. PMID:24555136

  18. Primary Hepatic Amyloidosis Presenting as Acute-on-Chronic Liver Failure

    PubMed Central

    Rangegowda, Devaraja; Vyas, Tanmay; Kulkarni, Anand; Grover, Shrruti; Mahiwall, Rakhi; Sarin, Shiv Kumar

    2017-01-01

    Systemic amyloidosis of amyloid light chain associated protein (AL), also called primary amyloidosis, frequently involves the liver, but rarely causes clinically apparent liver disease. The more common presentation is with acute renal failure. Hepatomegaly and mild elevation of alkaline phosphatase are the most common clinical and biochemical findings, respectively. We report a case of systemic amyloidosis of AL that clinically presented as acute-on-chronic liver failure and resulted in a fatal clinical course in a 56-year-old man. PMID:28286788

  19. Myeloma-associated systemic amyloidosis presenting with acquired digital cutis laxa-like changes.

    PubMed

    Dicker, Tony J; Morton, James; Williamson, Richard M; Chick, Jeff

    2002-05-01

    A 59-year-old woman presented with a 6-year history of lax skin on the distal fingers of both hands, as well as a recent increase in the size of her tongue. Histopathology of skin from her distal finger showed amyloid deposition and bone marrow biopsy revealed an underlying plasma cell dyscrasia. Initial treatment with cyclophosphamide, vincristine, adriamycin and methylprednisolone has produced a significant reduction in the swelling of both her hands and tongue. Acquired digital cutis laxa-like changes are a rare cutaneous manifestation of systemic amyloidosis.

  20. Sinonasal Globular Amyloidosis Simulating Malignancy: A Rare Presentation.

    PubMed

    Kumar, Binay; Pant, Bhawna; Kumar, Vikrant; Negi, Meghna

    2016-09-01

    Primary localized amyloidosis in the head and neck region is a rare entity. The most commonly involved organ is larynx. Primary amyloidosis localized to the sinonasal tract is extremely rare. We report one such case along with a brief review of the associated literature. The aim of reporting this case is to emphasize the fact that sometimes nasal amyloidosis can also present with signs and symptoms of nasal and nasopharyngeal malignancy. The definitive diagnosis in such cases depends upon histopathology and further confirmed by immunohistochemistry. A 55-year old male presented with recurrent episodes of nasal bleed, bilateral nasal obstruction, and bilateral hearing loss from last 7 years. On clinical examination a mass was found in the nasal cavity on both sides reaching up to the nasopharynx. Contrast enhanced CT scan revealed that the mass was extending up to the skull base and destroying bony landmarks of the nasal cavity and paranasal sinuses. Mass was proved to be amyloidosis after histopathological examination. It showed multiple blotches of globular submucosal deposit of amyloid, on staining with Congo red. Immunohistochemistry confirmed AL amyloidosis with expression of mixed kappa and lambda light chain immunoglobulin (κ > λ). No evidence of systemic amyloidosis was found after proper work up. It was managed by conservative surgery.

  1. AL amyloidosis with IgD-lambda monoclonal gammopathy and lambda-type Bence-Jones protein: successful treatment by autologous stem cell transplantation.

    PubMed

    Sakurai-Chin, Chanhyok; Ubara, Yoshifumi; Suwabe, Tatsuya; Hoshino, Junichi; Yonaha, Tomoki; Hasegawa, Eiko; Sumida, Keiichi; Hiramatsu, Rikako; Yamanouchi, Masayuki; Hayami, Noriko; Yamauchi, Junji; Tominaga, Naoyuki; Sawa, Naoki; Takemoto, Fumi; Masuoka, Kazuhiro; Takaichi, Kenmei; Oohashi, Kenichi

    2010-10-01

    A 45-year-old Japanese woman had been diagnosed with monoclonal gammopathy of undetermined significance (MGUS) featuring urinary Bence-Jones protein of the lambda type (BJP-lambda) for 11 years. She then developed eyelid purpura, dyspnea, and flank pain. Abdominal CT scans revealed renal infarction. Biopsy of the kidney, heart, jejunum, and skin demonstrated amyloid deposits in the vessel walls, but not in the glomeruli. She was diagnosed as having AL amyloidosis with IgD-lambda monoclonal gammopathy and BJP-lambda. Autologous stem cell transplantation (SCT) was done after chemotherapy with vincristine, daunorubicin, dexamethasone (VAD), and high-dose melphalan (HDM). This reduced the IgD level from 156 to 0.1 mg/dL, along with the disappearance of BJP, despite cerebral infarction during chemotherapy. We recommend SCT for patients with IgD-associated AL amyloidosis.

  2. Nuclear imaging of amyloidosis.

    PubMed

    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. (123)I-SAP proved to have high sensitivity in imaging of AA and AL amyloidosis in visceral organs. (99m)Tc-Aprotinin was found to be useful in detecting cardiac amyloidosis. A couple of classical radiotracers, such as (201)Tl, (123)I-mIBG, together with (111)In-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. (67)Ga-citrate and (99m)Tc-penta-DMSA. High sensitivity and specificity was achieved with β2-microglobulin labeled with (131)I or (111)In. Among PET tracers, (11)C-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.

  3. Primary systemic amyloidosis presenting as a colonic stricture: successful treatment with left hemicolectomy followed by autologous hematopoietic stem-cell transplantation: report of a case.

    PubMed

    Rives, S; Pera, M; Rosiñol, L; Vidal, O; Miquel, R; Solé, M; García-Valdecasas, J; Bladé, J

    2002-09-01

    Intestinal tract involvement by primary systemic amyloidosis is frequent but usually asymptomatic. Ischemic colitis caused by amyloid infiltration of wall blood vessels can occasionally be observed. We report a 62-year-old female with primary systemic amyloidosis who presented with intestinal obstruction caused by ischemic stricture of the sigmoid colon, secondary to submucosal amyloid deposition. The patient was successfully treated with surgical resection followed by high-dose chemotherapy and hematopoietic stem-cell transplantation. The clinical manifestations and differential diagnosis of gastrointestinal involvement of primary systemic amyloidosis, as well as its current treatment, are discussed.

  4. Experimental transmission of systemic AA amyloidosis in autoimmune disease and type 2 diabetes mellitus model mice

    PubMed Central

    Maeda, Mayuko; Murakami, Tomoaki; Muhammad, Naeem; Inoshima, Yasuo; Ishiguro, Naotaka

    2016-01-01

    AA amyloidosis is a protein misfolding disease characterized by extracellular deposition of amyloid A (AA) fibrils. AA amyloidosis has been identified in food animals, and it has been postulated that AA amyloidosis may be transmissible to different animal species. Since the precursor protein of AA fibrils is serum amyloid A (SAA), which is an inflammatory acute phase protein, AA amyloidosis is considered to be associated with inflammatory diseases such as rheumatoid arthritis. Chronic diseases such as autoimmune disease and type 2 diabetes mellitus could be potential factors for AA amyloidosis. In this study, to examine the relationship between the induction of AA amyloidosis and chromic abnormalities such as autoimmune disease or type 2 diabetes mellitus, amyloid fibrils from mice, cattle, or chickens were experimentally injected into disease model mice. Wild-type mice were used as controls. The concentrations of SAA, IL-6, and IL-10 in autoimmune disease model mice were higher than those of control mice. However, induction of AA amyloidosis in autoimmune disease and type 2 diabetes mellitus model mice was lower than that in control mice, and the amount of amyloid deposits in the spleens of both mouse models was lower than that of control mice according to Congo red staining and immunohistochemistry. These results suggest that factors other than SAA levels, such as an inflammatory or anti-inflammatory environment in the immune response, may be involved in amyloid deposition. PMID:27321428

  5. Amyloidosis and Anesthesia

    PubMed Central

    Wani, Zara; Harkawat, Dev Kumar; Sharma, Meenaxi

    2017-01-01

    Aim: The aim of this article is to provide a view of amyloidosis and discuss implications for the anesthetic management of patients with this condition. Material and Method: Urine samples from patients with plasma cell dyscrasias were obtained from a urine bank that gathers urine samples from patients who gave research use consent for specimens that would otherwise be considered waste. Results: Patients with amyloidosis may present to the anesthesiologist for procedures relating to diagnosis, surgery relating to the underlying condition (e.g., bronchial laser and organ transplant), or for incidental surgery. The condition carries a significant risk of perioperative morbidity and mortality. Conclusion: The term amyloid was coined by Virchow in the mid 19th century, meaning “starch like.” Amyloidosis is a disease complex, in which there is an abnormal deposition of extracellular hyaline material with particular staining characteristics and which contains protein fibrils embedded in a relatively amorphous ground substance. There are numerous clinical manifestations, the onset is insidious, and the diagnosis may not be made in a patient undergoing anesthesia and surgery for an apparently straightforward problem. Unexpected complications such as heart or kidney failure may arise, either before operation or in the postoperative period. Bullous lesions of the skin or oral mucosa and extensive areas of purpura are but two of the ways, in which amyloidosis may first present. The disease spectrum may be inherited or acquired, localized or systemic, and life threatening or an incidental finding. PMID:28298791

  6. [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.

  7. 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

  8. Treatment Patterns and Outcome Following Initial Relapse or Refractory Disease in Patients with Systemic Light Chain Amyloidosis.

    PubMed

    Tandon, Nidhi; Sidana, Surbhi; Gertz, Morie A; Dispenzieri, Angela; Lacy, Martha Q; Buadi, Francis K; Dingli, David; Fonder, Amie L; Hobbs, Miriam A; Hayman, Suzanne R; Gonsalves, Wilson I; Hwa, Yi Lisa; Kapoor, Prashant; Kyle, Robert A; Leung, Nelson; Go, Ronald S; Lust, John A; Russell, Stephen J; Zeldenrust, Steven R; Rajkumar, S Vincent; Kumar, Shaji K

    2017-03-17

    We analyzed the outcomes following initial relapse or refractory disease in systemic light chain amyloidosis (AL) and the impact of type of therapy employed.A total of 1327 patients with AL seen at Mayo Clinic within 90 days of diagnosis, between 2006 and 2015, were reviewed. The study included 366 patients experiencing a documented hematological or organ relapse or refractory disease requiring start of second line therapy. Overall survival (OS) and time to next treatment (TTNT) were calculated from start of second line treatment.The median time to require second line treatment was 16.2 months (1-93) from the start of first line therapy. At relapse, patients received proteasome inhibitors (PI; 45.1%), immunomodulators (IMiD; 22.7%), alkylators (9%), PI and IMiD combination (4.1%), autologous transplant (3.8%), steroids and other therapies (4.9%). Among these, 124 (33.9%) required change or reinstitution of therapy. The median time to require third line treatment was 31 months (95% CI; 24, 40.5) and the median overall survival (OS) was 38.8 months (95% CI; 29.6, 52.6) from the start of second line treatment. Retreatment with same therapy at relapse significantly reduced TTNT (22m vs 32.3m; p= 0.01) as compared to different therapy; but did not have any impact OS (30.8m vs 51.1m; p = 0.5). In conclusion, this study provides important information about outcomes of patients with AL who require second line treatment for relapsed/refractory disease . Treatment with a different therapy at relapse improves time to next therapy but does not impact OS. This article is protected by copyright. All rights reserved.

  9. Malabsorption Secondary to Gout-Induced Amyloidosis

    PubMed Central

    Balasubramaniam, Renuka; Safa, Shahram; McIvor, Carolyn; Mollee, Peter

    2017-01-01

    Many chronic inflammatory conditions can lead to systemic amyloidosis. However, secondary amyloidosis has rarely been associated with gout, and the literature reports only a handful of cases, all presenting with renal disease. We report a patient with a history of poorly controlled gout who presented with malabsorption. Endoscopic biopsies confirmed a diagnosis of small intestinal amyloidosis. This was believed to be a consequence of gout. Interestingly, renal involvement was subclinical. Our case raises awareness of this rare association and highlights the importance of considering a diagnosis of amyloidosis in patients who present with the combination of gout and gastrointestinal symptoms. PMID:28286797

  10. Clinical and imaging predictors of 1-year and long-term mortality in light chain (AL) amyloidosis: a 5-year follow-up study.

    PubMed

    Migrino, Raymond Q; Harmann, Leanne; Christenson, Richard; Hari, Parameswaran

    2014-11-01

    Light chain amyloidosis (AL) involves multiorgan failure induced by amyloidogenic light chain proteins, and is associated with high mortality. We aimed to identify clinical, laboratory, and imaging parameters that would predict 1-year and long-term AL mortality. Forty-four biopsy-proven AL patients (61.5 ± 12 years, 20 females) underwent clinical evaluation including laboratory assays, echocardiography, and contrast cardiac magnetic resonance imaging (CMR, n = 31) prior to chemotherapy. Patients were prospectively followed for median duration of 62.7 months (interquartile range 35.5 months). Clinical and laboratory parameters were compared between 1-year survivors and nonsurvivors. Univariate Kaplan-Meier survival plots were calculated followed by stepwise logistic regression analysis to assess independent predictors of long-term survival. Eighteen (40.9 %) patients died within 1 year and an additional 10 subjects died during long-term follow-up. Patients who expired within 1 year presented with more advanced class of heart failure, higher alkaline phosphatase and uric acid, lower limb lead voltage on electrocardiography, shorter left ventricular ejection time (ET) on echocardiography, and a higher proportion of late gadolinium enhancement on CMR. On multivariable analysis, only ET ≤240 ms on echocardiography (hazard ratio (HR) 5.07, 95 % confidence interval (CI) 1.83-14.1, P = 0.002) and New York Heart Association functional class II-IV presentation (HR 1.0058, 95 % CI 1.0014-1.0103, P = 0.01) were independent predictors of AL mortality. In conclusion, AL amyloidosis is associated with high 1-year and long-term mortality. Among clinical, laboratory, and imaging parameters tested, an echocardiographic finding of ET ≤240 ms has independent and additive prognostic value to clinical heart failure evaluation in determining long-term survival of AL patients. This result may be important in the early identification of patients at risk.

  11. Amyloidosis: diffuse involvement of the retroperitoneum.

    PubMed

    Glynn, T P; Kreipke, D L; Irons, J M

    1989-03-01

    Systemic amyloidosis diffusely involving the retroperitoneum has not, to the authors' knowledge, been previously reported. The computed tomographic scans of a 68-year-old man showed evidence of diffuse, nonenhancing thickening of the entire retroperitoneum. This appearance mimicked retroperitoneal fibrosis, but the diagnosis of amyloidosis was confirmed at autopsy.

  12. Nephrotic Syndrome Associated with Lung Cancer: A Rare Case of Malignancy Associated with AA Amyloidosis.

    PubMed

    Gueutin, Victor; Langlois, Anne-Lyse; Shehwaro, Nathalie; Elharraqui, Ryme; Rouvier, Philippe; Izzedine, Hassane

    2013-01-01

    Nonhematologic malignancies are rarely reported to be associated with AA amyloidosis. Although the association between renal cell carcinoma and systemic AA amyloidosis has been established, the evidence linking pulmonary cancer to AA amyloidosis is scarce. Here, a case of biopsy-proven renal AA amyloidosis complicated with nephrotic syndrome associated with lung carcinoma is reported.

  13. Nephrotic Syndrome Associated with Lung Cancer: A Rare Case of Malignancy Associated with AA Amyloidosis

    PubMed Central

    Gueutin, Victor; Langlois, Anne-Lyse; Shehwaro, Nathalie; Elharraqui, Ryme; Rouvier, Philippe; Izzedine, Hassane

    2013-01-01

    Nonhematologic malignancies are rarely reported to be associated with AA amyloidosis. Although the association between renal cell carcinoma and systemic AA amyloidosis has been established, the evidence linking pulmonary cancer to AA amyloidosis is scarce. Here, a case of biopsy-proven renal AA amyloidosis complicated with nephrotic syndrome associated with lung carcinoma is reported. PMID:24558629

  14. [White blood cell lysis syndrome after autologous peripheral blood stem cell transplantation in the treatment of renal AL amyloidosis. Case report].

    PubMed

    Gatica, Antonio; Bertin, Pablo; Tagle, Rodrigo

    2006-06-01

    The treatment of AL amyloidosis was not successful until the advent of myeloablative chemotherapy consisting of high-dose intravenous melphalan followed by autologous peripheral blood stem cell transplantation. This new treatment has achieved better survival rates and, remarkably, it has obtained complete remission. Among patients with renal involvement, achievement of a complete hematological response was associated with a 50% reduction in proteinuria and stable creatinine clearance in more than 2/3 of patients. Despite of these excellent results, this new therapy is associated with significant toxicity, including the development of acute renal failure due to white blood cell lysis syndrome. We report a 59 year-old female with a nephrotic syndrome due to primary amyloidosis successfully treated autologous stem cell transplantation who developed acute renal failure caused by white blood cell lysis syndrome. The patient required treatment with granulocytic colony stimulating factor and intermittent hemofiltration and was discharged 23 days after melphalan administration with a satisfactory renal function and white blood cell count. After one year of follow up, she maintains a good glomerular filtration rate, a proteinuria of less than, 1 g/day and normal hematological values.

  15. Light chain amyloidosis: Where are the light chains from and how they play their pathogenic role?

    PubMed

    Zhang, Chunlan; Huang, Xufei; Li, Jian

    2017-03-08

    Amyloid light-chain (AL) amyloidosis is a plasma-cell dyscrasia, as well as the most common type of systematic amyloidosis. Pathogenic plasma cells that have distinct cytogenetic and molecular properties secrete an excess amount of amyloidogenic light chains. Assisted by post-translational modifications, matrix components, and other environmental factors, these light chains undergo a conformational change that triggers the formation of amyloid fibrils that overrides the extracellular protein quality control system. Moreover, the amyloidogenic light-chain itself is cytotoxic. As a consequence, organ dysfunction is caused by both organ architecture disruption and the direct cytotoxic effect of amyloidogenic light chains. Here, we reviewed the molecular mechanisms underlying this sequence of events that ultimately leads to AL amyloidosis and also discuss current in vitro and in vivo models, as well as relevant novel therapeutic approaches.

  16. 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.

  17. [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.

  18. [Amyloidosis and familial Mediterranean fever].

    PubMed

    Pras, M

    1986-01-01

    Familial Mediterranean Fever (F. M. F.) is an autosomal recessive disorder occurring most commonly in Sepharadi Jews and Armenians. Two phenotypic features characterize the disease: brief episodic febrile attacks of peritonitis, pleuritis or synovitis recurring from childhood or adolescence and the development of systemic amyloidosis. Attacks are accompanied by striking elevations of acute phase proteins, including serum amyloid A protein. The amyloidosis of Familial Mediterranean Fever is of the AA type, and manifest clinically as a nephropathy that passes through proteinuria, nephrotic and uremic stages to renal death. Although there is ethnic variation in the incidence of amyloidosis of F. M. F. in our patient population--predominantly Sepharadi Jews of North African extraction--an amyloidotic death at an early age is their genetic destiny. Since the introduction in 1972 of colchicine to prevent the febrile attacks, the drug has been proven and become the main stay of therapy. Today, colchicine has been shown to be effective in preventing amyloidosis as well as the febrile attacks in Familial Mediterranean Fever. End stage renal disease is not the end of the road for patients with F.M.F. because of improving outlook for dialysis and renal transplantation in these patients.

  19. 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

  20. Amyloidosis of the Tongue-Report of A Rare Case

    PubMed Central

    Babburi, Suresh; B, Ramya; RV, Subramanyam; V, Aparna; Srivastava, Gautam

    2013-01-01

    Amyloid involvement of the tongue is almost always secondary to systemic amyloidosis. Isolated amyloidosis of the tongue is relatively rare and it accounts for less than 9% of all types of amyloidosis. We are presenting a case of a 54–year–old male patient who complained of an enlarged tongue and bilateral multiple swellings on the lateral borders of the tongue, which had been there since one year. Bilaterally symmetrical, violaceous, purpuric patches interspersed with nodules were seen surrounding the eyes. Histopathologically, the lesion exhibited homogenous eosinophilic amyloid-like material. Special staining with Congo red showed amyloid material as peach red colour under light microscopy and as apple green birefringence under polarized light. Based on these observations, a definitive diagnosis of amyloidosis of tongue was made. Amyloidosis of tongue is uncommon and its features resemble those of a benign tumour. A battery of tests is necessary to differentiate localized amyloidosis from its systemic forms PMID:24551740

  1. Amyloidosis and auto-inflammatory syndromes.

    PubMed

    Grateau, Gilles; Jéru, Isabelle; Rouaghe, Saad; Cazeneuve, Cécile; Ravet, Nathalie; Duquesnoy, Philippe; Cuisset, Laurence; Dodé, Catherine; Delpech, Marc; Amselem, Serge

    2005-02-01

    Amyloidosis remains currently a severe potential complication of many chronic inflammatory disorders. It is not exactly know why some patients develop a progressive amyloidosis, whereas others do not although latent deposits may be present. A permanent acute phase response, ideally evaluated with serial measurement of serum protein SAA, the precursor of the AA protein deposited in tissues, seems to be a prerequisite to the development of inflammatory (AA) amyloidosis. Genetic factors have however been recently emphasized. Among persistent or emerging causes of AA amyloidosis, hereditary periodic fever syndromes also known as auto-inflammatory syndromes are a group of diseases characterised by intermittent bouts of clinical inflammation with focal organ involvement mainly: abdomen, musculoskeletal system and skin. The most frequent is familial Mediterranean fever which affects patients of Mediterranean descent all over the world. Three other types have been recently clinically as well as genetically characterised. A thorough diagnosis is warranted, as clinical and therapeutic management is specific for each of these diseases.

  2. Amyloidosis: A cancer-derived paraproteinemia and kidney involvement.

    PubMed

    Małyszko, Jolanta; Kozłowska, Klaudia; Małyszko, Jacek Stanisław

    2017-01-30

    Amyloidosis is the general term describing the extracellular tissue deposition of fibrils composed of low molecular weight subunits of a variety of proteins. There are multiple different human protein precursors of amyloid fibrils. Amyloid deposits are stained using Congo Red and show typical apple-green birefringence in polarized microscopy. Nowadays, a novel technique LMD/MS technique or laser microdissection combined with mass spectrometry help to diagnose amyloidosis. Amyloidosis of the kidney is typically classified as being either one of two types: AL or AA. Less common is the hereditary amyloidosis. Clinical manifestations are usually determined by the type of precursor protein, the tissue distribution, and the amount of amyloid deposition. Renal manifestation is usually present as asymptomatic proteinuria or clinically apparent nephrotic syndrome. In some patients clinical presentation include impaired kidney function with no or mild proteinuria. Patients with renal amyloidosis who progress to end-stage renal disease (ESRD) can be treated with either dialysis or renal transplantation. Diagnosis of amyloidosis is prerequisite to consider treatment options to avoid unnecessary chemotherapy. Treatment of amyloidosis is aimed at decreasing the precursors of fibrillary proteins and/or decrease in synthesis/deposition of amyloid fibrils. It depends upon the type of amyloidosis and cause of excess fibril production.

  3. Amyloidosis in six dairy cows.

    PubMed

    Johnson, R; Jamison, K

    1984-12-15

    Amyloidosis was diagnosed in 6 Holstein cows that were examined because of chronic intractable diarrhea. Besides diarrhea, the chief finding was a nephrotic-like syndrome, in that there was edema, hypoproteinemia, and proteinuria. Other consistent clinicopathologic abnormalities were hyperfibrinogenemia, low-normal serum calcium content or hypocalcemia, hypomagnesemia, prolonged bromosulphalein half time, high serum urea nitrogen concentration, high serum creatinine concentration, and low urine specific gravity. Foci of inflammation including traumatic reticuloperitonitis, traumatic pericarditis, salpingitis, mastitis, and metritis were found. There was histologic evidence of amyloid in the kidneys, liver, adrenal glands, and spleen. The iodine-sulfuric acid test for amyloid was positive in 2 cows. The Congo red dye test for amyloid was positive in 2 other cows. In spite of supportive care, all the cows either died naturally or were euthanatized. Because foci of inflammation were found in each cow, it was concluded that the most likely classification of amyloidosis in these cases would be reactive systemic amyloidosis and that the major amyloid fibril protein would be type AA.

  4. [Systemic Buschke's scleredema with cardiomyopathy, monoclonal IgG kappa gammopathy and amyloidosis. Case report with autopsy].

    PubMed

    Paz, R A; Badra, R E; Martí, H M; Maxit, M J

    1998-01-01

    A 73 year old retired truck driver and blacksmith was studied in June 1996 for thoracic pain and was diagnosed as acute pericarditis which responded well to steroid treatment. In January 1997, he noted swelling of the abdominal skin, genitalia and limbs, sparing the feet. He was euthyroid, did not have evidence of diabetes or a Raynaud's phenomenon. His proteinogram showed an IgG-Kappa monoclonal paraprotein M component, 1.31 g/oo. TSH and tetraiodotironine were normal; ESR 16 mm in the first hour. As he did not respond to treatment he was referred to our hospital in March 1997. On physical examination the most relevant findings were a non-pitting edema of the abdomen and lower limbs, sparing the feet. An echocardiogram was consistent with an infiltrative cardiomyopathy. Soon after his hospitalization his condition worsened suddenly with severe bradicardia (28/minute) due to a junctional rhythm and righ bundle branch block. He suffered a cardiac arrest and died. The autopsy findings favoured the diagnosis of systemic scleredema adultorum of Buschke. Amyloid deposits were also found although not abundant, with a similar distribution except in the skin. In this article the clinical and autopsy findings are presented in a patient showing coexistence of systemic Buschke's scleredema with an infiltrative cardiomyopathy, IgG Kappa gammopathy and amyloidosis.

  5. /Cu-Al System

    NASA Astrophysics Data System (ADS)

    Kish, Orel; Froumin, Natalya; Aizenshtein, Michael; Frage, Nachum

    2014-05-01

    Wettability and interfacial interaction of the Ta2O5/Cu-Al system were studied. Pure Cu does not wet the Ta2O5 substrate, and improved spreading is achieved when relatively a high fraction of the active element (~40 at.% Al) was added. The Al2O3 and AlTaO4 phases were observed at the Ta2O5/Cu-Al interface. A thermodynamic evaluation allowed us to suggest that the lack of wetting bellow 40 at.% Al is due to the presence of a native oxide, which covers the drop. The conditions of the native oxide decomposition and the formation of the volatile Al2O suboxide strongly depend on the vacuum level during sessile drop experiments and the composition of the Cu-Al alloy. In our case, Al contents greater than 40% provides thermodynamic conditions for the formation of Al2O (as a result of Al reaction with Al2O3) and the drop spreading. It was suggested that the final contact angle in the Ta2O5/Cu-Al system (50°) is determined by Ta adsorption on the newly formed alumina interlayer.

  6. Cell-to-cell transfer of SAA1 protein in a cell culture model of systemic AA amyloidosis.

    PubMed

    Claus, Stephanie; Puscalau-Girtu, Ioana; Walther, Paul; Syrovets, Tatiana; Simmet, Thomas; Haupt, Christian; Fändrich, Marcus

    2017-03-31

    Systemic AA amyloidosis arises from the misfolding of serum amyloid A1 (SAA1) protein and the deposition of AA amyloid fibrils at multiple sites within the body. Previous research already established that mononuclear phagocytes are crucial for the formation of the deposits in vivo and exposure of cultures of such cells to SAA1 protein induces the formation of amyloid deposits within the culture dish. In this study we show that both non-fibrillar and fibrillar SAA1 protein can be readily transferred between cultured J774A.1 cells, a widely used model of mononuclear phagocytes. We find that the exchange is generally faster with non-fibrillar SAA1 protein than with fibrils. Exchange is blocked if cells are separated by a membrane, while increasing the volume of cell culture medium had only small effects on the observed exchange efficiency. Taken together with scanning electron microscopy showing the presence of the respective types of physical interactions between the cultured cells, we conclude that the transfer of SAA1 protein depends on direct cell-to-cell contacts or tunneling nanotubes.

  7. Cell-to-cell transfer of SAA1 protein in a cell culture model of systemic AA amyloidosis

    PubMed Central

    Claus, Stephanie; Puscalau-Girtu, Ioana; Walther, Paul; Syrovets, Tatiana; Simmet, Thomas; Haupt, Christian; Fändrich, Marcus

    2017-01-01

    Systemic AA amyloidosis arises from the misfolding of serum amyloid A1 (SAA1) protein and the deposition of AA amyloid fibrils at multiple sites within the body. Previous research already established that mononuclear phagocytes are crucial for the formation of the deposits in vivo and exposure of cultures of such cells to SAA1 protein induces the formation of amyloid deposits within the culture dish. In this study we show that both non-fibrillar and fibrillar SAA1 protein can be readily transferred between cultured J774A.1 cells, a widely used model of mononuclear phagocytes. We find that the exchange is generally faster with non-fibrillar SAA1 protein than with fibrils. Exchange is blocked if cells are separated by a membrane, while increasing the volume of cell culture medium had only small effects on the observed exchange efficiency. Taken together with scanning electron microscopy showing the presence of the respective types of physical interactions between the cultured cells, we conclude that the transfer of SAA1 protein depends on direct cell-to-cell contacts or tunneling nanotubes. PMID:28361953

  8. Primarily isolated hepatic involvement of amyloidosis

    PubMed Central

    Ye, Lei; Shi, Hui; Wu, Hui-Min; Wang, Fang-Yu

    2016-01-01

    Abstract Background: Amyloidosis is particularly difficult to diagnose because the signs and symptoms are subtle. Additionally, there are no specific imaging or laboratory tests, except histopathology. Although it is considered to be a systemic disorder, a small portion of cases may be localized. Introduction of the case: A 54-year-old man presented with nonspecific symptoms (jaundice and back pruritus). Biochemical tests showed a high level of bilirubin and elevated serum tumor markers (CA19–9 and CA125). Routine imaging showed hepatomegaly without heterogeneous enhancement. Liver biopsy confirmed the diagnosis of hepatic amyloidosis. No cardiac or renal involvement was found. The patient accepted treatment involving oral chemotherapy. Conclusion: A rare and unique presentation of hepatic amyloidosis was highlighted in this case. PMID:28033255

  9. A Case of Conjunctival Amyloidosis with Repeated Subconjunctival Hemorrhage

    PubMed Central

    Ando, Takaaki; Saito, Mamiko; Tawada, Ayako; Yotsukura, Jiro; Yamamoto, Shuichi

    2017-01-01

    Conjunctival amyloidosis is a very rare disease, and its presence may be a sign of systemic amyloidosis. We present our ocular and systemic findings in a patient with conjunctival amyloidosis. A 43-year-old man had repeated subconjunctival hemorrhages (SCHs) for two years and was referred to the Chiba University Hospital. He had comprehensive ophthalmological and systemic examinations to determine the cause of the SCHs. His visual acuities were 1.2 OU, and the intraocular pressures were 13-14 mmHg OU. Magnetic resonance imaging was normal. Initially, the SCH was the only abnormality. After 3 months, the SCH had partially cleared, and a pink mass was detected in the superior area of the subconjunctiva. Partial biopsy and histopathological examinations showed a greenish birefringence and dichroism under polarized light illumination. The birefringence was located in amyloid fibers. Immunofixation electrophoresis detected λ-light chain abnormality in the ocular biopsy specimen but systemic examinations did not find any lesions. Multiple myeloma was ruled out, and the patient is being followed closely to detect any early signs of systemic amyloidosis. Because repeated SCHs might be initial signs of systemic amyloidosis, patients with conjunctival amyloidosis should be comprehensively examined for systemic amyloidosis because of its poor life prognosis. PMID:28326212

  10. AL Amyloidosis and Agent Orange

    MedlinePlus

    ... Z) Hepatitis HIV Mental Health Mental Health Home Suicide Prevention Substance Abuse Military Sexual Trauma PTSD Research ( ... eligible for a free Agent Orange registry health exam . Surviving spouses, dependent children and dependent parents of ...

  11. Systemic senile amyloidosis. Identification of a new prealbumin (transthyretin) variant in cardiac tissue: immunologic and biochemical similarity to one form of familial amyloidotic polyneuropathy.

    PubMed Central

    Gorevic, P D; Prelli, F C; Wright, J; Pras, M; Frangione, B

    1989-01-01

    Isolated amyloid fibrils from three cases of systemic senile amyloidosis (SSA) contained subunit proteins with molecular masses of 14 (10-20%), 10-12 (60-80%), and 5-6 kD (5-10%) when fractionated under reducing and dissociating conditions. This grouping was identical to that seen in SKO, a case of familial amyloidotic polyneuropathy (FAP) studied earlier. Amino acid sequencing confirmed that SSA subunit proteins were in fact prealbumin (transthyretin). Complete sequence analysis of one SSA preparation revealed the presence of a new variant Pa (TTr) molecule with a single amino acid substitution of isoleucine for valine at position 122. Further studies used an antiserum specific for SKO IV, a subunit protein of SKO previously shown to correspond to carboxy-terminal 78 residues (positions 49-127) of (TTr). Anti-SKO IV reacted with SSA in tissue at equivalent dilutions to anti-Pa (TTr) and with the 10-12-kD fraction of SSA on Western blots; reactivity was blocked by SKO IV, but not by Pa (TTr). SSA is a form of systemic amyloidosis caused by tissue deposition of Pa (TTr) and its fragments, with shared conformational or subunit antigenicity to at least one form of FAP. Identification of a new variant Pa (TTr) molecule in one case suggests further that SSA may be a genetically determined disease expressed late in life. Images PMID:2646319

  12. “Occult” mastocytosis with activating c‐kit point mutation evolving into systemic mastocytosis associated with plasma cell myeloma and secondary amyloidosis

    PubMed Central

    Sotlar, K; Saeger, W; Stellmacher, F; Stahmer, J; Jäckle, S; Valent, P; Horny, H‐P

    2006-01-01

    A case of a 70‐year‐old man presenting with exsudative enteropathy due to light‐chain‐associated amyloidosis is reported. The diagnosis of systemic mastocytosis associated with IgG/λ plasma cell myeloma and secondary generalised amyloidosis was carried out by morphological evaluation of bone marrow biopsy. The c‐kit point mutation D816Y was detected by molecular analysis. Two years before, a cystadenolymphoma of the left parotid gland had been removed. A moderate increase of loosely scattered spindle‐shaped mast cells, a subpopulation of them expressing CD25, an antigen that is not expressed by normal or reactive mast cells, was shown by retrospective analysis carried out on an intraparotideal lymph node. The c‐kit mutation D816Y was shown by the molecular analysis of the lymph node. In summary, the notion that systemic mastocytosis may very rarely be associated with B cell neoplasms and that neoplastic mast cell infiltrates may be obscured because of only a minimal increase of atypical mast cells, which are outnumbered by other non‐neoplastic cells in the same tissue, is supported by this case. This finding was preliminarily termed “occult” mastocytosis. PMID:16873565

  13. 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

  14. Amyloidosis and POEMS Syndrome

    PubMed Central

    Chee, Cheng E. M.D.; Dispenzieri, Angela M.D.; Gertz, Morie A. M.D

    2010-01-01

    Importance of the field Treatment options for amyloidosis and POEMS have rapidly increased in the past years, but many patients are diagnosed late in the disease course and do not receive state of the art therapy Areas covered in this review Stem cell transplantation and novel agents have widened the chemotherapy alternatives available in these disorders and combinations of novel agents with high dose therapy further improve treatment opotions. This review covers the main areas of debate in the optimal treatment amyloidosis and POEMS patients, focusing on the implications for everyday clinical practice and management strategies published in the past 36 months. What the reader will gain Insights into treatment strategies are provided in the review. Keys to early recognition of the syndromes are reviewed Take home message With early diagnosis most patients are therapy candidates. New agents and new application of stem cell transplantation have dramatically improved outcomes for these previously uniformly poor prognosis disorders. PMID:20426710

  15. Long-term event-free and overall survival after risk-adapted melphalan and SCT for systemic light chain amyloidosis

    PubMed Central

    Landau, H; Smith, M; Landry, C; Chou, J F; Devlin, S M; Hassoun, H; Bello, C; Giralt, S; Comenzo, R L

    2017-01-01

    Stem cell transplantation (SCT), an effective therapy for amyloid light chain (AL) amyloidosis patients, is associated with low treatment-related mortality (TRM) with appropriate patient selection and risk-adapted dosing of melphalan (RA-SCT). Consolidation after SCT increases hematologic complete response (CR) rates and may improve overall survival (OS) for patients with AL amyloidosis. PMID:27560108

  16. 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.

  17. Cardiac amyloidosis in a heart transplant patient - A case report and retrospective analysis of amyloidosis evolution

    PubMed Central

    Kintsler, Svetlana; Jäkel, Jörg; Brandenburg, Vincent; Kersten, Katrin; Knuechel, Ruth; Röcken, Christoph

    2015-01-01

    Summary Cardiac amyloidosis is a very rare cause of heart failure in heart transplant recipients but an important differential diagnosis in cases of progressive cardiac failure. We report a 72-year-old male patient with the diagnosis of senile systemic amyloidosis (SSA) in a transplanted heart 15 years after transplantation by the initial diagnosis of the dilated cardiomyopathy. Additionally performed immunohistochemical analysis with anti-transthyretin antibody of the cardiac biopsies of the last 15 years enabled the possibility to show the evolution of this disease with characteristic biphasic pattern. PMID:25674390

  18. [Effective dimethyl sulfoxide (DMSO) occlusive dressing technique for amyloidosis of the urinary bladder].

    PubMed

    Hasegawa, Yoshihiro; Kanda, Hideki; Miki, Manabu; Masui, Satoru; Yoshio, Yuko; Yamada, Yasushi; Soga, Norihito; Arima, Kiminobu; Sugimura, Yoshiki

    2013-10-01

    A 48-year-old married woman complaining of macroscopic hematuria and cystitis symptom was admitted to our institute. Flexible cystoscopy revealed many yellowish, nodular masses at the paries posterior of the urinary bladder, and cold-punch biopsy proved it to be amyloidosis. Serum amyloid protein A (SAA) was high, and suggested systemic amyloidosis. Renal biopsy and colon fiberscopy did not reveal any abnormalities. We therefore diagnosed a primary localized amyloidosis of the urinary bladder. Transurethral resection and dimethyl sulfoxide (DMSO) infusion therapy are used to treat amyloidosis of the urinary bladder. However there is no definite cure for amyloidosis of the urinary bladder. Therefore we selected DMSO occlusive dressing technique therapy. After 5 years of therapy, there was no evidence of a recurrence of amyloidosis.

  19. 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

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

    PubMed Central

    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

  1. 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

  2. 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

    2016-01-21

    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.

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

    PubMed

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

    2015-11-06

    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

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

    PubMed Central

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

    2012-01-01

    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. PMID:22833808

  5. Thermal Stability Threshold for Amyloid Formation in Light Chain Amyloidosis

    PubMed Central

    Poshusta, Tanya L.; Katoh, Nagaaki; Gertz, Morie A.; Dispenzieri, Angela; Ramirez-Alvarado, Marina

    2013-01-01

    Light chain (AL) amyloidosis is a devastating disease characterized by amyloid deposits formed by immunoglobulin light chains. Current available treatments involve conventional chemotherapy and autologous stem cell transplant. We have recently concluded a phase III trial comparing these two treatments. AL amyloidosis patients who achieve hematological complete response (CR) do not necessarily achieve organ response regardless of the treatment they received. In order to investigate the possible correlation between amyloid formation kinetics and organ response, we selected AL amyloidosis patients from the trial with kidney involvement and CR after treatment. Six patients were selected and their monoclonal immunoglobulin light chains were characterized. The proteins showed differences in their stability and their kinetics of amyloid formation. A correlation was detected at pH 7.4, showing that less stable proteins are more likely to form amyloid fibrils. AL-T03 is too unstable to form amyloid fibrils at pH 7.4. This protein was found in the only patient in the study that had organ response, suggesting that partially folded species are required for amyloid formation to occur in AL amyloidosis. PMID:24248061

  6. Case report: isolated cardiac amyloidosis: an enigma unravelled.

    PubMed

    Khalid, Umair; Awar, Omar; Verstovsek, Gordana; Cheong, Benjamin; Yellapragada, Sarvari Venkata; Jneid, Hani; Deswal, Anita; Virani, Salim S

    2015-01-01

    Amyloidosis is a rare, multisystem disease characterized by deposition of fibrils in extracellular tissue involving kidney, liver, heart, autonomic nervous system, and several other organs. This report discusses a 75-year-old male who presented with worsening dyspnea on exertion, orthopnea, and lower-extremity edema. On physical exam, he had elevated jugular venous pressure and lower-extremity edema. Electrocardiogram depicted low voltage in limb leads and a prolonged PR interval. Echocardiogram revealed left ventricular hypertrophy, severe biatrial dilatation, and restrictive filling physiology. Coronary angiography showed absence of significant epicardial coronary artery disease. On right heart catheterization, a "dip-and-plateau sign" was noted on right ventricular pressure tracings. A diagnosis of cardiac amyloidosis was considered, but a complete hematology work-up for systemic amyloidosis was negative. Cardiac magnetic resonance imaging was pursued, showing delayed gadolinium enhancement, and this ultimately led to the myocardial biopsy confirming the diagnosis of isolated cardiac amyloidosis. Further genetic analyses confirmed isolated cardiac amyloid caused by mutant transthyretin protein (Val-122-Ile). Isolated cardiac amyloidosis is an extremely rare entity, and diagnosis may be difficult despite the use of multimodality imaging. If the index of suspicion is high, then myocardial biopsy should be considered.

  7. Case Report: Isolated Cardiac Amyloidosis: An Enigma Unravelled

    PubMed Central

    Khalid, Umair; Awar, Omar; Verstovsek, Gordana; Cheong, Benjamin; Yellapragada, Sarvari Venkata; Jneid, Hani; Deswal, Anita; Virani, Salim S.

    2015-01-01

    Amyloidosis is a rare, multisystem disease characterized by deposition of fibrils in extracellular tissue involving kidney, liver, heart, autonomic nervous system, and several other organs. This report discusses a 75-year-old male who presented with worsening dyspnea on exertion, orthopnea, and lower-extremity edema. On physical exam, he had elevated jugular venous pressure and lowerextremity edema. Electrocardiogram depicted low voltage in limb leads and a prolonged PR interval. Echocardiogram revealed left ventricular hypertrophy, severe biatrial dilatation, and restrictive filling physiology. Coronary angiography showed absence of significant epicardial coronary artery disease. On right heart catheterization, a “dip-and-plateau sign” was noted on right ventricular pressure tracings. A diagnosis of cardiac amyloidosis was considered, but a complete hematology work-up for systemic amyloidosis was negative. Cardiac magnetic resonance imaging was pursued, showing delayed gadolinium enhancement, and this ultimately led to the myocardial biopsy confirming the diagnosis of isolated cardiac amyloidosis. Further genetic analyses confirmed isolated cardiac amyloid caused by mutant transthyretin protein (Val-122-Ile). Isolated cardiac amyloidosis is an extremely rare entity, and diagnosis may be difficult despite the use of multimodality imaging. If the index of suspicion is high, then myocardial biopsy should be considered. PMID:25793032

  8. 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

  9. Etiology of amyloidosis determines myocardial 99mTc-DPD uptake in amyloidotic cardiomyopathy.

    PubMed

    Longhi, Simone; Bonfiglioli, Rachele; Obici, Laura; Gagliardi, Christian; Milandri, Agnese; Lorenzini, Massimiliano; Guidalotti, Pier Luigi; Merlini, Giampaolo; Rapezzi, Claudio

    2015-05-01

    Tc-DPD (Tc-3,3-diphosphono-1,2-propanodicarboxylic acid) has a high affinity for transthyretin (TTR)-infiltrated myocardium, allowing a differential diagnosis with light chain cardiac amyloidosis and other nonamyloidotic cardiomyopathies with a hypertrophic phenotype, in which myocardial tracer uptake is low or absent. Myocardial bone tracer uptake in the rarer forms of amyloidosis (eg, apolipoprotein-related) has been rarely studied. We present 4 cases of cardiac amyloidosis that underwent Tc-DPD scintigraphy; myocardial DPD uptake was present in patients with ATTR, wtTTR and apolipoprotein AI and negative in cases with AL and apolipoprotein AII-related disease.

  10. Extracellular volume quantification by dynamic equilibrium cardiac computed tomography in cardiac amyloidosis

    PubMed Central

    Treibel, Thomas A.; Bandula, Steve; Fontana, Marianna; White, Steven K.; Gilbertson, Janet A.; Herrey, Anna S.; Gillmore, Julian D.; Punwani, Shonit; Hawkins, Philip N.; Taylor, Stuart A.; Moon, James C.

    2015-01-01

    Background Cardiac involvement determines outcome in patients with systemic amyloidosis. There is major unmet need for quantification of cardiac amyloid burden, which is currently only met in part through semi-quantitative bone scintigraphy or Cardiovascular Magnetic Resonance (CMR), which measures ECVCMR. Other accessible tests are needed. Objectives To develop cardiac computed tomography to diagnose and quantify cardiac amyloidosis by measuring the myocardial Extracellular Volume, ECVCT. Methods Twenty-six patients (21 male, 64 ± 14 years) with a biopsy-proven systemic amyloidosis (ATTR n = 18; AL n = 8) were compared with twenty-seven patients (19 male, 68 ± 8 years) with severe aortic stenosis (AS). All patients had undergone echocardiography, bone scintigraphy, NT-pro-BNP measurement and EQ-CMR. Dynamic Equilibrium CT (DynEQ-CT) was performed using a prospectively gated cardiac scan prior to and after (5 and 15 minutes) a standard Iodixanol (1 ml/kg) bolus to measure ECVCT. ECVCT was compared to the reference ECVCMR and conventional amyloid measures: bone scintigraphy and clinical markers of cardiac amyloid severity (NT-pro-BNP, Troponin, LVEF, LV mass, LA and RA area). Results ECVCT and ECVCMR results were well correlated (r2 = 0.85 vs r2 = 0.74 for 5 and 15 minutes post bolus respectively). ECVCT was higher in amyloidosis than AS (0.54 ± 0.11 vs 0.28 ± 0.04, p<0.001) with no overlap. ECVCT tracked clinical markers of cardiac amyloid severity (NT-pro-BNP, Troponin, LVEF, LV mass, LA and RA area), and bone scintigraphy amyloid burden (p<0.001). Conclusion Dynamic Equilibrium CT, a 5 minute contrast-enhanced gated cardiac CT, has potential for non-invasive diagnosis and quantification of cardiac amyloidosis. PMID:26209459

  11. Amyloid A amyloidosis secondary to rheumatoid arthritis: pathophysiology and treatments.

    PubMed

    Nakamura, Tadashi

    2011-01-01

    The introduction of biological therapies targeting specific inflammatory mediators revolutionised the treatment of rheumatoid arthritis (RA). Targeting key components of the immune system allows efficient suppression of the pathological inflammatory cascade that leads to RA symptoms and subsequent joint destruction. Reactive amyloid A (AA) amyloidosis, one of the most severe complications of RA, is a serious, potentially life-threatening disorder caused by deposition of AA amyloid fibrils in multiple organs. These AA amyloid fibrils derive from the circulatory acute-phase reactant serum amyloid A protein (SAA), and may be controlled by treatment. New biologics may permit AA amyloidosis secondary to RA to become a treatable, manageable disease. Rheumatologists, when diagnosing and treating patients with AA amyloidosis secondary to RA, must understand the pathophysiology and clinical factors related to development and progression of the disease, including genetic predisposition and biological versatility of SAA.

  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. Hereditary Amyloidosis with Recurrent Lung Infiltrates

    PubMed Central

    Revelo, Alberto E.; Magaspi, Crischelle; Maguire, George; Aronow, Wilbert S.

    2016-01-01

    Patient: Male, 51 Final Diagnosis: Familial amyloidotic polyneuropathy with lung involvement Symptoms: Cough • dyspnea • lethargy Medication: Diflunisal Clinical Procedure: Fiberoptic bronchoscopy with trans-bronchial biopsy Specialty: Pulmonary Medicine Objective: Rare co-existance of disease or patholog Background: Amyloidosis is a protein conformational disorder characterized by extracellular deposition of amyloid fibrils in extracellular tissue. Lung involvement is most commonly caused by secondary AL amyloidosis. The familial autosomal-dominant senile transthyretin (ATTR) disease manifests mainly as polyneuropathy and restrictive cardiomyopathy denoting the name familial amyloidotic polyneuropathy (FAP). Rarely, this form manifests with clinical and radiologically relevant respiratory tract symptoms and lung involvement. Case Report: A 51-year-old male former smoker presented with progressive lower-extremity weakness of several months’ duration. He was ultimately diagnosed with chronic demyelinating polyneuropathy and treated with intravenous immunoglobulin therapy. Subsequently, he was admitted with heart failure symptoms and pulmonary infiltrates and his echocardiogram showed a ‘myocardial speckled pattern’, prompting an endomyocardial biopsy, which showed transthyretin amyloid deposition. He was started on diflunisal. Additionally, serial radiographic imaging of his chest over 3 different admissions for cough, dyspnea, hypoxemia, and lethargy demonstrated recurrent pulmonary infiltrates. A fiberoptic bronchoscopy with trans-bronchial biopsies revealed amyloid deposition in the lung tissue. Conclusions: The clinical presentation of recurrent or persistent pulmonary symptoms and fleeting infiltrates on imaging in a patient with familial amyloidotic polyneuropathy is not common; when present, it should raise the suspicion of respiratory tract involvement. PMID:27872470

  14. 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.

  15. Primary diffuse alveolar septal amyloidosis.

    PubMed Central

    Poh, S C; Tjia, T S; Seah, H C

    1975-01-01

    The case is reported of a 61-year-old man with primary diffuse alveolar septal pulmonary amyloidosis. Amyloid infiltration of the heart and other organs was also observed. The clinical findings and laboratory investigations reveal features characteristic of defective gas transfer with pulmonary oedema due to left ventricular failure from myocardial involvement. Images PMID:1179316

  16. Clinical Presentation of Tubulointerstitial Nephritis Caused by Amyloid Light-chain Amyloidosis in a Patient with Sjögren's Syndrome

    PubMed Central

    Inoue, Reiko; Fujigaki, Yoshihide; Kobayashi, Kana; Tamura, Yoshifuru; Ota, Tatsuru; Shibata, Shigeru; Ishida, Tsuyoshi; Kondo, Fukuo; Yamaguchi, Yutaka; Uchida, Shunya

    2017-01-01

    We report a 70-year-old woman with Sjögren's syndrome who had severe renal dysfunction with mild proteinuria and elevated urinary low-molecular-weight proteins. Based on these clinical presentations, interstitial nephritis due to Sjögren's syndrome was strongly suspected. Unexpectedly, renal pathology revealed amyloid light-chain (AL) lambda-type depositions predominantly in the vasculatures with severe tubulointerstitial damage. Concentrated urine immunofixation was positive for Bence Jones lambda-type monoclonal proteins. Given the involvement in other organs, systemic AL amyloidosis was diagnosed. The patient underwent chemotherapy, but hemodialysis was ultimately instituted. It should be remembered that renal amyloidosis occurs as a clinical presentation of interstitial nephritis. PMID:28202864

  17. 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.

  18. 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.

  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. 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

  1. Intestinal amyloidosis: two cases with different patterns of clinical and imaging presentation.

    PubMed

    Mainenti, Pier-Paolo; Segreto, Sabrina; Mancini, Marcello; Rispo, Antonio; Cozzolino, Immacolata; Masone, Stefania; Rinaldi, Ciro-Roberto; Nardone, Gerardo; Salvatore, Marco

    2010-05-28

    The involvement of the small bowel in systemic forms of amyloidosis may be diffuse or very rarely focal. Some cases of focal amyloidomas of the duodenum and jejunum without extraintestinal manifestations have been reported. The focal amyloidomas consisted of extensive amyloid infiltration of the entire intestinal wall thickness. Radiological barium studies, ultrasound and computed tomography (CT) patterns of diffuse small bowel amyloidosis have been described: the signs are non-specific and may include small-bowel dilatation, symmetric bowel wall thickening, mesenteric infiltration, and mesenteric adenopathy. No data are available about the positron emission tomography (PET)/CT and magnetic resonance imaging (MRI) patterns of intestinal amyloidosis. We report two cases of small bowel amyloidosis: the former characterized by focal deposition of amyloid proteins exclusively within blood vessel walls of the terminal ileum, the latter characterized by diffuse intestinal involvement observed on MRI and PET/CT studies.

  2. Intestinal amyloidosis: Two cases with different patterns of clinical and imaging presentation

    PubMed Central

    Mainenti, Pier Paolo; Segreto, Sabrina; Mancini, Marcello; Rispo, Antonio; Cozzolino, Immacolata; Masone, Stefania; Rinaldi, Ciro Roberto; Nardone, Gerardo; Salvatore, Marco

    2010-01-01

    The involvement of the small bowel in systemic forms of amyloidosis may be diffuse or very rarely focal. Some cases of focal amyloidomas of the duodenum and jejunum without extraintestinal manifestations have been reported. The focal amyloidomas consisted of extensive amyloid infiltration of the entire intestinal wall thickness. Radiological barium studies, ultrasound and computed tomography (CT) patterns of diffuse small bowel amyloidosis have been described: the signs are non-specific and may include small-bowel dilatation, symmetric bowel wall thickening, mesenteric infiltration, and mesenteric adenopathy. No data are available about the positron emission tomography (PET)/CT and magnetic resonance imaging (MRI) patterns of intestinal amyloidosis. We report two cases of small bowel amyloidosis: the former characterized by focal deposition of amyloid proteins exclusively within blood vessel walls of the terminal ileum, the latter characterized by diffuse intestinal involvement observed on MRI and PET/CT studies. PMID:20503459

  3. Association of Skin with the Pathogenesis and Treatment of Neurodegenerative Amyloidosis

    PubMed Central

    Clos, Audra L.; Kayed, Rakez; Lasagna-Reeves, Cristian A.

    2012-01-01

    Amyloidosis are a large group of conformational diseases characterized by abnormal protein folding and assembly which results in the accumulation of insoluble protein aggregates that may accumulate systemically or locally in certain organs or tissue. In local amyloidosis, amyloid deposits are restricted to a particular organ or tissue. Alzheimer’s, Parkinson’s disease, and amyotrophic lateral sclerosis are some examples of neurodegenerative amyloidosis. Local manifestation of protein aggregation in the skin has also been reported. Brain and skin are highly connected at a physiological and pathological level. Recently several studies demonstrated a strong connection between brain and skin in different amyloid diseases. In the present review, we discuss the relevance of the “brain–skin connection” in different neurodegenerative amyloidosis, not only at the pathological level, but also as a strategy for the treatment of these diseases. PMID:22319507

  4. [Amyloidosis associated with chronic granulomatous disease in a patient with a renal transplant and recurrent urinary tract infections].

    PubMed

    Peces, R; Ablanedo, P; Seco, M

    2002-01-01

    Chronic granulomatous disease is a group of syndromes which share a defect in a component of the phagocyte NADPH-oxidase complex. Without this enzyme activity, phagocytic cells cannot produce superoxide, peroxide, and other potent microbicidal radicals, and are less able to kill ingested pathogens. The clinical picture is characterised by recurrent life-threatening bacterial and fungal infections and abnormal tissue granuloma formation. On the other hand, amyloidosis is a systemic disease with renal involvement occurring in the majority of cases. Recurrent amyloidosis is a rare but well documented event in renal transplant recipients. However, graft loss secondary to amyloidosis has been noted infrequently. In addition, de novo amyloidosis has not been previously associated with graft loss. We report here a renal transplant recipient with chronic granulomatous disease and history of recurrent urinary tract infections, who developed nephrotic syndrome and progressive renal insufficiency secondary to de novo AA amyloidosis leading to graft loss 66 months after transplantation.

  5. Tracheobronchial Amyloidosis-A Series of Two Cases

    PubMed Central

    Raiyani, Palak D; Vyas, Sunil S

    2014-01-01

    Amyloidosis is a rare disorder characterised by accumulation of insoluble fibrillar proteins in extracellular space. Respiratory amyloidosis presents as two tracheobronchial forms (local and diffuse) and two parenchymal forms (nodular and diffuse), of which diffuse tracheobronchial amyloidosis is the least common. We herein present two cases of tracheobronchial amyloidosis. PMID:25386446

  6. Primary amyloidosis and severe intrahepatic cholestatic jaundice.

    PubMed Central

    Peters, R A; Koukoulis, G; Gimson, A; Portmann, B; Westaby, D; Williams, R

    1994-01-01

    Liver involvement in systemic amyloidosis is frequent but is rarely of clinical importance. Five patients with severe cholestatic jaundice are described and an additional 20 from published reports are reviewed. The most frequent presenting symptoms were lethargy and abdominal pain, which were present for a median of 11 months before the onset of jaundice. Hepatomegaly, usually marked, was present in 92%, with ascites in 56% of the cases. The serum bilirubin concentration was noticeably high and the serum globulin low. Histology of the liver showed considerable perisinusoidal deposition with a slight predilection for the periportal area. Two patients presented with predominant centrilobular deposition. Congo red staining was not uniformly positive. A variety of treatment regimens was tried but median survival was only three months from the onset of jaundice. PMID:7959246

  7. 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.

  8. [Hereditary cerebral hemorrhage. Dementia with cystatin C amyloidosis].

    PubMed

    Blöndal, H; Guomundsson, G; Benedikz, E; Jóhannesson, G

    1990-01-01

    Nineteen cases of hereditary cystatin C amyloidosis with cerebral haemorrhage are described. The first haemorrhage occurred between the ages of 20 and 41 years and the period of survival varied from 10 days to 23 years after the first insult. Progressive dementia was a striking clinical symptom in 17 of the patients and in two cases dementia was the first sign. At the last examination severe dementia and pronounced pathological EEG were established in the majority of the patients. Infiltration of amyloid substance positive for anti-cystatin C was found in the proximity of the blood vessels and in their walls. Lesions in the cerebral microvascular system together with haemorrhages and infarcts caused thereby were considered to be an adequate explanation of the dementia in these patients. In view of the discovery of amyloid discharges in tissues outside the CNS it is adjudged more correct to use the name Hereditary Cystatin C Amyloidosis (HCCA).

  9. Renal amyloidosis. Evaluation by gallium imaging

    SciTech Connect

    Lee, V.W.; Skinner, M.; Cohen, A.S.; Ngai, S.; Peng, T.T.

    1986-09-01

    A study has been performed to evaluate the efficacy of gallium imaging in the detection of renal amyloidosis. Ten of the 11 patients who had biopsy-proven renal amyloidosis demonstrated marked uptake in both kidneys. One patient revealed moderate gallium uptake in his kidneys. None of the patients had underlying renal or extrarenal pathology other than amyloidosis, which could account for renal gallium uptake (renal infection, neoplasm, hepatic failure or frequent blood transfusions). Four patients also had extrarenal foci of abnormal gallium uptake, suggesting other sites of amyloid deposits. Our data strongly suggest that gallium imaging has a high sensitivity for detection of renal amyloidosis. Its specificity is enhanced significantly by careful review of the clinical history to exclude other known causes of renal gallium uptake. Potentially, gallium imaging may be used to monitor the progress of patients under experimental therapy.

  10. Renal amyloidosis in a drug abuser.

    PubMed

    Tan, A U; Cohen, A H; Levine, B S

    1995-03-01

    Drug abusers, particularly those who inject drugs s.c. ("skin popping"), may develop amyloidosis. Chronic infections are thought to play a pathogenetic role in this setting. A patient is presented who had a history of "skin popping" cocaine and heroin and developed nephrotic syndrome, with an elevated serum creatinine and a creatinine clearance of 61 mL/min. Renal biopsy demonstrated amyloidosis. Treatment with colchicine was initiated, and proteinuria decreased to near normal levels after 12 months. Concomitant with the decrease in proteinuria, creatinine clearance improved, although a repeat renal biopsy failed to show any significant improvement in amyloid burden. These observations suggest that colchicine may be a useful treatment in reversing the proteinuria of renal amyloidosis associated with drug abuse. Furthermore, clinical improvement may occur before any demonstrable regression in the amyloidosis.

  11. Genetics Home Reference: primary localized cutaneous amyloidosis

    MedlinePlus

    ... AP, Hans-Filho G, Sakuma TH, Lai-Cheong J, Clements S, Odashiro M, Odashiro DN, Hans-Neto G, ... mutations underlie familial primary localized cutaneous amyloidosis. Am J Hum Genet. 2008 Jan;82(1):73-80. ...

  12. Primary amyloidosis presenting as renal infarction.

    PubMed

    Arias, M; Abreu, J A; Iglesias, A; Longo, J; Lecumberri, F; Vega, F

    1996-01-01

    We report a case of primary amyloidosis affecting the kidney and presenting as a renal infarction on computed tomography and ultrasound examination. To our knowledge, it is the first case in the radio-logical literature with these imaging characteristics.

  13. Al Qaeda as a System

    DTIC Science & Technology

    2006-03-15

    either as welcome guests or parasites . As welcome guests, Al Qaeda members take sanctuary in sponsor states. Sponsor states provide Al Qaeda with...sponsorship, Al Qaeda takes sanctuary as parasites either overtly or covertly. They take overt sanctuary in countries that publicly claim a policy...39 Yehudit Barsky, “Al Qa’ida, Iran, and Hezbollah: A Continuing Symbiosis ,” The American Jewish Committee Series on Terrorism , February 2004, 2-3

  14. 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

  15. Amyloid fibril proteins and amyloidosis: chemical identification and clinical classification International Society of Amyloidosis 2016 Nomenclature Guidelines.

    PubMed

    Sipe, Jean D; Benson, Merrill D; Buxbaum, Joel N; Ikeda, Shu-Ichi; Merlini, Giampaolo; Saraiva, Maria J M; Westermark, Per

    2016-12-01

    The Nomenclature Committee of the International Society of Amyloidosis (ISA) met during the XVth Symposium of the Society, 3 July-7 July 2016, Uppsala, Sweden, to assess and formulate recommendations for nomenclature for amyloid fibril proteins and the clinical classification of the amyloidoses. An amyloid fibril must exhibit affinity for Congo red and with green, yellow or orange birefringence when the Congo red-stained deposits are viewed with polarized light. While congophilia and birefringence remain the gold standard for demonstration of amyloid deposits, new staining and imaging techniques are proving useful. To be included in the nomenclature list, in addition to congophilia and birefringence, the chemical identity of the protein must be unambiguously characterized by protein sequence analysis when possible. In general, it is insufficient to identify a mutation in the gene of a candidate amyloid protein without confirming the variant changes in the amyloid fibril protein. Each distinct form of amyloidosis is uniquely characterized by the chemical identity of the amyloid fibril protein that deposits in the extracellular spaces of tissues and organs and gives rise to the disease syndrome. The fibril proteins are designated as protein A followed by a suffix that is an abbreviation of the parent or precursor protein name. To date, there are 36 known extracellular fibril proteins in humans, 2 of which are iatrogenic in nature and 9 of which have also been identified in animals. Two newly recognized fibril proteins, AApoCII derived from apolipoprotein CII and AApoCIII derived from apolipoprotein CIII, have been added. AApoCII amyloidosis and AApoCIII amyloidosis are hereditary systemic amyloidoses. Intracellular protein inclusions displaying some of the properties of amyloid, "intracellular amyloid" have been reported. Two proteins which were previously characterized as intracellular inclusions, tau and α-synuclein, are now recognized to form extracellular

  16. Serum amyloid A  renal amyloidosis in a chronic subcutaneous (“skin popping”) heroin user

    PubMed Central

    Cooper, Chad; Bilbao, Jorge E.; Said, Sarmad; Alkhateeb, Haider; Bizet, Jorge; Elfar, Ahmed; Davalos, Olinamyr; Meza, Ana T.; Hernandez, German T.

    2013-01-01

    Background: Systemic AA amyloidosis is a long-term complication of several chronic inflammatory disorders. Organ damage results from the extracellular deposition of proteolytic fragments of the acute-phase reactant serum amyloid A (SAA) as amyloid fibrils. Drug users that inject drug by a subcutaneous route (“skin popping”) have a higher chance of developing secondary amyloidosis. The kidneys, liver, and spleen are the main target organs of AA amyloid deposits. More than 90% of patients with renal amyloidosis will present with proteinuria, nephrotic syndrome, or renal function. Case presentation: A 37 year-old female presented to the hospital with a one-week history of pain and redness in her right axilla. Her relevant medical history included multiple skin abscesses secondary to “skin popping”, heroin abuse for 18 years, and hepatitis C. The physical examination revealed “skin popping” lesions, bilateral costovertebral angle tenderness, and bilateral knee swelling. The laboratory workup was significant for renal insufficiency with a serum creatinine of 5 mg/dL and 14.8 grams of urine protein per 1 gram of urine creatinine. The renal biopsy findings were consistent with a diagnosis of renal amyloidosis due to serum amyloid A deposition and acute tubulointerstitial nephritis. Conclusions: AA renal amyloidosis among heroin addicts seems to be associated with chronic suppurative skin infection secondary to “skin popping”. It is postulated that the chronic immunologic stimulation by one or more exogenous antigens or multiple acute inflammatory episodes is an important factor in the pathogenesis of amyloidosis in these patients. Therefore, AA renal amyloidosis should always be considered in chronic heroin users presenting with proteinuria and renal impairment. PMID:24475449

  17. 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

  18. Amyloidosis

    MedlinePlus

    ... Content ASCO Conquer Cancer Foundation Journal of Clinical Oncology Journal of Oncology Practice ASCO University Donate eNEWS SIGNUP f Cancer. ... of medical, surgical, radiation, gynecologic, and pediatric oncologists, oncology nurses, physician assistants, social workers, and patient advocates. ...

  19. [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.

  20. Localized amyloidosis of the stomach mimicking a superficial gastric cancer.

    PubMed

    Kagawa, Miwako; Fujino, Yasuteru; Muguruma, Naoki; Murayama, Noriaki; Okamoto, Koichi; Kitamura, Shinji; Kimura, Tetsuo; Kishi, Kazuhiro; Miyamoto, Hiroshi; Uehara, Hisanori; Takayama, Tetsuji

    2016-06-01

    A 73-year-old man was referred to our hospital for further examination of a depressed lesion in the stomach found by cancer screening gastroscopy. A barium upper gastrointestinal series showed an area of irregular mucosa measuring 15 mm on the anterior wall of the gastric body. Esophagogastroduodenoscopy revealed a 15 mm depressed lesion on the anterior wall of the lower gastric body. We suspected an undifferentiated adenocarcinoma from the appearance and took some biopsies. However, histology of the specimens revealed amyloidal deposits in the submucosal layer without malignant findings. Congo red staining was positive for amyloidal protein and green birefringence was observed under polarized light microscopy. Congo red staining with prior potassium permanganate incubation confirmed the light chain (AL) amyloid type. There were no amyloid deposits in the colon or duodenum. Computed tomography of the chest, abdomen, and pelvis showed no remarkable findings. Thus, this case was diagnosed as a localized gastric amyloidosis characterized by AL type amyloid deposition in the mucosal or submucosal layer. As the clinical outcome of gastric AL amyloidosis seems favorable, this case is scheduled for periodic examination to recognize potential disease progression and has been stable for 2 years.

  1. Phase 2 trial of daily, oral epigallocatechin gallate in patients with light-chain amyloidosis.

    PubMed

    Meshitsuka, Sohsuke; Shingaki, Sumito; Hotta, Masatoshi; Goto, Miku; Kobayashi, Makoto; Ukawa, Yuuichi; Sagesaka, Yuko M; Wada, Yasuyo; Nojima, Masanori; Suzuki, Kenshi

    2017-03-01

    Previous studies have suggested that an increase in mitochondrial reactive oxygen species may cause organ damage in patients with light-chain (AL) amyloidosis; however, this damage can be decreased by antioxidant-agent treatment. Epigallocatechin gallate (EGCG), the major natural catechin in green tea, has potent antioxidant activity. Because EGCG has recently been reported to have a favorable toxicity profile for treating amyloidosis, we sought to examine the clinical efficacy and toxicity of EGCG in patients with AL amyloidosis. Fifty-seven patients were randomly assigned to the EGCG and observation groups and observed for six months. There were no increases in grade 3-5 adverse events and EGCG therapy was well tolerated. Although a decrease in the urinary albumin level was found in the EGCG group in patients with obvious albuminuria after treatment initiation, its antioxidant activity may not be sufficient to clarify the potential effect of EGCG in patients with AL amyloidosis. Because some of the biological markers responsible for organ damage were well correlated to the level of antioxidant potential in patients' plasma, the status of oxidative stress in the blood may indicate the extent of organ damage in clinical situations.

  2. Apolipoprotein C-II Deposition Amyloidosis: A Potential Misdiagnosis as Light Chain Amyloidosis

    PubMed Central

    Schuiteman, Emily; Zarouk, Sami

    2016-01-01

    Hereditary amyloidoses are rare and pose a diagnostic challenge. We report a case of hereditary amyloidosis associated with apolipoprotein C-II deposition in a 61-year-old female presenting with renal failure and nephrotic syndrome misdiagnosed as light chain amyloidosis. Renal biopsy was consistent with amyloidosis on microscopy; however, immunofluorescence was inconclusive for the type of amyloid protein. Monoclonal gammopathy evaluation revealed kappa light chain. Bone marrow biopsy revealed minimal involvement with amyloidosis with kappa monotypic plasma cells on flow cytometry. She was started on chemotherapy for light chain amyloidosis. She was referred to the Mayo clinic where laser microdissection and liquid chromatography mass spectrometry detected high levels of apolipoprotein C-II, making a definitive diagnosis. Apolipoprotein C-II is a component of very low-density lipoprotein and aggregates in lipid-free conditions to form amyloid fibrils. The identification of apolipoprotein C-II as the cause of amyloidosis cannot be solely made with routine microscopy or immunofluorescence. Further evaluation of biopsy specimens with laser microdissection and mass spectrometry and DNA sequencing of exons should be done routinely in patients with amyloidoses for definitive diagnosis. Our case highlights the importance of determining the subtype of amyloidosis that is critical for avoiding unnecessary therapy such as chemotherapy. PMID:27840752

  3. 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

  4. 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.

  5. 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

  6. Colchicine use in isolated renal AA amyloidosis.

    PubMed

    Meneses, Carlos F; Egües, César A; Uriarte, Miren; Belzunegui, Joaquín; Rezola, Marta

    2015-01-01

    We present the case of a 45-year-old woman, with two-year history of chronic renal insufficiency and proteinuria. A kidney biopsy showed the presence of AA amyloidosis (positive Congo red staining and immunohistochemistry). There was no evidence of amyloid deposits in other organs and there was no underlying disease. AA amyloidosis normally is secondary to chronic inflammatory or infectious diseases. High levels of IL-1, IL-6 and TNF-α play a role in the pathogenesis of amyloidosis and induce the synthesis of serum amyloid A protein (SAA), a precursor of tissue amyloid deposits. We empirically treated the patient with a low dose colchicine. The patient responded well. Colchicine has been used for the treatment of Familiar Mediterranean Fever and related auto-inflammatory diseases. To monitor treatment responses, we measured SAA finding low titers. Soon after treatment onset there were signs of improvement pertaining to proteinuria and stabilization of renal function.

  7. Isolated Atrial Amyloidosis in Patients with Various Types of Atrial Fibrillation.

    PubMed

    Sukhacheva, T V; Eremeeva, M V; Ibragimova, A G; Vaskovskii, V A; Serov, R A; Revishvili, A Sh

    2016-04-01

    The myocardium of the right and left atrial appendages (auricles) in patients with paroxysmal, persistent, and permanent forms of atrial fibrillation was examined by histological methods and electron microscopy. Isolated atrial amyloidosis was detected in the left (50.0-56.3% patients) and in the right (45.0-55.6% patients) atrial appendages. In all cases, immunohistochemistry revealed atrial natriuretic peptide in fibrillary amyloid deposits. Ultrastructurally, amyloid masses formed clusters of myofibrils 8-10 nm in diameter. They were chaotically located in the extracellular space along the sarcolemma as well as in membrane invaginations, dilated tubules of cardiomyocyte T-tubular system, and vascular walls. Amyloidosis was predominantly observed in women; its degree positively correlated with age of patients and duration of atrial fibrillation but negatively correlated with atrial fibrosis. The study revealed positive (in permanent atrial fibrillation) and negative (in paroxysmal atrial fibrillation) correlation of amyloidosis with myofibril content in atrial cardiomyocytes.

  8. 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.

  9. Ocular adnexal and orbital amyloidosis: a case series and literature review.

    PubMed

    Mora-Horna, Eduardo R; Rojas-Padilla, Rubí; López, Vianhi G; Guzmán, Martín J; Ceriotto, Ariel; Salcedo, Guillermo

    2016-04-01

    The purpose of the study was to describe the main clinical and epidemiologic characteristics, treatment options, and outcome in a large series of patients with periocular and orbital amyloidosis. This is a retrospective, descriptive, observational study of a case series of 14 patients with periocular and orbital amyloidosis and is a review of previously published cases with this diagnosis between September 2004 and January 2015. In this study, we analyzed our 14 patients in conjunction with 69 well-documented cases of orbital and/or periocular amyloidosis previously reported, with a total of 83. Of these, 54 were female (65.1 %), 28 male (33.7 %), and one with unspecified gender. The mean age at diagnosis was 54.9 years (range, 18-87). The localization of the amyloidosis was classified as superficial, deep and combined, with involvement of 53 (63.9 %), 26 (31.3 %), and four cases (4.8 %) in each group, respectively. The main findings in superficial amyloidosis were mass or tissue infiltration (84.9 %) and ptosis (30.2 %) and, in the cases with deep involvement, mass (65.4 %), proptosis (57.7 %), limited ocular movements (34.6 %), ocular displacement (30.8 %), and ptosis (26.9 %). The cases with combined involvement presented with signs and symptoms of the two groups. Regarding the outcome, 43 patients were reported stable after the diagnosis and 21 had recurrence or required new surgical procedures. Periocular and orbital amyloidosis is a rare disease that can present with a variety of symptoms and signs depending on the localization and extension of involvement. Its prompt recognition is important in order to investigate systemic disease, which will affect the prognosis of each case.

  10. 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

  11. Primary amyloidosis presenting as "dropped head syndrome".

    PubMed

    Chuquilin, Miguel; Al-Lozi, Muhammad

    2011-06-01

    A 77-year-old man, with a history of lymphoma, presented with isolated neck extensor weakness and a 2-year history of bilateral carpal tunnel syndrome (CTS). Needle electromyography showed myopathic changes, and biopsy of cervical paraspinal muscles showed amyloid deposition in blood vessels. Amyloidosis should be considered in the differential diagnosis of dropped head syndrome.

  12. Nasal and ocular amyloidosis in a 15-year-old horse.

    PubMed

    Østevik, Liv; Gunnes, Gjermund; de Souza, Gustavo A; Wien, Tale N; Sørby, Randi

    2014-08-27

    Localized nasal, conjunctival and corneal amyloidosis was diagnosed in a 15-year-old pony with nasal and conjunctival masses and severe dyspnoea. Multiple swellings had been evident in the nostrils for at least two years and had gradually increased in size before presentation due to dyspnoea and exercise intolerance. Surgical debulking of the masses was performed and histological examination revealed large amounts of extracellular, hyaline, eosinophilic, Congo red positive material in the lamina propria of the nasal mucosa. A tentative diagnosis of localized nasal amyloidosis was made. The treatment relieved the clinical signs, however, the nasal masses recurred and bilateral conjunctival, papillary masses developed. The horse was euthanized. Nodular nasal and papillary conjunctival masses consisting of rubbery, grey to yellow tissue were found at necropsy. At the limbus this tissue infiltrated and expanded the cornea. The masses consisted of amyloid and moderate infiltrates of T lymphocytes and B lymphocytes were present in the tissue. No predominance of either cell type was observed and no distinct neoplastic mass could be identified. Ultrastructural examination of the nasal mucosa and cornea confirmed the presence of abundant extracellular deposits of non-branching fibrils ranging from 9-11 nm in diameter consistent with amyloid. Immunohistochemistry of amyloid revealed no labelling for AA amyloid, and no peptides representing serum amyloid A (SAA) were detected by microscopic laser dissection and subsequent mass spectrometry. Peptides from immunoglobulin kappa-like light chains were detected and are suggestive of AL amyloidosis, however the results were inconclusive and a final identification of the amyloid protein could not be made.Nasal amyloidosis is a clinical entity of localized amyloid deposits in the horse. Localized amyloidosis involving the conjunctiva of the horse is previously described in only seven cases and the present case is the first case of

  13. 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

  14. 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

  15. 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

  16. 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.

  17. Waldenström's macroglobulinemia associated with AA amyloidosis.

    PubMed

    Gardyn, J; Schwartz, A; Gal, R; Lewinski, U; Kristt, D; Cohen, A M

    2001-07-01

    It is widely accepted that amyloidosis in Waldenström's macroglobulinemia (WM) is exclusively due to amyloid light-chain deposition. However, only a small number of previous reports have actually characterized the type of amyloid in WM. We now report the third patient with WM and amyloid A protein (AA) amyloidosis. This patient developed malabsorption, nephrotic syndrome, and orthostatic hypotension. AA was immunohistochemically demonstrated in the rectal biopsy. In conjunction with previous examples of AA amyloidosis, the present report raises the possibility that AA amyloidosis may also occur in WM patients.

  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. Update on treatment of light chain amyloidosis

    PubMed Central

    Mahmood, Shameem; Palladini, Giovanni; Sanchorawala, Vaishali; Wechalekar, Ashutosh

    2014-01-01

    Light chain amyloidosis is the most common type of amyloidosis as a consequence of protein misfolding of aggregates composed of amyloid fibrils. The clinical features are dependent on the organs involved, typically cardiac, renal, hepatic, peripheral and autonomic neuropathy and soft tissue. A tissue biopsy or fat aspirate is needed to confirm the presence/type of amyloid and prognostic tools are important in a risk stratified approach to treatment. Autologous stem cell transplant eligibility should be assessed at baseline, weighing the reversible or non-reversible contraindications, toxicity of treatment and chemotherapy alternatives available. Chemotherapy options include melphalan, thalidomide, bortezomib, lenalidomide, bendamustine in combination with dexamethasone. Many studies have explored these treatment modalities, with ongoing debate about the optimal first line and sequential treatment thereafter. Attaining a very good partial response or better is the treatment goal coupled with early assessment central to optimizing treatment. One major challenge remains increasing the awareness of this disease, frequently diagnosed late as the presenting symptoms mimic many other medical conditions. This review focuses on the treatments for light chain amyloidosis, how these treatments have evolved over the years, improved patient risk stratification, toxicities encountered and future directions. PMID:24497558

  1. Cardiac amyloidosis: the importance of a multidisciplinary approach.

    PubMed

    Gómez-Bueno, Manuel; Segovia, Javier; García-Pavía, Pablo; Barceló, Juan M; Krsnik, Isabel; Sánchez-Turrión, Víctor; Salas, Clara; Alonso-Pulpón, Luis

    2009-06-01

    Cardiac amyloidosis is associated with the interstitial deposition of abnormal protein in the myocardium, which can lead to a form of restrictive cardiomyopathy with a poor prognosis. This protein can have a number of different origins, which give rise to various subtypes of amyloidosis that have different prognoses and that require different therapeutic approaches. Drugs commonly used in heart failure have little effect in amyloidosis and the use of heart transplantation is controversial because amyloidosis is a multi-organ disease and because there is a possibility of disease recurrence in the graft. The use of new techniques to identify the specific amyloidosis subtype, the emergence of novel ways of preventing or decreasing amyloid production, the ability to monitor responses to therapy and, above all, the introduction of multidisciplinary teams that can implement a combination of therapies, including multiple organ transplantation, have contributed to a substantial improvement in the prognosis of this disease.

  2. Seminal Tract Amyloidosis: Synchronous Amyloidosis of the Seminal Vesicles, Deferent Ducts and Ejaculatory Ducts.

    PubMed

    Rath-Wolfson, Lea; Bubis, Golan; Shtrasburg, Shmuel; Shvero, Asaf; Koren, Rumelia

    2017-01-17

    Senile Seminal Vesicle Amyloidosis (SSVA) increases with age. Involvement of the whole seminal tract, i.e. the seminal vesicles, ejaculatory and deferent ducts was first reported by us in the International Symposium on Amyloidosis 1998. Since then we encountered four more cases of SSVA. In all these cases the ejaculatory and deferent ducts were also involved by amyloid. The amyloid was located mostly sub-epithelially, stained positively with Congo red, gave green birefringence under polarized light and was permanganate sensitive, slightly positive for lactoferrin immunostaining and negative for all known amyloid types. In recent years the amyloid was found to be derived from Semenogelin I, a major constituent of the seminal fluid which is present in the epithelial cells of the seminal vesicle and vas deference. This would explain the deposition of amyloid not only in the seminal vesicles but also in the deferent an ejaculatory ducts which transport the seminal fluid. In a review of the literature we found three more articles on SSVA in which the amyloid was not limited to the seminal vesicles alone. We propose to designate this type of amyloid as "Senile seminal Tract Amyloidosis" (SSTA) instead of "Senile Seminal Vesicle Amyloidosis (SSVA)".

  3. A Case of Amyloidosis Presenting as Chronic Cholecystitis, Misdiagnosed as Polymyalgia Rheumatica.

    PubMed

    Um, Yoo Jin; Kim, Hyoun Ah; Jung, Jin Hee; Cho, Hundo; Kang, Joon Koo

    2016-07-25

    Amyloidosis is a rare disease defined by extracellular deposits of amorphous fibrillar proteins, derived from aggregations of misfolded proteins. Localization of amyloidosis in the gallbladder is uncommon; only eight cases have been reported. We describe a case of amyloidosis diagnosed by cholecystectomy, which possibly also affected the liver and kidney. The patient was misdiagnosed with polymyalgia rheumatica, but after a cholecystectomy to treat chronic cholecystitis, we ultimately diagnosed him with amyloidosis. We review amyloidosis with gallbladder involvement in the literature.

  4. Dialysis-related amyloidosis: challenges and solutions

    PubMed Central

    Scarpioni, R; Ricardi, M; Albertazzi, V; De Amicis, S; Rastelli, F; Zerbini, L

    2016-01-01

    Amyloidosis refers to the extracellular tissue deposition of fibrils composed of low-molecular-weight subunits of a variety of proteins. These deposits may result in a wide range of clinical manifestations depending upon their type, location, and the amount of deposition. Dialysis-related amyloidosis is a serious complication of long-term dialysis therapy and is characterized by the deposition of amyloid fibrils, principally composed of β2 microglobulins (β2M), in the osteoarticular structures and viscera. Most of the β2M is eliminated through glomerular filtration and subsequent reabsorption and catabolism by the proximal tubules. As a consequence, the serum levels of β2M are inversely related to the glomerular filtration rate; therefore, in end-stage renal disease patients, β2M levels increase up to 60-fold. Serum levels of β2M are also elevated in several pathological conditions such as chronic inflammation, liver disease, and above all, in renal dysfunction. Retention of amyloidogenic protein has been attributed to several factors including type of dialysis membrane, prolonged uremic state and/or decreased diuresis, advanced glycation end products, elevated levels of cytokines and dialysate. Dialysis treatment per se has been considered to be an inflammatory stimulus, inducing cytokine production (such as interleukin-1, tumor necrosis factor-α, interleukin-6) and complement activation. The released cytokines are thought to stimulate the synthesis and release of β2M by the macrophages and/or augment the expression of human leukocyte antigens (class I), increasing β2M expression. Residual renal function is probably the best determinant of β2M levels. Therefore, it has to be maintained as long as possible. In this article, we will focus our attention on the etiology of dialysis-related amyloidosis, its prevention, therapy, and future solutions. PMID:27994478

  5. 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

  6. Amyloidosis: The Newer Discovered ALECT2 Associated with der7q add(7)

    PubMed Central

    Samal, Priyanka; Chakrabartty, Joydeep

    2016-01-01

    Amyloidosis is characterized by pathological deposition of abnormal protein aggregates in various tissues, AL protein being the commonest. ALECT 2 is the newest protein described, having a predisposition to affect the kidneys, sometimes the liver and rarely other organs. We present a case of renal amyloid ALECT 2 due to leucocyte cell derived chemotaxin 2, a novel amyloidogenic protein. The patient presented with mild proteinuria, scattered plasma cells on bone marrow examination and altered kappa/lambda ratio with associated cytogenetic abnormality of der7q add(7). It is essential to correctly type this protein and differentiate it from AL during diagnosis for appropriate and effective clinical management. PMID:27790444

  7. 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

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

    PubMed

    Ibi, Kanata; Murakami, Tomoaki; Goda, Wael Mohamed; Kobayashi, Naoki; Ishiguro, Naotaka; Yanai, Tokuma

    2015-10-01

    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.

  9. Transthyretin amyloidosis: an under-recognized neuropathy and cardiomyopathy.

    PubMed

    Galant, Natalie J; Westermark, Per; Higaki, Jeffrey N; Chakrabartty, Avijit

    2017-03-01

    Transthyretin (TTR) amyloidosis (ATTR amyloidosis) is an underdiagnosed and important type of cardiomyopathy and/or polyneuropathy that requires increased awareness within the medical community. Raising awareness among clinicians about this type of neuropathy and lethal form of heart disease is critical for improving earlier diagnosis and the identification of patients for treatment. The following review summarizes current criteria used to diagnose both hereditary and wild-type ATTR (ATTRwt) amyloidosis, tools available to clinicians to improve diagnostic accuracy, available and newly developing therapeutics, as well as a brief biochemical and biophysical background of TTR amyloidogenesis.

  10. 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.

  11. Novel NLRP12 mutations associated with intestinal amyloidosis in a patient diagnosed with common variable immunodeficiency.

    PubMed

    Borte, Stephan; Celiksoy, Mehmet Halil; Menzel, Volker; Ozkaya, Ozan; Ozen, Fatma Zeynep; Hammarström, Lennart; Yildiran, Alisan

    2014-10-01

    Heterozygous mutations in the NLRP12 gene have been found in patients with systemic auto-inflammatory diseases. However, the NLRP12-associated periodic fever syndromes show a wide clinical spectrum, including patients without classical diagnostic symptoms. Here, we report on a 20-year-old female patient diagnosed with common variable immunodeficiency (CVID), who developed intestinal amyloidosis and carried novel compound heterozygous mutations in NLRP12, identified by whole exome and transcriptome sequencing. CVID is a primary immunodeficiency characterized by low serum immunoglobulins, recurrent bacterial infections and development of malignancy, but it also presents with a magnitude of autoimmune features. Because of the unspecific heterogeneous clinical features of the disease, a delay in diagnosis is common. Secondary, inflammatory (AA type) amyloidosis has infrequently been observed in CVID patients. Based on our case observation and a critical review of the literature, we suggest that NLRP12 mutations might account for a small fraction of CVID patients with severe auto-inflammatory complications.

  12. Renal apolipoprotein A-I amyloidosis: a rare and usually ignored cause of hereditary tubulointerstitial nephritis.

    PubMed

    Gregorini, Gina; Izzi, Claudia; Obici, Laura; Tardanico, Regina; Röcken, Christoph; Viola, Battista Fabio; Capistrano, Mariano; Donadei, Simona; Biasi, Luciano; Scalvini, Tiziano; Merlini, Giampaolo; Scolari, Francesco

    2005-12-01

    Apolipoprotein A-I amyloidosis is a rare, late-onset, autosomal dominant condition characterized by systemic deposition of amyloid in tissues, the major clinical problems being related to renal, hepatic, and cardiac involvement. Described is the clinical and histologic picture of renal involvement as a result of apolipoprotein A-I amyloidosis in five families of Italian ancestry. In all of the affected family members, the disease was caused by the Leu75Pro heterozygous mutation in exon 4 of apolipoprotein A-I gene, as demonstrated by direct sequencing and RFLP analysis. Immunohistochemistry confirmed that amyloid deposits were specifically stained with an anti-apolipoprotein A-I antibody. The clinical phenotype was mainly characterized by a variable combination of kidney and liver disturbance. The occurrence of renal involvement seemed to be almost universal, although its severity varied greatly ranging from subclinical organ damage to overt, slowly progressive renal dysfunction. The renal presentation was consistent with a tubulointerstitial disease, as suggested by the findings of defective urine-concentrating capacity, moderate polyuria, negative urinalysis, and mild tubular proteinuria. Histology confirmed tubulointerstitial nephritis. Surprising, amyloid was restricted to nonglomerular regions and limited to the renal medulla. This location of apolipoprotein A-I amyloid differs sharply from other systemic amyloidoses that are mainly characterized by glomerular and vascular deposits. The tubulointerstitial nephritis as a result of hereditary apolipoprotein A-I amyloidosis is a rare disease and a challenging diagnosis to recognize. Patients who present with familial tubulointerstitial nephritis associated with liver disease require a high index of suspicion for apolipoprotein A-I amyloidosis.

  13. Liver transplantation in transthyretin amyloidosis: Characteristics and management related to kidney disease.

    PubMed

    Rocha, Ana; Lobato, Luísa

    2016-09-14

    Orthotopic liver transplantation (LT) was implemented as the inaugural disease-modifying therapy for hereditary transthyretin (ATTR) amyloidosis, a systemic amyloidosis mainly affecting the peripheral nervous system and heart. The first approach to pharmacologic therapy was focused on the stabilization of the TTR tetramer; following that new advent LT was assumed as the second step of treatment, for those patients whose neuropathy becomes worse after a course of pharmacologic therapy. The renal disease has been ignored in hereditary ATTR amyloidosis. The low level of proteinuria or slight renal impairment does not suppose such a heavy glomerular and vascular amyloid deposition. Moreover, severity of renal deposits does not consistently parallel that of myelinated nerve fiber loss. These are pitfalls that limit the success of LT and suggest troublesome criteria for pharmacological therapy or LT. An algorithm of evaluation concerning renal disease and treatment options is presented and some bridges-to-decision are exposed. In stage 4 or 5 kidney disease, the approach remains to deliver combined or sequential liver-kidney transplantation in eligible patients. However, in the majority, hemodialysis is the only option even in the presence of a well-functioning liver graft. In this review, we highlight useful information to aid the transplant hepatologist in the clinical practice.

  14. Antibodies to myelin-associated glycoprotein (anti-Mag) in IgM amyloidosis may influence expression of neuropathy in rare patients.

    PubMed

    Garces-Sanchez, Mercedes; Dyck, Peter J; Kyle, Robert A; Zeldenrust, Steven; Wu, Yanhong; Ladha, Shafeeq S; Klein, Christopher J

    2008-04-01

    We have examined whether antibodies to myelin-associated glycoprotein (anti-MAG) influence neuropathy occurrence and phenotype in primary (AL IgM) amyloidosis. Anti-MAG and the cross-reacted sulfoglucuronyl paragloboside antibodies (SGPG) were studied in 46 patients with IgM amyloidosis (21 with polyneuropathy), and 21 matched IgM MGUS (monoclonal gammopathies of undetermined significance) controls without neuropathy. We assessed the occurrence, phenotype of neuropathy, and attributes of nerve conduction and their relation to antibody activity. Twenty of 46 patients with IgM amyloidosis (7 with and 13 without polyneuropathy) had elevation of anti-MAG or SGPG by enzyme-linked immunosorbent assay (ELISA). Two of the polyneuropathy patients with IgM amyloidosis had antibodies to MAG based on Western blot (WB) positivity. One of these patients, with the highest anti-MAG titer, had a painful sensory ataxia, with prominent demyelination, and amyloid deposition in sural nerve. The other anti-MAG WB-positive amyloid patient had an axonal neuropathy and dysautonomia. Low levels of anti-MAG antibodies were found in 12 of 21 IgM MGUS controls without neuropathy (mean follow-up, 11 years). We conclude that finding serum anti-MAG antibodies does not exclude the diagnosis of primary amyloidosis. They do not appear to affect the occurrence or expression of polyneuropathy, except possibly in occasional cases with WB positivity.

  15. [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.

  16. Effects of Biologic Agents in Patients with Rheumatoid Arthritis and Amyloidosis Treated with Hemodialysis

    PubMed Central

    Kuroda, Takeshi; Tanabe, Naohito; Nozawa, Yukiko; Sato, Hiroe; Nakatsue, Takeshi; Kobayashi, Daisuke; Wada, Yoko; Saeki, Takako; Nakano, Masaaki; Narita, Ichiei

    2016-01-01

    Objective Our objective was to examine the safety and effects of therapy with biologics on the prognosis of rheumatoid arthritis (RA) patients with reactive amyloid A (AA) amyloidosis on hemodialysis (HD). Methods Twenty-eight patients with an established diagnosis of reactive AA amyloidosis participated in the study. The survival was calculated from the date of HD initiation until the time of death, or up to end of June 2015 for the patients who were still alive. HD initiation was according to the program of HD initiation for systemic amyloidosis patients associated with RA. Results Ten patients had been treated with biologics before HD initiation for a mean of 28.2 months (biologic group), while 18 had not (non-biologic group). HD was initiated in patients with similar characteristics except for the tender joint count, swollen joint count, and disease activity score (DAS)28-C-reactive protein (CRP). History of biologics showed that etanercept was frequently used for 8 patients as the first biologic. There was no significant difference in the mortality rate according to a Kaplan-Meier analysis (p=0.939) and or associated risk of death in an age-adjusted Cox proportional hazards model (p=0.758) between both groups. Infections were significantly more frequent causes of death in the biologic group than in the non-biologic group (p=0.021). However, treatment with biologics improved the DAS28-CRP score (p=0.004). Conclusion Under the limited conditions of AA amyloidosis treated with HD, the use of biologics might affect infection and thus may not improve the prognosis. Strict infection control is necessary for the use of biologics with HD to improve the prognosis. PMID:27725536

  17. Hereditary renal amyloidosis with a novel variant fibrinogen.

    PubMed Central

    Uemichi, T; Liepnieks, J J; Benson, M D

    1994-01-01

    Two families with hereditary renal amyloidosis were found to have a novel mutation in the fibrinogen A alpha chain gene. This form of amyloidosis is an autosomal dominant condition characterized by proteinuria, hypertension, and subsequent azotemia. DNAs of patients with amyloidosis were screened for a polymorphism in fibrinogen A alpha chain gene by single-strand conformation polymorphism analysis, and affected individuals from two kindreds were found to have a mutation. Both of these kindreds are American of Irish descent presenting with non-neuropathic, nephropathic amyloidosis in the fifth to the seventh decade of life. DNA sequencing showed a point mutation in the fibrinogen A alpha chain gene that is responsible for substitution of valine for glutamic acid at position 526. By restriction fragment length polymorphism analysis, 7 affected individuals and 14 asymptomatic individuals in these two kindreds were positive for the fibrinogen A alpha chain Val 526 gene. Fibrinogen was isolated from plasma of a heterozygous gene carrier and shown to contain approximately 50% variant fibrinogen. Discovery of this new mutation confirms the association between fibrinogen A alpha chain variant and hereditary renal amyloidosis and establishes a new biochemical subtype of amyloidosis. Images PMID:8113408

  18. 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.

  19. 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

  20. Phase diagrams of Al-Si and Al-Ge systems

    NASA Astrophysics Data System (ADS)

    Kagaya, Hiroko-Matsuo; Imazawa, Kazumoto; Sato, Mayumi; Soma, Toshinobu

    1998-03-01

    Considering the contributions from the band and local mode frequencies, the temperature- and pressure-dependent mean-square displacement for Al-Si and Al-Ge solid solutions is quantitatively calculated to be similar to that for matrix Al. Then, the concentration-dependence of the Debye temperature at higher and lower temperatures is estimated, the pressure effect on the solidus curve is presented by applying Lindeman's melting law to the Al-Si and Al-Ge alloy systems. The solidus curve obtained increases as a function of the pressure, and is in good agreement with the observed tendency for these solid solutions.

  1. 99mTc DPD is the preferential bone tracer for diagnosis of cardiac transthyretin amyloidosis.

    PubMed

    Rossi, Pascal; Tessonnier, Laurent; Frances, Yves; Mundler, Olivier; Granel, Brigitte

    2012-08-01

    We emphasize the role of Tc-99m-3,3-diphosphono-1,2-propanodicarboxylicacid (DPD) scintigraphy as a noninvasive tool to distinguish transthyretin (TTR)-related cardiac amyloidosis from other forms of cardiac amyloidosis. We report the case of a 76-year-old male patient suffering from congestive heart failure in whom imaging investigation by DPD scintigraphy showed a strong cardiac uptake highly suggestive of TTR amyloidosis variant. TTR-related cardiac amyloidosis was confirmed on myocardial biopsies by immunohistochemistry analysis. This case supports the growing interest in DPD scintigraphy for typing cardiac amyloidosis and for its contribution in the place of invasive myocardial biopsy.

  2. 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

  3. [Osteoarticular amyloidosis and dysglobulinemia: apropos of 2 cases].

    PubMed

    Morillon-Vié, M A; Petit, E; Galezowski, N; Rebischung, J L; Baviéra, E; Herreman, G

    1997-01-01

    We report two cases of bone and joint amyloidosis involvement related to plasma cell dyscrasia. The radiographic appearances mimic numerous benign or malignant diseases. MR imaging shows a diffuse low signal in T1 and an heterogeneous low or mild low signal in T2 weighted spin-echo sequence.

  4. A Case of Cardiac Amyloidosis Initially Misdiagnosed as Syndrome X

    PubMed Central

    Sohn, Hyung Rae; Song, Bong Gun; Jeong, Seong Yeon; Hong, Su-Min; Jung, Hyun Gul; Jung, Hye-Jin; Cho, Wook-Hyun; Choi, Suk-Koo

    2011-01-01

    Cardiac infiltration of amyloid fibril results in progressive cardiomyopathy with a grave prognosis and results in cardiac diseases such as congestive heart disease, cardiomyopathy, valvular heart disease, and arrhythmias. We present a rare case of cardiac amyloidosis initially misdiagnosed as syndrome X in which recurrent chest pain and progressive heart failure could be managed finally by heart transplantation.

  5. '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.

  6. Prevalence, Severity, and Prognostic Value of Sleep Apnea Syndromes in Cardiac Amyloidosis

    PubMed Central

    Bodez, Diane; Guellich, Aziz; Kharoubi, Mounira; Covali-Noroc, Ala; Tissot, Claire-Marie; Guendouz, Soulef; Hittinger, Luc; Dubois-Randé, Jean-Luc; Lefaucheur, Jean-Pascal; Planté-Bordeneuve, Violaine; Adnot, Serge; Boyer, Laurent; Damy, Thibaud

    2016-01-01

    Study Objectives: To assess prevalence, severity, and prognostic value of sleep-disordered breathing (SDB), in the three main cardiac amyloidosis (CA) types, i.e., light-chain (AL), transthyretin-related familial (m-TTR), or senile (WT-TTR). Methods: Patients consecutively referred for CA diagnosis work-up underwent cardiac assessment and nocturnal polygraphy. SDB was defined as apnea-hypopnea index (AHI) ≥ 5/h. Multivariate analysis was used to identify predictors of a major adverse cardiac event (MACE) defined as death, heart transplantation and acute heart failure. Results: Seventy CA patients were included (31 AL, 22 m-TTR, 17 WT-TTR). The mean ± standard deviation age and left ventricular ejection fraction were 71 ± 12 years and 49% ± 13% and median (interquartile range) N terminal pro brain natriuretic peptide (NT-proBNP) was 3,932 (1,607; 7,028) pg/mL. The prevalence of SDB was 90% without difference between amyloidosis types. SDB was central in 27% and obstructive in 73%. AL had less frequent severe SDB compared to m-TTR and WT-TTR (P = 0.015) but longer time with peripheral capillary oxygen saturation (SpO2) < 90% (P = 0.037). After a median follow-up of 7.5 (2.8; 14.9) months, 49% patients experienced MACE. Time with nocturnal SpO2 < 90% was the only independent predictor of MACE. The best-identified threshold was 30 min. Values > 30 min were associated with bad prognosis (Log-rank χ2: 8.01, P value = 0.005). Using binomial logistic regression, determinants of time with nocturnal SpO2 < 90% were New York Heart Association class (P = 0.011), and log-NT-proBNP (P = 0.04) but not AHI. Conclusions: In CA population, prevalence of SDB is high (90%) and dominated by the obstructive pattern. Bad prognosis in this population was driven by nocturnal desaturation, reflecting heart failure severity and respiratory involvement. Citation: Bodez D, Guellich A, Kharoubi M, Covali-Noroc A, Tissot CM, Guendouz S, Hittinger L, Dubois-Randé JL, Lefaucheur JP, Plant

  7. Globular hepatic amyloid is highly sensitive and specific for LECT2 amyloidosis.

    PubMed

    Chandan, Vishal S; Shah, Sejal S; Lam-Himlin, Dora M; Petris, Giovanni De; Mereuta, Oana M; Dogan, Ahmet; Torbenson, Michael S; Wu, Tsung-Teh

    2015-04-01

    Globular hepatic amyloid (GHA) is rare, and its clinical significance remains unclear. Recently, leukocyte chemotactic factor-associated amyloidosis (ALECT2) has been reported to involve the liver, showing a globular pattern. We reviewed 70 consecutive cases of hepatic amyloidosis to determine the prevalence and morphology of hepatic amyloid subtypes, especially ALECT2 and its association with GHA. Each case was reviewed for amyloid subtype (immunohistochemistry and/or mass spectrometry), its pattern (linear or globular), and distribution (vascular, perisinusoidal, or stromal). In addition, 24 cases of confirmed hepatic ALECT2 on mass spectrometry from our consultation files were also reviewed. LECT2 immunostaining was performed in 49 cases. Of the 70 cases, immunoglobulin light chain (AL) type was most common with 41 cases (59%), followed by transthyretin (ATTR) 15 cases (22%), 3 cases each of fibrinogen A (AFib) (4%), serum amyloid A (AA) (4%), and ALECT2 (4%), 2 cases of apolipoproteins (AApoA1) (3%), and 3 cases (4%) were unclassified. Three of our 70 cases (4%), with ALECT2, and all 24 cases (100%) of mass spectrometry-confirmed hepatic ALECT2 showed only GHA deposits in the hepatic sinusoids and portal tracts. Three (4%) other cases of AL type showed a focal globular pattern admixed with prominent linear amyloid. None of the other amyloid subtypes showed GHA. LECT2 immunostain was positive in all 27 cases (100%) of ALECT2 and negative in the other 22 non-ALECT2 cases (100%) (14 AL, 5 ATTR, 1 AA, 1 AFib, 1 AApoA1). Pure GHA is uncommon (4%) but is highly specific for ALECT2, and LECT2 immunostain is helpful in confirming this amyloid type.

  8. Liver transplantation in transthyretin amyloidosis: issues and challenges.

    PubMed

    Carvalho, Andreia; Rocha, Ana; Lobato, Luísa

    2015-03-01

    Hereditary transthyretin amyloidosis (ATTR) is a rare worldwide autosomal dominant disease caused by the systemic deposition of an amyloidogenic variant of transthyretin (TTR), which is usually derived from a single amino acid substitution in the TTR gene. More than 100 mutations have been described, with V30M being the most prevalent. Each variant has a different involvement, although peripheral neuropathy and cardiomyopathy are the most common. Orthotopic liver transplantation (OLT) was implemented as the inaugural disease-modifying therapy because the liver produces the circulating unstable TTR. In this review, we focus on the results and long-term outcomes of OLT for ATTR after more than 2063 procedures and 23 years of experience. After successful OLT, neuropathy and organ impairment are not usually reversed, and in some cases, the disease progresses. The overall 5-year survival rate is approximately 100% for V30M patients and 59% for non-ATTR V30M patients. Cardiac-related death and septicemia are the main causes of mortality. Lower survival is related to malnutrition, a longer duration of disease, cardiomyopathy, and a later onset (particularly for males). Deposits, which are composed of a mixture of truncated and full-length TTR (type A) fibrils, have been associated with posttransplant myocardial dysfunction. A higher incidence of early hepatic artery thrombosis of the graft has also been documented for these patients. Liver-kidney/heart transplantation is an alternative for patients with advanced renal disease or heart failure. The sequential procedure, in which ATTR livers are reused in patients with liver disease, reveals that neuropathy in the recipient may appear as soon as 6 years after OLT, and ATTR deposits may appear even earlier. Long-term results of trials with amyloid protein stabilizers or disrupters, silencing RNA, and antisense oligonucleotides will highlight the value and limitations of liver transplantation.

  9. Investigation of Phase Equilibria and Some Properties of Alloys of Ti-Al-Fe and Ti-Al-V Systems,

    DTIC Science & Technology

    Some data on the structure and properties of Ti-Al-Fe alloys are presented. The phase equilibria in alloys in the system Ti-Al-V were studies...However, the data available in the literature on phase equilibria in the systems Ti-Al-Fe and Ti-Al-V require refinement, as they are insufficiently

  10. Thermodynamic Modeling of the Al-Cr-Mn Ternary System

    NASA Astrophysics Data System (ADS)

    Cui, Senlin; Jung, In-Ho

    2017-03-01

    The phase diagram information available in the literature on the Al-Cr-Mn system was comprehensively evaluated and optimized for the first time to obtain a set of Gibbs energies of all the solid and liquid phases in the Al-Cr-Mn system. The Modified Quasi-chemical Model (MQM) was utilized to describe the Gibbs energy of the liquid phase of the Al-Cr-Mn system. The Compound Energy Formalism (CEF) was used to model the solid solution phases. A revision of the Al-Mn system was simultaneously conducted to consider the γ_H (Al8Mn5) phase. The liquid Cr-Mn phase was also remodeled using the Modified Quasi-chemical Model (MQM) to obtain a consistent description of the ternary Al-Cr-Mn liquid phase. Accurate description of the phase diagram of the entire Al-Cr-Mn system was obtained from the thermodynamic models with optimized parameters in the present study, and the model parameters can be used to predict the thermodynamic properties of the ternary system.

  11. Fecal transmission of AA amyloidosis in the cheetah contributes to high incidence of disease.

    PubMed

    Zhang, Beiru; Une, Yumi; Fu, Xiaoying; Yan, Jingmin; Ge, FengXia; Yao, Junjie; Sawashita, Jinko; Mori, Masayuki; Tomozawa, Hiroshi; Kametani, Fuyuki; Higuchi, Keiichi

    2008-05-20

    AA amyloidosis is one of the principal causes of morbidity and mortality in captive cheetahs (Acinonyx jubatus), which are in danger of extinction, but little is known about the underlying mechanisms. Given the transmissible characteristics of AA amyloidosis, transmission between captive cheetahs may be a possible mechanism involved in the high incidence of AA amyloidosis. In this study of animals with AA amyloidosis, we found that cheetah feces contained AA amyloid fibrils that were different from those of the liver with regard to molecular weight and shape and had greater transmissibility. The infectious activity of fecal AA amyloid fibrils was reduced or abolished by the protein denaturants 6 M guanidine.HCl and formic acid or by AA immunodepletion. Thus, we propose that feces are a vehicle of transmission that may accelerate AA amyloidosis in captive cheetah populations. These results provide a pathogenesis for AA amyloidosis and suggest possible measures for rescuing cheetahs from extinction.

  12. The pathological and biochemical identification of possible seed‐lesions of transmitted transthyretin amyloidosis after domino liver transplantation

    PubMed Central

    Yoshinaga, Tsuneaki; Sekijima, Yoshiki; Kametani, Fuyuki; Miyashita, Kana; Hachiya, Naomi; Tanaka, Tomohiro; Kokudo, Norihiro; Higuchi, Keiichi; Ikeda, Shu‐ichi

    2016-01-01

    Abstract The most serious issue in domino liver transplantation (DLT) using liver grafts from patients with transthyretin (TTR)‐related familial amyloid polyneuropathy (FAP) is the development of iatrogenic transmitted amyloidosis (de novo amyloidosis) in DLT‐recipients. However, little is known regarding the mechanisms of the initial stage of amyloid formation in these recipients. We detected initial lesions (possible seed‐lesions) of this iatrogenic amyloidosis in two recipients following liver grafting from FAP patients. Patient 1 underwent DLT at age 65 from an FAP patient with a Val30Met TTR variant and patient 2 received DLT from an FAP patient with a Val30Leu TTR variant at age 32. Patient 2 was started on diflunisal administration from 4 years after DLT. While neither patient had symptoms of FAP, small amyloid deposits were detected on the gastroduodenal mucosae 14 months and 12 years after DLT in patient 1 and patient 2, respectively. The amyloid was analyzed using a laser microdissection system and tandem mass spectrometry. Biochemical analysis indicated that the amyloid was composed mostly of variant TTR produced from the transplanted liver in both patients. In patient 1, wild‐type TTR amyloid was detectable in the duodenal mucosa obtained 2 years after DLT. This is the first study to successfully capture the pathological and biochemical features of initial‐stage amyloid lesions in DLT recipients. The findings clearly indicate that amyloid deposition can start by deposition of variant TTR followed by deposition of wild‐type TTR, and blocking of amyloid seed formation from variant TTR may be a key to prevent or delay the development of DLT‐associated amyloidosis. PMID:27499917

  13. Early alterations in functional connectivity and white matter structure in a transgenic mouse model of cerebral amyloidosis.

    PubMed

    Grandjean, Joanes; Schroeter, Aileen; He, Pan; Tanadini, Matteo; Keist, Ruth; Krstic, Dimitrije; Konietzko, Uwe; Klohs, Jan; Nitsch, Roger M; Rudin, Markus

    2014-10-08

    Impairment of brain functional connectivity (FC) is thought to be an early event occurring in diseases with cerebral amyloidosis, such as Alzheimer's disease. Regions sustaining altered functional networks have been shown to colocalize with regions marked with amyloid plaques burden suggesting a strong link between FC and amyloidosis. Whether the decline in FC precedes amyloid plaque deposition or is a consequence thereof is currently unknown. The sequence of events during early stages of the disease is difficult to capture in humans due to the difficulties in providing an early diagnosis and also in view of the heterogeneity among patients. Transgenic mouse lines overexpressing amyloid precursor proteins develop cerebral amyloidosis and constitute an attractive model system for studying the relationship between plaque and functional changes. In this study, ArcAβ transgenic and wild-type mice were imaged using resting-state fMRI methods across their life-span in a cross-sectional design to analyze changes in FC in relation to the pathology. Transgenic mice show compromised development of FC during the first months of postnatal life compared with wild-type animals, resulting in functional impairments that affect in particular the sensory-motor cortex already in preplaque stage. These functional alterations were accompanied by structural changes as reflected by reduced fractional anisotropy values, as derived from diffusion tensor imaging. Our results suggest cerebral amyloidosis in mice is preceded by impairment of neuronal networks and white matter structures. FC analysis in mice is an attractive tool for studying the implications of impaired neuronal networks in models of cerebral amyloid pathology.

  14. 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.

  15. 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.

  16. Anakinra induces complete remission of nephrotic syndrome in a patient with familial mediterranean fever and amyloidosis.

    PubMed

    Sevillano, Ángel M; Hernandez, Eduardo; Gonzalez, Esther; Mateo, Isabel; Gutierrez, Eduardo; Morales, Enrique; Praga, Manuel

    2016-01-01

    Renal amyloidosis is one of the most severe complications of familial Mediterranean fever (FMF). Colchicine has reduced the incidence of this complication, which now only appears in untreated, under-treated and resistant patients, but it is usually ineffective in patients with advanced amyloidosis. Here we report a patient with FMF and biopsy-proven amyloidosis who presented with nephrotic syndrome despite colchicine treatment. Anakinra (an interleukin-1β inhibitor) was started and a dramatic complete remission of nephrotic syndrome was observed in the following months. Anakinra can be an effective treatment for FMF patients with severe secondary amyloidosis.

  17. Pregnancy outcome of five patients with renal amyloidosis regarding familial Mediterranean fever.

    PubMed

    Turgal, Mert; Selcuk, Ilker; Ozyuncu, Ozgur

    2014-03-01

    Familial Mediterranean fever (FMF) is an autosomal recessive disease affecting mainly patients of the Mediterranean basin and its major complication is the development of renal AA amyloidosis. On the other hand pregnancy with amyloidosis is not common; nevertheless, amyloidosis will complicate pregnancies also with the underlying disease and may cause terrible perinatal morbidities and mortalities. We report here the cases of five pregnant women and their pregnancy outcomes, who have been diagnosed with FMF complicated by renal amyloidosis. In the five cases, we observed that increased pregnancy complication such as small for gestational age, intrauterine growth restriction, preeclampsia and preterm birth.

  18. [Ulcerative colitis and amyloidosis. Presentation of a case and review of the literature].

    PubMed

    Triviño, A; Sánchez Lombraña, J L; Linares, A; Pérez, R; Herrero Zapatero, A; Rodrigo, L

    1992-08-01

    A case of ulcerative colitis associated with secondary amyloidosis in a 62-year-old man who died from septic shock and pneumonia complicating head injury is reported. Amyloid deposition was incidentally found at autopsy. Proteinuria and hepatomegaly discovered a few days before his death were the only signs of amyloidosis. The postmortem examination showed chronic ulcerative colitis (remitting form) with pseudo-polyps and amyloid deposition in the liver, spleen, pancreas, rectum, adrenals and kidneys. Although secondary amyloidosis complicating with Crohn's disease has been frequently reported, amyloidosis associated with ulcerative colitis has been exceptionally described and only 10 cases have been collected from the literature.

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

    NASA Astrophysics Data System (ADS)

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

    2016-09-01

    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.

  20. 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

  1. Guideline of transthyretin-related hereditary amyloidosis for clinicians

    PubMed Central

    2013-01-01

    Transthyretin amyloidosis is a progressive and eventually fatal disease primarily characterized by sensory, motor, and autonomic neuropathy and/or cardiomyopathy. Given its phenotypic unpredictability and variability, transthyretin amyloidosis can be difficult to recognize and manage. Misdiagnosis is common, and patients may wait several years before accurate diagnosis, risking additional significant irreversible deterioration. This article aims to help physicians better understand transthyretin amyloidosis—and, specifically, familial amyloidotic polyneuropathy—so they can recognize and manage the disease more easily and discuss it with their patients. We provide guidance on making a definitive diagnosis, explain methods for disease staging and evaluation of disease progression, and discuss symptom mitigation and treatment strategies, including liver transplant and several pharmacotherapies that have shown promise in clinical trials. PMID:23425518

  2. Senile cardiac amyloidosis: an underappreciated cause of heart failure

    PubMed Central

    Shah, Shreena; Dungu, Jason; Dubrey, Simon William

    2013-01-01

    This case presents a patient with biopsy-proven, wild-type transthyretin (TTR) senile amyloidosis. The case was that of a man in his early 70s who presented with gradually progressive symptoms and signs of heart failure. The recent history included an episode of severe pancreatitis secondary to cholelithiasis and subsequently (and incidentally) noted hepatomegaly and marked ascites. Further evaluation of the aetiology of the heart failure, through echocardiography, coronary angiography and endomyocardial biopsy, led to an exact diagnosis of SSA. The patient is being treated with conventional heart failure medications while consideration is given to the use of diflusinal as an antiamyloidogenic small molecular stabiliser of TTR. Monitoring and further management advice are being coordinated by the National Amyloidosis Centre. PMID:23391947

  3. Cardiac amyloidosis imaged by dual-source computed tomography.

    PubMed

    Marwan, Mohamed; Pflederer, Tobias; Ropers, Dieter; Schmid, Michael; Wasmeier, Gerald; Söder, Stephan; Daniel, Werner G; Achenbach, Stephan

    2008-11-01

    The ability of contrast-enhanced CT to detect "late enhancement" in a fashion similar to magnetic resonance imaging has been reported previously. Typical myocardial distribution patterns of "late enhancement" have been described for MRI. The same patterns can be observed in CT imaging, albeit at a lower signal to noise ratio. We report a case of cardiac amyloidosis with a typical pattern of subendocardial, circumferential late enhancement in all four cardiac chambers.

  4. The prognostic value of diagnosing concurrent multiple myeloma in immunoglobulin light chain amyloidosis.

    PubMed

    Dinner, Shira; Witteles, Wesley; Witteles, Ronald; Lam, Anthony; Arai, Sally; Lafayette, Richard; George, Tracy I; Schrier, Stanley L; Liedtke, Michaela

    2013-05-01

    The prevalence and prognostic value of a concomitant diagnosis of symptomatic or asymptomatic multiple myeloma (MM), as defined by the current International Myeloma Working Group (IMWG) criteria, in patients with immunoglobulin light chain amyloidosis (AL), are unknown. We studied 46 consecutive patients with AL who underwent quantification of serum M-protein and clonal bone marrow plasma cells, as well as a comprehensive evaluation for end organ damage by MM. Using standard morphology and CD138 immunohistochemical staining, 57% and 80% of patients were found to have concomitant MM, respectively. Nine patients exhibited end organ damage consistent with a diagnosis of symptomatic MM. While overall survival was similar between AL patients with or without concurrent myeloma (1-year overall survival 68% vs. 87%; P = 0.27), a diagnosis of symptomatic myeloma was associated with inferior outcome (1-year overall survival 39% vs. 81%; P = 0.005). Quantification of bone marrow plasma cells by both standard morphology and CD138 immunohistochemistry identified a much higher prevalence of concurrent MM in patients with AL than previously reported. Evaluation of bone marrow plasma cell infiltration and presence of myeloma associated end organ damage could be clinically useful for prognostication of patients with AL.

  5. Recurrent mutations of MAPK pathway genes in multiple myeloma but not in amyloid light-chain amyloidosis

    PubMed Central

    Kim, Nayoung K.D.; Yun, Jae Won; Hwang, Jee Hyang; Kim, Kihyun; Park, Woong-Yang

    2016-01-01

    Clinically applicable platforms revealing actionable genomic alterations may improve the treatment efficacy of myeloma patients. In this pilot study, we used a high depth targeted sequencing panel containing 83 anti-cancer drug target genes to sequence genomic DNAs extracted from bone marrow aspirates of 23 patients with myeloma and 12 patients with amyloid light-chain (AL) amyloidosis. Mutation analysis revealed NRAS as the most commonly mutated gene (30%, 7/23) in myeloma patients followed by KRAS (26%, 6/23) and BRAF (22%, 5/23). However, no significant mutations were found in the 12 patients with AL amyloidosis. Notably, 6 of the 23 myeloma patients showed multi-site and/or multi-gene mutations in NRAS, KRAS, or BRAF, indicating compound aberrations in the Mitogen activated protein kinase (MAPK) pathway. Gene panel sequencing also revealed cytogenetic abnormalities associated with prognosis in myeloma patients. In conclusion, our pilot study suggests that targeted gene sequencing may have an important prognostic value for myeloma patients for the identification of actionable genomic alterations and cytogenetic aberrations. PMID:27634910

  6. Heart transplantation and cardiac amyloidosis: approach to screening and novel management strategies.

    PubMed

    Varr, Brandon C; Liedtke, Michaela; Arai, Sally; Lafayette, Richard A; Schrier, Stanley L; Witteles, Ronald M

    2012-03-01

    Limited data exist regarding screening methods and outcomes for orthotopic heart transplantation (OHT) in cardiac amyloidosis. As a result, uncertainty exists over the best approach to OHT for cardiac amyloidosis and for the timing of critical post-transplant therapies. This article reviews 6 patients who underwent OHT for cardiac amyloidosis at the Stanford University Amyloid Center from 2008 to present. All patients with light-chain amyloidosis received chemotherapy in the interval between OHT and autologous hematopoietic stem cell transplant. Five patients remain alive up to 25 months after OHT, without evidence of recurrent cardiac amyloid deposition. A novel strategy of OHT, followed by light-chain suppressive chemotherapy before autologous hematopoietic stem cell transplant, is feasible for patients with light-chain amyloidosis.

  7. 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

  8. [Amyloidosis of the tongue as a possible diagnostic manifestation of plasmacytoma].

    PubMed

    Hoefert, S; Schilling, E; Philippou, S; Eufinger, H

    1999-01-01

    Plasmocytic non-Hodgkin's lymphoma is the most common tumor of bone and bone marrow, typically diagnosed by symptoms such as monoclonal paraproteinemia, proteinuria, anemia and hypercalcemia. In its progress, deposits of amyloids in almost all organs can be observed. However, plasmacytomas which are diagnosed by macroglossia of primarily unknown etiology are rare. This case report presents a 61-year-old woman who suffered from a persistent swelling of the tongue with painful ulcerations. A biopsy led to the diagnosis of primary systemic amyloidosis of the light-chain type, which subsequently proved to be a plasmacytoma with lambda light-chains stage II after Durie and Salmon. In the course of the disease the patient developed further deposits of amyloids in the whole gastro-enteric system. Macroglossia as a primary manifestation of plasmacytoma is rarely described in medical literature. However, reports on deposits of amyloid in the tongue in advanced stages of disease are well known.

  9. Unicentric Castleman’s disease associated with end stage renal disease caused by amyloidosis

    PubMed Central

    Eroglu, Eray; Kocyigit, Ismail; Unal, Aydin; Sipahioglu, Murat Hayri; Akgun, Hulya; Kaynar, Leylagul; Tokgoz, Bulent; Oymak, Oktay

    2017-01-01

    Castleman’s disease (CD), also known as angiofolicular lymph node hyperplasia, is a rare heterogenous group of lymphoproliferative disorders. Histologically, it can be classified as hyaline vascular type, plasma cell type, or mixed type. Clinically two different subtypes of the CD are present: Unicentric and multicentric. Unicentric CD is generally asymptomatic and associated with hyaline vascular type, and its diagnoses depend on the localized lymphadenopathy on examination or imaging studies. However, multicentric CD presents with generalized lymphadenopathy and systemic symptoms including malaise, fever, night sweats, weight loss, and it is associated with the plasma cell type and mix type. Herein, we report a patient with unicentric CD of the plasma cell type without systemic symptoms, who developed end stage renal failure caused by amyloidosis 6 years after onset of CD. PMID:28352636

  10. Crystal structure of novel compounds in the systems Zr-Cu-Al, Mo-Pd-Al and partial phase equilibria in the Mo-Pd-Al system.

    PubMed

    Khan, Atta U; Rogl, P; Giester, G

    2012-02-28

    The crystal structures of three Al-rich compounds have been solved from X-ray single crystal diffractometry: τ(1)-MoPd(2-x)Al(8+x) (x = 0.067); τ(7)-Zr(Cu(1-x)Al(x))(12) (x = 0.514) and τ(9)-ZrCu(1-x)Al(4) (x = 0.144). τ(1)-MoPd(2-x)Al(8+x) adopts a unique structure type (space group Pbcm; lattice parameters a = 0.78153(2), b = 1.02643(3) and c = 0.86098(2) nm), which can be conceived as a superstructure of the Mo(Cu(x)Al(1-x))(6)Al(4) type. Whereas Mo-atoms occupy the 4d site, Pd(2) occupies the 4c site, Al and Pd(1) atoms randomly share the 4d position and the rest of the positions are fully occupied by Al. A Bärnighausen tree documents the crystallographic group-subgroup relation between the structure types of Mo(Cu(x)Al(1-x))(6)Al(4) and τ(1). τ(7)-Zr(Cu(1-x)Al(x))(12) (x = 0.514) has been confirmed to crystallize with the ThMn(12) type (space group I4/mmm; lattice parameters a = 0.85243(2) and c = 0.50862(3) nm). In total, 4 crystallographic sites were defined, out of which, Zr occupies site 2a, the 8f site is fully occupied by Cu, the 8i site is entirely occupied by Al, but the 8j site turned out to comprise a random mixture of Cu and Al atoms. The compound τ(9)-ZrCu(1-x)Al(4) (x = 0.144) crystallizes in a unique structure type (space group P4/nmm; lattice parameters a = 0.40275(3) and c = 1.17688(4) nm) which exhibits full atom order but a vacancy (14.4%) on the 2c site, shared with Cu atoms. τ(9)-ZrCu(1-x)Al(4) is a superstructure of Cu with an arrangement of three unit cells of Cu in the direction of the c-axis. A Bärnighausen tree documents this relationship. The ZrCu(1-x)Al(4) type (n = 3) is part of a series of structures which follow this building principle: Cu (n = 1), TiAl(3) (n = 2), τ(5)-TiNi(2-x)Al(5) (n = 4), HfGa(2) (n = 6) and Cu(3)Pd (n = 7). A partial isothermal section for the Al-rich part of the Mo-Pd-Al system at 860 °C has been established with two ternary compounds τ(1)-MoPd(2-x)Al(8+x) and τ(2) (unknown structure). The

  11. Solid-state NMR chemical shift assignments for AL-09 VL immunoglobulin light chain fibrils.

    PubMed

    Piehl, Dennis W; Blancas-Mejía, Luis M; Ramirez-Alvarado, Marina; Rienstra, Chad M

    2017-04-01

    Light chain (AL) amyloidosis is a systemic disease characterized by the formation of immunoglobulin light-chain fibrils in critical organs of the body. The light-chain protein AL-09 presents one severe case of cardiac AL amyloidosis, which contains seven mutations in the variable domain (VL) relative to its germline counterpart, κI O18/O8 VL. Three of these mutations are non-conservative-Y87H, N34I, and K42Q-and previous work has shown that they are responsible for significantly reducing the protein's thermodynamic stability, allowing fibril formation to occur with fast kinetics and across a wide-range of pH conditions. Currently, however, there is extremely limited structural information available which explicitly describes the residues that are involved in supporting the misfolded fibril structure. Here, we assign the site-specific (15)N and (13)C chemical shifts of the rigid residues of AL-09 VL fibrils by solid-state NMR, reporting on the regions of the protein involved in the fibril as well as the extent of secondary structure.

  12. Transthyretin-related hereditary amyloidosis with recurrent vomiting and renal insufficiency as the initial presentation

    PubMed Central

    Xu, Jing; Yang, Meng; Pan, Xiaoxia; Yu, Xialian; Xie, Jingyuan; Ren, Hong; Li, Xiao; Chen, Nan

    2017-01-01

    Abstract Rationale: Hereditary amyloidosis is diagnosed worldwidely with an increasing incidence. As the most common form, transthyretin-related hereditary amyloidosis (ATTR amyloidosis) is an autosomal dominant inherited disease due to mutations of TTR. Over the past several decades, more than 130 mutations have been reported. Previous studies suggested that ATTR amyloidosis initially showed polyneuropathy and autonomic dysfunction but later involving many visceral organs, such as kidney. Patient concerns: A young proband carrying TTR p.Leu75Pro mutation, a reported aggressive variant, initially presenting repeat vomiting and impaired renal function was described in a Chinese family. Diagnoses: ATTR amyloidosis patient was diagnosed by renal biopsy and gene sequencing. Interventions: Allograft liver transplantation (LT). Outcomes: Symptom relief but serum creatinine increased. Lessons subsections: This case illustrated the clinical and pathologic phenotype of an ATTR amyloidosis patient who initially presented impaired renal function and p.Leu75Pro variant was found by sequencing the coding region of TTR gene. Kidney is one of the most common and vulnerable organs of amyloidosis, and renal function should be closely monitored. PMID:28272196

  13. Secondary Cutaneous Amyloidosis in a Patient with Mycosis Fungoides

    PubMed Central

    Nam, Chan Hee; Park, Min Kee; Choi, Mi Soo; Hong, Seung Phil; Park, Byung Cheol

    2017-01-01

    Secondary cutaneous amyloidosis refers to clinically unapparent amyloid deposits within the skin in association with a pre-existing skin condition or skin tumors, such as basal cell carcinoma, porokeratosis, solar elastosis, Bowen's disease, and mycosis fungoides. A 70-year-old woman presented with a 6-month history of asymptomatic multiple yellowish plaques on both legs. She had been diagnosed with mycosis fungoides 7 years ago and was treated with psoralen and ultraviolet A radiation (PUVA) therapy, narrow-band ultraviolet B (UVB) therapy, and acitretin for 5 years. Finally, she reached complete remission of mycosis fungoides. However, new yellowish lesions started to appear 1 year after discontinuing the phototherapy. A physical examination revealed multiple yellowish plaques on both extremities. The plaques were well circumscribed and slightly elevated. All laboratory tests were normal. A biopsy specimen showed multiple nodular deposits of eosinophilic amorphous material in papillary dermis and upper reticular dermis. The deposits represented apple green birefringence on Congo red stain viewed under polarized light. Acellular small nodules in the upper dermis consisted of randomly oriented, non-branching, 6.67~12.7 nm thick amyloid fibrils on electron microscopy. We report an interesting and rare case of secondary cutaneous amyloidosis after narrow-band UVB therapy and PUVA therapy in a patient with mycosis fungoides. PMID:28223751

  14. Secondary Cutaneous Amyloidosis in a Patient with Mycosis Fungoides.

    PubMed

    Nam, Chan Hee; Park, Min Kee; Choi, Mi Soo; Hong, Seung Phil; Park, Byung Cheol; Kim, Myung Hwa

    2017-02-01

    Secondary cutaneous amyloidosis refers to clinically unapparent amyloid deposits within the skin in association with a pre-existing skin condition or skin tumors, such as basal cell carcinoma, porokeratosis, solar elastosis, Bowen's disease, and mycosis fungoides. A 70-year-old woman presented with a 6-month history of asymptomatic multiple yellowish plaques on both legs. She had been diagnosed with mycosis fungoides 7 years ago and was treated with psoralen and ultraviolet A radiation (PUVA) therapy, narrow-band ultraviolet B (UVB) therapy, and acitretin for 5 years. Finally, she reached complete remission of mycosis fungoides. However, new yellowish lesions started to appear 1 year after discontinuing the phototherapy. A physical examination revealed multiple yellowish plaques on both extremities. The plaques were well circumscribed and slightly elevated. All laboratory tests were normal. A biopsy specimen showed multiple nodular deposits of eosinophilic amorphous material in papillary dermis and upper reticular dermis. The deposits represented apple green birefringence on Congo red stain viewed under polarized light. Acellular small nodules in the upper dermis consisted of randomly oriented, non-branching, 6.67~12.7 nm thick amyloid fibrils on electron microscopy. We report an interesting and rare case of secondary cutaneous amyloidosis after narrow-band UVB therapy and PUVA therapy in a patient with mycosis fungoides.

  15. An Assessment of the Al- Fe- N System

    NASA Astrophysics Data System (ADS)

    Hillert, Mats; Jonsson, Stefan

    1992-11-01

    The thermodynamic properties of the Al-Fe-N system are assessed, taking various types of information into account. For solid AIN, a description very similar to that given by JANAF is found to yield reasonable predictions for the solubility of A1N in face-centered cubic (fcc) Fe and in liquid Fe. An ionic two-sublattice model is applied to the liquid phase, containing two N species, N-3 and N0 The melting point of A1N is taken as 3068 K, and a required gas pressure of 9.75 bar is predicted. A sublimation point of 2690 K at 1 bar is also predicted. A plot of the liquidus surfaces in the Fe-rich end of the Al-Fe-N system is presented.

  16. Advanced Launch System (ALS) Space Transportation Expert System Study

    DTIC Science & Technology

    1991-03-01

    CONSIDERATIONS, CONTRACTUAL OBLIGATIONS, OR NOTICE ON A SPECIFIC DOCUMENT. DISCLAIMER NOTICE THIS DOCUMENT IS BEST QUALITY AVAILABLE. THE COPY...System Assessment Methodology 2- 11 2.3.1.1 Methodology Overview 2- 11 2.3.1.2 Approach 2- 11 2.3.1.3 Defmitions of Attributes 2- 12 2.3.2 Assessment...how the use of knowledge-based systems can help increase autonomy. A design approach to this degree of autonomy will be demonstrated in Phase 2 (ADP

  17. Improvement of the Electrochemical Behavior of Steel Surfaces Using a [Ti-Al/Ti-Al-N] n Multilayer System

    NASA Astrophysics Data System (ADS)

    Ipaz, L.; Aperador, W.; Caicedo, J. C.; Esteve, J.; Zambrano, G.

    2013-05-01

    The aim of this work is to improve the corrosion resistance of AISI D3 steel surfaces using a [Ti-Al/Ti-Al-N] n multilayer system deposited with different periods (Λ) and bilayer numbers ( n), via magnetron co-sputtering pulsed d.c. procedure, from a metallic (Ti-Al) binary target. The multilayer coatings were characterized by cross-sectional scanning electron microscopy that showed the modulation and microstructure of the [Ti-Al/Ti-Al-N] n multilayer system. The composition of the single Ti-Al and Ti-Al-N layer films was studied via x-ray photoelectron spectroscopy, where typical signals for Ti2p1/2, Ti2p, N1s, and Al2p3/2 were detected. The electrochemical properties were evaluated by electrochemical impedance spectroscopy and Tafel polarization curves. The optimal electrochemical behavior was obtained for the [Ti-Al/Ti-Al-N] n multilayered period of Λ = 25 nm (100 bilayers). At these conditions, the maximum polarization resistance (1719.32 kΩ cm2) and corrosion rate (0.7 μmy) were 300 and 35 times higher than that of uncoated AISI D3 steel substrate (5.61 kΩ cm2 and 25 μmy, respectively). Finally, scanning electron microscopy was used to analyze the [Ti-Al/Ti-Al-N] n multilayered surface after the corrosive attack. The improvement effects in the electrochemical behavior of the AISI D3 coated with the [Ti-Al/Ti-Al-N] n multilayered coatings could be attributed to the number of interfaces that act as obstacles for the inward and outward diffusions of Cl- ions, generating an increment in the energy or potential required for translating the corrosive ions across the coating/substrate interface.

  18. Cutaneous amyloidosis associated with autoimmune hepatitis-primary biliary cirrhosis overlap syndrome.

    PubMed

    González-Moreno, Emmanuel I; Cámara-Lemarroy, Carlos R; Borjas-Almaguer, David O; Martínez-Cabriales, Sylvia A; Paz-Delgadillo, Jonathan; Gutiérrez-Udave, Rodrigo; Ayala-Cortés, Ana S; Ocampo-Candiani, Jorge; Cortéz-Hernández, Carlos A; Maldonado-Garza, Héctor J

    2015-01-01

    Cutaneous amyloidosis is a rare disease characterized by the deposition of amyloid in the dermis. It can be primary or secondary, depending on associated diseases. It has been linked to various autoimmune diseases, including primary biliary cirrhosis. We present the case of a patient with an autoimmune hepatitis-primary biliary cirrhosis overlap syndrome with concomitant cutaneous amyloidosis, a very unusual association, and discuss similar cases and possible pathophysiological implications.

  19. T-cell brain infiltration and immature antigen-presenting cells in transgenic models of Alzheimer's disease-like cerebral amyloidosis.

    PubMed

    Ferretti, M T; Merlini, M; Späni, C; Gericke, C; Schweizer, N; Enzmann, G; Engelhardt, B; Kulic, L; Suter, T; Nitsch, R M

    2016-05-01

    Cerebral beta-amyloidosis, one of the pathological hallmarks of Alzheimer's disease (AD), elicits a well-characterised, microglia-mediated local innate immune response. In contrast, it is not clear whether cells of the adaptive immune system, in particular T-cells, react to cerebral amyloidosis in AD. Even though parenchymal T-cells have been described in post-mortem brains of AD patients, it is not known whether infiltrating T-cells are specifically recruited to the extracellular deposits of beta-amyloid, and whether they are locally activated into proliferating, effector cells upon interaction with antigen-presenting cells (APCs). To address these issues we have analysed by confocal microscopy and flow-cytometry the localisation and activation status of both T-cells and APCs in transgenic (tg) mice models of AD-like cerebral amyloidosis. Increased numbers of infiltrating T-cells were found in amyloid-burdened brain regions of tg mice, with concomitant up-regulation of endothelial adhesion molecules ICAM-1 and VCAM-1, compared to non-tg littermates. The infiltrating T-cells in tg brains did not co-localise with amyloid plaques, produced less interferon-gamma than those in controls and did not proliferate locally. Bona-fide dendritic cells were virtually absent from the brain parenchyma of both non-tg and tg mice, and APCs from tg brains showed an immature phenotype, with accumulation of MHC-II in intracellular compartments. These results indicate that cerebral amyloidosis promotes T-cell infiltration but interferes with local antigen presentation and T-cell activation. The inability of the brain immune surveillance to orchestrate a protective immune response to amyloid-beta peptide might contribute to the accumulation of amyloid in the progression of the disease.

  20. High-temperature phase equilibria in the Al-rich corner of the Al-Ti-C system

    SciTech Connect

    Frage, N.; Frumin, N.; Levin, L.; Polak, M.; Dariel, M.P.

    1998-04-01

    A thermodynamic analysis of the Al-rich corner in the ternary Al-Ti-C diagram, providing phase relations and regions of phase stability, is presented. An invariant four-phase equilibrium between Al, Al{sub 4}C{sub 3}, Al{sub 3}Ti, and TiC{sub x} takes place at 0.53 at. pct Ti, 7.10{sup {minus}6} at. pct C, and TiC{sub 0.883} at 966 K. The carbon content of the TiC{sub x} phase, which extends from x = 0.48 to 0.98, exerts a significant effect on phase relationships in this ternary system. In particular, it is shown that stoichiometric TiC is not stable in the presence of liquid Al. For example, at 1,300 K, a two-phase equilibrium between Al{sub L} and TiC{sub x} exists only in the 0.91 < x < 0.82 range. Thus, the interaction of Al{sub L} with stoichiometric TiC leads to the formation of the Al{sub 4}C{sub 3} aluminum carbide phase, whereas for x < 0.82, only the intermetallic compound Al{sub 3}Ti can form at this temperature. The results of this analysis were confirmed by X-ray diffraction (XRD) measurements of relevant composites.

  1. Metastability in the MgAl2O4-Al2O3 System

    DOE PAGES

    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

  2. Unusual Case of Cardiac Amyloidosis Preceded by a Twenty-year History of Dilated Cardiomyopathy and Heart Failure.

    PubMed

    Shimada, Seijiro; Maekura, Shunji; Ino, Hikaru; Matsuura, Masayosi; Masunaga, Nobutaka; Matsumoto, Takahiro; Hama, Junkichi

    2016-01-01

    Amyloidosis is a well-known but uncommon disease, and the physician must maintain a high index of suspicion in order to make a timely diagnosis. The expected survival of patients with cardiac amyloidosis is generally poor. In particular, survival has been reported to be 4-12 months for patients with amyloid light-chain amyloidosis with congestive heart failure. We herein report a rare case of cardiac amyloidosis in which the patient presented with cardiac hypertrophy after a 20-year history of dilated cardiomyopathy and heart failure.

  3. Acceleration of Amyloidosis by Inflammation in the Amyloid-Beta Marmoset Monkey Model of Alzheimer’s Disease

    PubMed Central

    Philippens, Ingrid H.; Ormel, Paul R.; Baarends, Guus; Johansson, Maja; Remarque, Ed J.; Doverskog, Magnus

    2016-01-01

    Background: The immune system is increasingly mentioned as a potential target for Alzheimer’s disease (AD) treatment. Objective: In the present pilot study, the effect of (neuro)inflammation on amyloidopathy was investigated in the marmoset monkey, which has potential as an AD animal model due to its natural cerebral amyloidosis similar to humans. Methods: Six adult/aged marmosets (Callithrix jacchus) were intracranial injected with amyloid-beta (Aβ) fibrils at three cortical locations in the right hemisphere. Additionally, in half of the monkeys, lipopolysaccharide (LPS) was co-injected with the Aβ fibrils and injected in the other hemisphere without Aβ fibrils. The other three monkeys received phosphate buffered saline instead of LPS, as a control for the inflammatory state. The effect of inflammation on amyloidopathy was also investigated in an additional monkey that suffered from chronic inflammatory wasting syndrome. Mirror histology sections were analyzed to assess amyloidopathy and immune reaction, and peripheral blood for AD biomarker expression. Results: All LPS-injected monkeys showed an early AD immune blood cell expression profile on CD95 and CD45RA. Two out of three monkeys injected with Aβ and LPS and the additional monkey, suffering from chronic inflammation, developed plaques. None of the controls, injected with Aβ only, developed any plaques. Conclusion: This study shows the importance of immune modulation on the susceptibility for amyloidosis, a hallmark of AD, which offers new perspectives for disease modifying approaches in AD. PMID:27662314

  4. Homozygosity for the E526V Mutation in Fibrinogen A Alpha-Chain Amyloidosis: The First Report

    PubMed Central

    Tavares, Isabel; Lobato, Luísa; Matos, Carlos; Santos, Josefina; Moreira, Paul; Saraiva, Maria João; Castro Henriques, António

    2015-01-01

    Systemic hereditary amyloidoses are autosomal dominant diseases associated with mutations in genes encoding ten different proteins. The clinical phenotype has implications on therapeutic approach, but it is commonly variable and largely dependent on the type of mutation. Except for rare cases involving gelsolin or transthyretin, patients are heterozygous for the amyloidogenic variants. Here we describe the first patient identified worldwide as homozygous for a nephropathic amyloidosis, involving the fibrinogen variant associated with the fibrinogen alpha-chain E526V (p.Glu545Val) mutation. In 1989, a 44-year-old woman presented with hypertension, hepatosplenomegaly, nephrotic syndrome, and renal failure. She started hemodialysis in 1990 and 6 years later underwent isolated kidney transplantation from a deceased donor. Graft function and clinical status were unremarkable for 16 years, despite progressively increased left ventricular mass on echocardiography. In 2012, 4 months before death, she deteriorated rapidly with severe heart failure, precipitated by Clostridium difficile colitis and urosepsis. Affected family members developed nephropathy, on average, nearly three decades later, which may be explained by the gene dosage effects on the phenotype of E526V (p.Glu545Val) fibrinogen A alpha-chain amyloidosis. PMID:26199771

  5. Long-period ordered superstructures that appear in an (Al,Ga)-rich (Al,Ga)Ti system

    NASA Astrophysics Data System (ADS)

    Nakano, Takayoshi; Hagihara, Koji; Hata, Satoshi; Shigyo, Hajime; Nakashima, Hideharu; Umakoshi, Yukichi; Arya, Ashok; Kulkarni, Ulhas D.

    2013-01-01

    Long-period ordered superstructures in an (Al,Ga)-rich (Al,Ga)Ti system composed of (Al,Ga) x Ti100- x (x = 50-65 at. %) were investigated by using melt-spun ribbons, focusing on the ordering process of the superstructural phases. Compositional regions in which h-(Al,Ga)2Ti (Ga2Zr-type), (Al,Ga)5Ti3 and (Al,Ga)3Ti2 with threefold, fourfold and fivefold periodicity, respectively, appeared coherently in the L10 matrix were identified by electron diffraction patterns in two different temperature conditions of as-spun (a relatively high-temperature state) and heat-treatment at 700°C (a relatively low-temperature state). The (Al,Ga)5Ti3 superstructural phase always existed between the compositional region where h-(Al,Ga)2Ti and (Al,Ga)3Ti2 dominantly appear. Therefore, periodical ordering proceeds following the periodicity of the concentration wave of pure (Al,Ga) layers parallel to {310) in the Ti (002) layers from threefold to fivefold periodicity via fourfold periodicity. We found that the (Al,Ga)3Ti2 long-period superstructural phase preferentially appeared with increasing Ga concentration in the melt-spun ribbon annealed at 700°C. In other words, the h-(Al,Ga)2Ti superstructure with threefold periodicity preferentially appeared at the high temperature and a low Ga concentration. Thus, the (Al,Ga)3Ti2-type superstructure was never produced without the addition of Ga in the present study. The long-period ordered superstructures were composed of three pairs of primitive cells, (Al,Ga)Ti2, (Al,Ga)Ti3 and (Al,Ga)Ti4, with periodic atomic arrangements corresponding to lean rhombus, fat rhombus and square, respectively. We discuss the ordering process throughout the (Al,Ga)5 ? superstructure based on the periodic concentration wave and exchange of atoms. The effect of long-period ordered superstructures on hardness is also mentioned.

  6. 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.

  7. 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.

  8. 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.

  9. 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.

  10. Three German fibrinogen Aalpha-chain amyloidosis patients with the p.Glu526Val mutation.

    PubMed

    Eriksson, Magdalena; Schönland, Stefan; Bergner, Raoul; Hegenbart, Ute; Lohse, Peter; Schmidt, Hartmut; Röcken, Christoph

    2008-07-01

    Plasma protein fibrinogen variants cause fibrinogen A alpha-chain (AFib) amyloidosis, which presents with hypertension, proteinuria, and azotemia. Six AFib mutations have been reported thus far. We identified three patients who presented with marked proteinuria and serum creatinine elevations. Their kidney biopsies revealed destruction of the glomerular architecture by amyloid deposits with typical, apple-green birefringence in polarized light after Congo red staining. We found immunoreactivity against fibrinogen, which is typical for this type of amyloidosis. We sequenced the FGA exon 5 and demonstrated heterozygosity for the p.Glu526Val mutation in all three cases. This amino acid substitution is the most common fibrinogen A alpha-chain variant causing AFib amyloidosis. The mutation has been reported in individuals of European and American descent but not yet in German patients. AFib amyloidosis should therefore be considered an important differential diagnosis in German patients with renal amyloidosis. In the cases described here, the use of antibodies directed against fibrinogen, followed by direct gene sequencing, revealed the underlying cause.

  11. 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

  12. Higher stem cell dose infusion after intensive chemotherapy does not improve symptom burden in older patients with multiple myeloma and amyloidosis

    PubMed Central

    Shah, Nina; Shi, Qiuling; Williams, Loretta A.; Mendoza, Tito R.; Wang, Xin Shelley; Reuben, James M.; Dougherty, Patrick M.; Bashir, Qaiser; Qazilbash, Muzaffar H.; Champlin, Richard E.; Cleeland, Charles S.; Giralt, Sergio A.

    2015-01-01

    Autologous hematopoietic stem cell transplantation (ASCT) for multiple myeloma (MM) is associated with high symptom burden, particularly for older patients and those with amyloid light-chain (AL) amyloidosis. Symptom burden peaks during leukopenia. We hypothesized that higher doses of CD34+ stem cells would be associated with an improved symptom outcome. Patients undergoing ASCT for MM who were ≥60 years old or had AL amyloidosis were randomized to receive either a standard (4–6×106 cells/kg) or high dose (10–15×106 cells/kg) of CD34+ cells after melphalan 200 mg/m2. Symptom burden was assessed via the MD Anderson Symptom Inventory MM module (MDASI-MM). Eighty patients were enrolled. Median CD34+ cell doses were 5.1×106 cells/kg (standard dose) and 10.5×106 cells/kg (high dose). The most severe symptoms during the first week were fatigue, lack of appetite, drowsiness, disturbed sleep, and pain. The AUC for the mean composite severity score of these symptoms was similar between treatment arms (P = .819). Median times to neutrophil, lymphocyte, and platelet engraftment were also similar between groups. IL-6 increased similarly for both groups throughout the ASCT course. Infusion of higher autologous stem cell dose after high-dose chemotherapy does not yield a difference in symptom burden or engraftment time in the first few weeks post-ASCT. PMID:26253006

  13. NiAl-based Polyphase in situ Composites in the NiAl-Ta-X (X = Cr, Mo, or V) Systems

    NASA Technical Reports Server (NTRS)

    Johnson, D. R.; Oliver, B. F.; Noebe, R. D.; Whittenberger, J. D.

    1995-01-01

    Polyphase in situ composites were generated by directional solidification of ternary eutectics. This work was performed to discover if a balance of properties could be produced by combining the NiAl-Laves phase and the NiAl-refractory metal phase eutectics. The systems investigated were the Ni-Al-Ta-X (X = Cr, Mo, or V) alloys. Ternary eutectics were found in each of these systems and the eutectic composition, temperature, and morphology were determined. The ternary eutectic systems examined were the NiAl-NiAlTa-(Mo, Ta), NiAl-(Cr, Al) NiTa-Cr, and the NiAl-NiAlTa-V systems. Each eutectic consists of NiAl, a C14 Laves phase, and a refractory metal phase. Directional solidification was performed by containerless processing techniques in a levitation zone refiner to minimize alloy contamination. Room temperature fracture toughness of these materials was determined by a four-point bend test. Preliminary creep behavior was determined by compression tests at elevated temperatures, 1100-l400 K. Of the ternary eutectics, the one in the NiAl-Ta-Cr system was found to be the most promising. The fracture toughness of the NiAl-(Cr, Al)NiTa-Cr eutectic was intermediate between the values of the NiAl-NiAlTa eutectic and the NiAl-Cr eutectic. The creep strength of this ternary eutectic was similar to or greater than that of the NiAl-Cr eutectic.

  14. Compact TPV Generation System Using Al2O3/Er3Al5O12 Eutectic Ceramics Selective Emitters

    NASA Astrophysics Data System (ADS)

    Adachi, Yusuke; Yugami, Hiroo; Shibata, Kouji; Nakagawa, Narihito

    2004-11-01

    The emissive properties of Al2O3Er3Al5O12 eutectic ceramics have been measured as a candidate of selective emitters. It is identified that the Al2O3Er3Al5O12 eutectic ceramics have selective emission bands at the wavelength of 1.5 μm, and the emittance at the emission peak is over 0.8. The selective emissive power for GaSb photovoltaic (PV) cells of 49 kW/m2 is achieved by this material at 1600 K. Since the emission bands match the sensitive region of GaSb PV cells, the Al2O3Er3Al5O12 eutectic ceramics are suited as emitter materials in thermophotovoltaic (TPV) systems using GaSb PV cells. The optical design of small-scale TPV systems for portable power sources has been investigated with the three-dimensional ray tracing simulation. Using a mirror/PV optical design, the concentrated thermal radiation is irradiated on PV cells with the view factor over 0.75.

  15. 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

  16. Characterization of proteoglycans associated with mouse splenic AA amyloidosis.

    PubMed Central

    Stenstad, T; Magnus, J H; Husby, G

    1994-01-01

    We here report for the first time on the chemical characteristics of proteoglycans associated with mouse splenic reactive AA amyloid. Amyloid was induced in CBA/J mice by two different procedures; conventional casein treatment and by employing Freund's complete adjuvant, accelerated by Trypan Blue. Pulse-labelling was employed at distinct stages during amyloid development, followed by [35S]proteoglycan characterization of organ extracts. Repetitive 35S injections were also administered during the phase where amyloid deposition occurred most rapidly. Proteoglycans were extracted with guanidine in the presence of protease inhibitors and purified. The results showed that the production of proteoglycans is dramatically enhanced during amyloidogenesis, the glycosaminoglycan and proteoglycan accumulation being not only dependent on alterations in proteoglycan catabolism, but rather on increased synthesis. The increment could be demonstrated even at the stage before microscopic detection of amyloid deposits, clearly suggesting that the upregulation of proteoglycan expression precedes amyloid fibril formation. Two major proteoglycans were found to accumulate in advanced splenic amyloid; one a heparan sulphate proteoglycan of approx. 200 kDa with a core protein of 70 kDa, the other a chondroitin sulphate proteoglycan of smaller size. Moreover, free dermatan sulphate chains seemed to specifically accumulate in the organs during amyloid fibrillogenesis. We suggest that free glycosaminoglycans may be a specific feature of amyloidosis and that different proteoglycans and glycosaminoglycans play a role in formation and stabilization of amyloid fibrils in vivo. Images Figure 2 Figure 6 PMID:7980430

  17. 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.

  18. 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.

  19. 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.

  20. A Study of Diffusivity in the BCC Solid Solution of Nb-Al and Nb-Ti-Al System

    DTIC Science & Technology

    1990-01-01

    A STUDY OF DIFFUSIVITY IN THE BCC SOLID SOLUTION OF Nb-Al AND Nb-Ti-Al SYSTEM P DTIC ELECTE NOV 3o01993 A- J.BY JOSE GUADALUPE LUIS RUIZ APARICIO A...analysis was employed for the evaluation of composition profiles in all the diffusion couples. Tne interdiffusion coefficient for the 0 solid solution of Nb...system suggest that Ti is the fastest element in the J0 solid solution . Qualitatively the penetration tendencies correlate with the melting point of

  1. 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

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

    PubMed

    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

    2013-01-01

    The human germinal centre-associated lymphoma 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 human germinal centre-associated lymphoma directly binds to Syk in B cells, increases its kinase activity on B-cell receptor stimulation and leads to enhanced activation of Syk downstream effectors. To further investigate these findings in vivo, human germinal centre-associated lymphoma transgenic mice were generated. Starting from 12 months of age these mice developed polyclonal B-cell lymphoid hyperplasia, hypergammaglobulinemia and systemic reactive amyloid A (AA) amyloidosis, leading to shortened survival. The lymphoid hyperplasia in the human germinal centre-associated lymphoma 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 human germinal centre-associated lymphoma regulates B-cell receptor signalling in B-lymphocytes which, without appropriate control, may lead to B-cell lymphoproliferation.

  3. 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

  4. 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

  5. Generalized AA-amyloidosis in Siberian tigers (Panthera tigris altaica) with predominant renal medullary amyloid deposition.

    PubMed

    Schulze, C; Brügmann, M; Böer, M; Brandt, H P; Pohlenz, J; Linke, R P

    1998-01-01

    Generalized amyloidosis with predominant renal medullary amyloid deposition was found in four closely related Siberian tigers (Panthera tigris altaica) suffering from end stage kidney diseases. Only minimal to mild amounts of amyloid were deposited in various organs outside the kidneys with individually variable organ involvement. The Congo red staining affinity of amyloid deposits was sensitive to potassium permanganate oxidation. The deposits were further characterized as being of the amyloid-A (AA) type by immunohistochemistry using the mouse monoclonal antibody mc4 directed against a conserved region of the human AA-protein. A combination of immunohistochemistry and Congo red staining was much more sensitive for the diagnosis of amyloid deposits than Congo red staining alone. With this combination, even minimal amyloid deposits were detected that had been missed in the first reading using Congo-red-stained slides alone. Since no common primary cause was identified, the amyloidosis was classified as idiopathic generalized AA-amyloidosis with a potential familial predisposition.

  6. Hot spots in apolipoprotein A-II misfolding and amyloidosis in mice and men

    PubMed Central

    Gursky, Olga

    2014-01-01

    ApoA-II is the second-major protein of high-density lipoproteins. C-terminal extension in human apoA-II or point substitutions in murine apoA-II cause amyloidosis. The molecular mechanism of apolipoprotein misfolding, from the native predominantly α-helical conformation to cross-β-sheet in amyloid, is unknown. We used 12 sequence-based prediction algorithms to identify two ten-residue segments in apoA-II that probably initiate β-aggregation. Previous studies of apoA-II fragments experimentally verify this prediction. Together, experimental and bioinformatics studies explain why the C-terminal extension in human apoA-II causes amyloidosis and why, unlike murine apoA-II, human apoA-II normally does not cause amyloidosis despite its unusually high sequence propensity for β-aggregation. PMID:24561203

  7. (99m)Tc-DPD uptake reflects amyloid fibril composition in hereditary transthyretin amyloidosis.

    PubMed

    Pilebro, Björn; Suhr, Ole B; Näslund, Ulf; Westermark, Per; Lindqvist, Per; Sundström, Torbjörn

    2016-01-01

    Aims In transthyretin amyloid (ATTR) amyloidosis various principal phenotypes have been described: cardiac, neuropathic, or a mixed cardiac and neuropathic. In addition, two different types of amyloid fibrils have been identified (type A and type B). Type B fibrils have thus far only been found in predominantly early-onset V30M and in patients carrying the Y114C mutation, whereas type A is noted in all other mutations currently examined as well as in wild-type ATTR amyloidosis. The fibril type is a determinant of the ATTR V30M disease phenotype. (99m)Tc-DPD scintigraphy is a highly sensitive method for diagnosing heart involvement in ATTR amyloidosis. The objective of this study was to determine the relationship between ATTR fibril composition and (99m)Tc-DPD scintigraphy outcome in patients with biopsy-proven ATTR amyloidosis. Methods Altogether 55 patients with biopsy-proven diagnosis of ATTR amyloidosis and amyloid fibril composition determined were examined by (99m)Tc-DPD scintigraphy. The patients were grouped and compared according to their type of amyloid fibrils. Cardiovascular evaluation included ECG, echocardiography, and cardiac biomarkers. The medical records were scrutinized to identify subjects with hypertension or other diseases that have an impact on cardiac dimensions. Results A total of 97% with type A and none of the patients with type B fibrils displayed (99m)Tc-DPD uptake at scintigraphy (p < 0.001). Findings from analyses of cardiac biomarkers, ECG, and echocardiography, though significantly different, could not differentiate between type A and B fibrils in individual patients. Conclusion In ATTR amyloidosis, the outcome of (99m)Tc-DPD scintigraphy is strongly related to the patients' transthyretin amyloid fibril composition.

  8. Nodular amyloidosis of the lung and the breast mimicking breast carcinoma with pulmonary metastasis.

    PubMed

    Liaw, Y S; Kuo, S H; Yang, P C; Chen, C L; Luh, K T

    1995-05-01

    Nodular amyloidosis of the breast and lung is a rare condition of unknown aetiology. The disease runs a benign course, but offers a diagnostic problem due to nonspecific histological features. We describe the case of a 56 year old woman with a 5 year history of multiple nodules of both lungs and left breast, clinically mimicking breast carcinoma with pulmonary metastasis. To our knowledge, this is the first case of cytologically proven amyloidosis diagnosed by ultrasound-guided percutaneous transthoracic fine-needle aspiration of pulmonary nodules.

  9. Endobronchial amyloidosis mimicking bronchial asthma: a case report and review of the literature

    PubMed Central

    Kang, Hyun-Wook; Oh, Hyung-Joo; Park, Ha Young; Park, Cheol-Kyu; Shin, Hong-Joon; Lim, Jung-Hwan; Kwon, Yong-Soo; Choi, Yoo-Duk

    2016-01-01

    Abstract Among two tracheobronchial forms (local and diffuse) and two parenchymal forms (nodular and alveolar septal) that were reported in previous literature, localized endobronchial amyloidosis is an uncommon disease of unknown cause. Bronchial amyloid deposits can occur as focal nodules or multifocal infiltration of the submucosa. We report the case of a 47-year-old man who had complained of dyspnea and wheezing for 1 month and who had been treated for severe asthma at another hospital. Endobronchial amyloidosis was confirmed by histological examination of the bronchial biopsies.

  10. 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.

  11. Manufacture of the ALS storage ring vacuum system

    NASA Astrophysics Data System (ADS)

    Kennedy, Kurt

    1991-08-01

    The Advanced Light Source (ALS) storage ring has a 4.9 meter magnetic radius and an antechamber type vacuum chamber. These two requirements makes conventional bent tube manufacturing techniques difficult. The ALS sector vacuum chambers have been made by machining two halves out of aluminum plate and welding at the mid plane. Each of these chambers have over 50 penetrations with metal sealed flanges and seven metal sealed poppet valves which use the chamber wall as the valve seat. The sector chambers are 10 meters long and some features in the chambers must be located to .25 mm. This paper describes how and how successfully these features have been achieved.

  12. [Wild-type transthyretin-related cardiac amyloidosis and degenerative aortic stenosis: Two inter-related pathologies in the elderly].

    PubMed

    Calero Núñez, Sofía; Tercero Martínez, Antonia; García López, Juan Carlos; Jiménez-Mazuecos, Jesús

    2016-06-09

    Wild-type transthyretin-related cardiac amyloidosis (ATTRwt) and degenerative aortic stenosis share a common demographic and clinical profile. It was recently suggested that some of the complications arising during and after transcatheter aortic valve replacement (TAVR) could be due to a co-existing cardiac amyloidosis. In a series of autopsies of patients who had undergone TAVR, researchers found ATTR amyloidosis in one third of the cases. A report is presented on two patients with aortic stenosis who were diagnosed with ATTRwt when they were about to undergo a TAVI. ATTRwt is a slowly progressing disease so we need to review the decisions on the therapeutic approach in these patients.

  13. 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.

  14. 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.

  15. The Frequency of Familial Mediterranean Fever Related Amyloidosis in Renal Waiting List for Transplantation

    PubMed Central

    Keles, Mustafa; Eyerci, Nilnur; Uyanik, Abdullah; Aydinli, Bulent; Sahin, Gonul Zisan; Cetinkaya, Ramazan; Pirim, Ibrahim; Polat, Kamil Yalcin

    2010-01-01

    Objective: Our goal is to investigate the distribution of MEFV mutations in patients with renal amyloidosis who are in renal transplant waiting list which is prepared for transplantation. Materials and Methods: FMF was diagnosed in 25 of the 297 patients between the years 2004 and 2008, who were involved in the study (15 male, 10 female; age 34±7.8). 5 out of 25 patients were transplanted, remaining were waiting for Tx. Biopsy results were amyloidosis and taken from renal (n:16), rectal (n:8) and duodenal (1).All of them were carrier of mutations in both pyrin alleles.The primer cause of chronic renal failure in our group was secondary AA amyloidosis. DNA was isolated from 25 whole blood samples. The NanoChip Molecular Biology Workstation (Nanogen) uses electronic microarrays for mutation detection. Exon 2,3,5 and 10 of pyrin gene genotypes were identified in the NanoChip. Results: Genetic analysis of the patients demonstrated that each subject carries either homozygote or compound heterozygote mutations of the gene. The most common mutations were M694V, V726A, E148Q and M680I. Conclusions: The clinic manifestation and complain of our patients were febrile and painful attacks such as in the abdomen, chest and joints due to inflammation of the peritoneum, pleura and synovial membrane. The major problem in FMF is the occurrence of amyloidosis that primarily affects the kidneys causing proteinuria and renal failure. Dialysis and renal transplantation can be treatment, but it is important to diagnose FMF at earliest stages. The percentage of FMF patients in our waiting list was 8.4%. Moreover, in our region FMF incidence is highly frequent, so FMF should be chased by genetically so as to prevent chronic renal failure due to amyloidosis. PMID:25610112

  16. 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

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

    PubMed

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

    2016-06-01

    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.

  18. 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

  19. 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.

  20. Isolated primary amyloidosis of the inferior rectus muscle mimicking Graves' orbitopathy.

    PubMed

    Monteiro, Mário Luiz Ribeiro; Gonçalves, Allan Christian Pieroni; Bezerra, Alanna Mara Pinheiro Sobreira

    2016-01-01

    The diagnosis of Graves' orbitopathy is usually straightforward. However, orbital diseases that mimick some clinical signs of Graves' orbitopathy may cause diagnostic confusion, particularly when associated to some form of thyroid dysfunction. This report describes the rare occurrence of localized inferior rectus muscle amyloidosis in a patient with autoimmune hypothyroidism, who was misdiagnosed as Graves' orbitopathy. A 48-year-old man complained of painless progressive proptosis on the left side and intermittent vertical diplopia for 6 months. The diagnosis of Graves' orbitopathy was entertained after magnetic resonance imaging revealing a markedly enlarged, tendon-sparing inferior rectus enlargement on the left side, and an autoimmune hypothyroidism was disclosed on systemic medical workup. After no clinical improvement with treatment, the patient was referred to an ophthalmologist and further investigation was performed. The presence of calcification in the inferior rectus muscle on computed tomography, associated with the clinical findings led to a diagnostic biopsy, which revealed amyloid deposition. This report emphasizes that a careful evaluation of atypical forms of Graves' orbitopathy may be crucial and should include, yet with rare occurrence, amyloidosis in its differential diagnosis. RESUMO O diagnóstico de orbitopatia de Graves usualmente é fácil de ser estabelecido. No entanto, doenças da órbita que simulam alguns sinais clínicos da orbitopatia de Graves podem levar à confusão diagnóstica, particularmente quando associada à alguma forma de disfunção tireoidiana. Relatamos a ocorrência rara de amiloidose localizada no músculo reto inferior em paciente com hipotireoidismo autoimune, que recebeu inicialmente o diagnóstico errôneo de orbitopatia de Graves. Paciente masculino, 48 anos, com queixa de proptose progressiva e indolor do lado esquerdo e diplopia vertical intermitente há 6 meses. O diagnóstico de orbitopatia de Graves foi

  1. 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

  2. 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.

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

    PubMed Central

    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

    2015-01-01

    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. PMID:26563579

  4. 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.

  5. 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%.

  6. Juvenile idiopathic arthritis complicated by amyloidosis with secondary nephrotic syndrome - effective treatment with tocilizumab.

    PubMed

    Kwiatkowska, Małgorzata; Jednacz, Ewa; Rutkowska-Sak, Lidia

    2015-01-01

    A case report of a boy with juvenile idiopathic arthritis since the age of 2 years, generalized onset, complicated by nephrotic syndrome due to secondary type A amyloidosis is presented. In the patient the disease had an especially severe course, complicated by frequent infections, making routine treatment difficult. Amyloidosis was diagnosed in the 5(th) year of the disease based on a rectal biopsy. Since the disease onset the boy has been taking prednisolone and sequentially cyclosporine A, methotrexate, chlorambucil, etanercept, and cyclophosphamide. Clinical and laboratory remission was observed after treatment with tocilizumab. After 42 months of treatment with tocilizumab the boy's condition is good. There is no pain or joint edema, and no signs of nephrotic syndrome.

  7. Pathological features of amyloidosis in stranded California sea lions (Zalophus californianus).

    PubMed

    Colegrove, K M; Gulland, F M D; Harr, K; Naydan, D K; Lowenstine, L J

    2009-01-01

    Amyloidosis was diagnosed in 26 stranded adult California sea lions between 1983 and 2006 by retrospective case analysis. The kidneys (92.3% of animals), blood vessels (80.7%) and thyroid glands (65.4%) were most commonly affected. Macroscopically, affected kidneys were swollen, with pale tan cortices and loss of corticomedullary differentiation. Amyloid deposits in the kidney were located in the glomeruli, blood vessels, and peritubular interstitium, most prominently in the outer stripe of the medulla. The amyloid deposits were identified as type amyloid A (AA) by potassium permanganate staining and immunolabelling with antibodies against AA protein. Concurrent diseases, including inflammatory processes and genital carcinoma, were common in affected animals. Serum amyloid A concentrations were high (>1200 microg/ml) in six of seven affected sea lions, while the median value in clinically healthy animals was <10 microg/ml. These findings suggest that renal amyloidosis contributes to morbidity and mortality in stranded adult California sea lions.

  8. 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.

  9. [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.

  10. {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.

  11. 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.

  12. 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.

  13. Clinical and laboratory findings in primary generalized and multiple-myeloma-related amyloidosis.

    PubMed Central

    Pruzanski, W.; Katz, A.

    1976-01-01

    Thirty-four patients with primary generalized amyloidosis (PGA) and 14 with multiple-myeloma-related amyloidosis (MRA) were studied. The commonest clinical manifestations in PGA were nephrotic syndrome, hepatomegaly and congestive heart failure, and in MRA, low back pain, plasmacytoma and rheumatoid-arthritis-like syndrome. Eight patients with PGA had limited clinical expression of the disease, such as involvement of only kidneys, joints, parotid glands or gastrointestinal tract; in one patient amyloidosis was limited to lymph nodes. Low serum concentrations of total protein and albumin were common. M components were detected in the serum of 91% of patients with PGA and 92% of patients with MRA: 70% of the M components in PGA and 25% of those in MRA had lambda light chains. Bence Jones proteinemia was detected in 56% of the patients with PGA and in 77% of those with MRA. The serum concentration of immunoglobulins was decreased substantially in more than two thirds of the patients with PGA. Proteinuria (greater than 250 mg/24 h) was observed in 78% of patients with PGA and in 93% of patients with MRA. Bence Jones proteinuria was noted in 75 and 77% of patients, respectively. Plasmacytic infiltration of the bone marrow was found in 90% of the patients with PGA. The mean survival time of the patients with PGA was 28 months and of those with MRA, 29 months from the time of diagnosis. PMID:1268776

  14. 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

  15. 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

  16. Thermodynamic assessments and inter-relationships between systems involving Al, Am, Ga, Pu, and U

    NASA Astrophysics Data System (ADS)

    Perron, A.; Turchi, P. E. A.; Landa, A.; Oudot, B.; Ravat, B.; Delaunay, F.

    2016-12-01

    A newly developed self-consistent CALPHAD thermodynamic database involving Al, Am, Ga, Pu, and U is presented. A first optimization of the slightly characterized Am-Al and completely unknown Am-Ga phase diagrams is proposed. To this end, phase diagram features as crystal structures, stoichiometric compounds, solubility limits, and melting temperatures have been studied along the U-Al → Pu-Al → Am-Al, and U-Ga → Pu-Ga → Am-Ga series, and the thermodynamic assessments involving Al and Ga alloying are compared. In addition, two distinct optimizations of the Pu-Al phase diagram are proposed to account for the low temperature and Pu-rich region controversy. The previously assessed thermodynamics of the other binary systems (Am-Pu, Am-U, Pu-U, and Al-Ga) is also included in the database and is briefly described in the present work. Finally, predictions on phase stability of ternary and quaternary systems of interest are reported to check the consistency of the database.

  17. Thermodynamic assessments and inter-relationships between systems involving Al, Am, Ga, Pu, and U

    SciTech Connect

    Perron, A.; Turchi, P. E. A.; Landa, A.; Oudot, B.; Ravat, B.; Delaunay, F.

    2016-12-01

    We present a newly developed self-consistent CALPHAD thermodynamic database involving Al, Am, Ga, Pu, and U. A first optimization of the slightly characterized Am-Al and completely unknown Am-Ga phase diagrams is proposed. To this end, phase diagram features as crystal structures, stoichiometric compounds, solubility limits, and melting temperatures have been studied along the U-Al → Pu-Al → Am-Al, and U-Ga → Pu-Ga → Am-Ga series, and the thermodynamic assessments involving Al and Ga alloying are compared. In addition, two distinct optimizations of the Pu-Al phase diagram are proposed to account for the low temperature and Pu-rich region controversy. We included the previously assessed thermodynamics of the other binary systems (Am-Pu, Am-U, Pu-U, and Al-Ga) in the database and is briefly described in the present work. In conclusion, predictions on phase stability of ternary and quaternary systems of interest are reported to check the consistency of the database.

  18. Thermodynamic assessments and inter-relationships between systems involving Al, Am, Ga, Pu, and U

    DOE PAGES

    Perron, A.; Turchi, P. E. A.; Landa, A.; ...

    2016-12-01

    We present a newly developed self-consistent CALPHAD thermodynamic database involving Al, Am, Ga, Pu, and U. A first optimization of the slightly characterized Am-Al and completely unknown Am-Ga phase diagrams is proposed. To this end, phase diagram features as crystal structures, stoichiometric compounds, solubility limits, and melting temperatures have been studied along the U-Al → Pu-Al → Am-Al, and U-Ga → Pu-Ga → Am-Ga series, and the thermodynamic assessments involving Al and Ga alloying are compared. In addition, two distinct optimizations of the Pu-Al phase diagram are proposed to account for the low temperature and Pu-rich region controversy. We includedmore » the previously assessed thermodynamics of the other binary systems (Am-Pu, Am-U, Pu-U, and Al-Ga) in the database and is briefly described in the present work. In conclusion, predictions on phase stability of ternary and quaternary systems of interest are reported to check the consistency of the database.« less

  19. 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.

  20. Vacuum system for the LBL Advanced Light Source (ALS)

    SciTech Connect

    Kennedy, K.; Henderson, T.; Meneghetti, J. )

    1989-03-01

    A 1.5 to 1.9 GeV synchrotron light source is being built at LBL. The vacuum system is designed to permit most synchrotron photons to escape the electron channel and be absorbed in an antechamber. The gas generated by the photons hitting the absorbers in the antechambers will be pumped by titanium sublimation pumps located directly under the absorbers. The electron channel and the antechamber are connected by a 10-mm-high slot that offers good electrodynamic isolation of the two chambers of frequencies affecting the store electron orbit. 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. Machining both the inside and outside of the vacuum chamber permits the use of complex and accurate surfaces. The use of thick plates allows flanges to be machined directly into the wall of each chamber, thus avoiding much welding. 1 ref., 3 figs.

  1. Early accretion of protoplanets inferred from a reduced inner solar system (26)Al inventory.

    PubMed

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

    2015-06-15

    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 (26)Al ((26)Al→(26)Mg; 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 (26)Al abundance [((26)Al/(27)Al)0] exists only for the oldest known solids, calcium aluminum-rich inclusions (CAIs) - the so-called canonical value. We have determined the (26)Al/(27)Al 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 ((26)Al/(27)Al)0 of [Formula: see text] 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 (54)Cr/(52)Cr ratios, most inner solar system materials likely accreted from material containing a similar (26)Al/(27)Al ratio as the APB precursor at the time of CAI formation. To satisfy the abundant evidence for widespread planetesimal differentiation, the subcanonical (26)Al budget requires that differentiated planetesimals, and hence protoplanets, accreted rapidly within 0.25 ± 0.15 Ma of the formation of canonical CAIs.

  2. 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.

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

    PubMed

    Tsuchiya, Jun; Tsuchiya, Taku

    2008-12-09

    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.

  4. Amyloid A amyloidosis secondary to hyper IgD syndrome and response to IL-1 blockage therapy.

    PubMed

    Kallianidis, A F; Ray, A; Goudkade, D; de Fijter, J W

    2016-01-01

    A 62-year-old woman with a history of genetically confirmed hyperimmunoglobulinaemia D and periodic fever syndrome (HIDS) was admitted because of chronic diarrhoea. During admission she developed a rapidly progressive nephrotic syndrome. Reactive amyloid A (AA) amyloidosis was confirmed after colonic and renal biopsy which showed deposition of amyloid. After initial treatment with high-dosed corticosteroids, therapy was switched to anakinra, an IL-1 receptor antagonist, but her symptoms persisted. After cessation of anakinra, a marked exacerbation of the intestinal symptoms was noted. Nine months after the initial diagnosis of reactive amyloidosis without any amelioration of the symptoms and a decreasing quality of life, our patient declined further treatment and died soon after. This case demonstrates that AA amyloidosis does occur in patients with HIDS and can present with intestinal symptoms and proteinuria. Once amyloidosis is diagnosed the goal of treatment is to prevent further complications. In this case report we give an overview of previous cases with amyloidosis complicating HIDS with the treatments received and propose a step-up treatment plan for future cases.

  5. 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.

  6. Familial amyloidotic polyneuropathy: current and emerging treatment options for transthyretin-mediated amyloidosis.

    PubMed

    Hund, Ernst

    2012-01-01

    Transthyretin familial amyloid polyneuropathy (TTR-FAP) is a fatal clinical disorder characterized by extracellular deposition of abnormal fibrils derived from misfolded, normally soluble transthyretin (TTR) molecules. The disease is most commonly caused by a point mutation within the TTR gene inherited in an autosomal dominant fashion. Over 100 of such mutations have been identified, leading to destabilization of the physiological TTR tetramer. As a result, many monomers originate with a tendency for spontaneous conformational changes and self-aggregation. The main clinical feature of TTR-FAP is progressive sensorimotor and autonomic neuropathy. In the beginning, this polyneuropathy predominantly involves small unmyelinated nerve fibers with the result of dissociated sensory loss disproportionately affecting sensation of pain and temperature. Autonomic neuropathy typically accompanies sensory deficits early in the disease course. The symptoms include orthostatic hypotension, constipation alternating with diarrhea, erectile dysfunction, anhydrosis, and urinary retention or incontinence. Later, involvement of motor fibers causes rapidly progressive weakness and gait disturbances. In addition to the peripheral nervous system, the heart and the gut are frequently affected. Onset of symptoms is bimodal, with one peak at age 33 years (early onset) and another distinct peak in the sixth decade of life (late onset). The course of TTR-FAP is uniformly progressive and fatal. Death occurs an average of 10.8 years after the onset of symptoms in Portuguese patients, and 7.3 years in late-onset Japanese patients. Common causes include cachexia, cardiac failure, arrhythmia, and secondary infections. Liver transplantation is the standard therapy for patients who are in a clinical condition good enough to tolerate this intervention because it stops progression of neuropathy by removing the main source of mutant TTR. Recently, orally administered tafamidis meglumine has been

  7. Familial amyloidotic polyneuropathy: current and emerging treatment options for transthyretin-mediated amyloidosis

    PubMed Central

    Hund, Ernst

    2012-01-01

    Transthyretin familial amyloid polyneuropathy (TTR-FAP) is a fatal clinical disorder characterized by extracellular deposition of abnormal fibrils derived from misfolded, normally soluble transthyretin (TTR) molecules. The disease is most commonly caused by a point mutation within the TTR gene inherited in an autosomal dominant fashion. Over 100 of such mutations have been identified, leading to destabilization of the physiological TTR tetramer. As a result, many monomers originate with a tendency for spontaneous conformational changes and self-aggregation. The main clinical feature of TTR-FAP is progressive sensorimotor and autonomic neuropathy. In the beginning, this polyneuropathy predominantly involves small unmyelinated nerve fibers with the result of dissociated sensory loss disproportionately affecting sensation of pain and temperature. Autonomic neuropathy typically accompanies sensory deficits early in the disease course. The symptoms include orthostatic hypotension, constipation alternating with diarrhea, erectile dysfunction, anhydrosis, and urinary retention or incontinence. Later, involvement of motor fibers causes rapidly progressive weakness and gait disturbances. In addition to the peripheral nervous system, the heart and the gut are frequently affected. Onset of symptoms is bimodal, with one peak at age 33 years (early onset) and another distinct peak in the sixth decade of life (late onset). The course of TTR-FAP is uniformly progressive and fatal. Death occurs an average of 10.8 years after the onset of symptoms in Portuguese patients, and 7.3 years in late-onset Japanese patients. Common causes include cachexia, cardiac failure, arrhythmia, and secondary infections. Liver transplantation is the standard therapy for patients who are in a clinical condition good enough to tolerate this intervention because it stops progression of neuropathy by removing the main source of mutant TTR. Recently, orally administered tafamidis meglumine has been

  8. The Al-Rich Part of the System CaO-Al 2O 3-MgO . Part I. Phase Relationships

    NASA Astrophysics Data System (ADS)

    Göbbels, M.; Woermann, E.; Jung, J.

    1995-12-01

    In the Al-rich part of the ternary system CaO-Al 2O 3MgO two new ternary phases Ca 2Mg 2Al 28O 46 (CAM-I) and CaMg 2Al 16O 27 (CAM-II) with limited solid solution ranges were found. Due to the fact that the compositions of the Mg-rich end members of these solid solutions lie on the join between hibonite (CaAl 12O 19) and spinel (MgAl 2O 4), the model of the crystal structures of these phases can be constructed by a suitable combination of hibonite and spinel units. Both phases, CAM-I and CAM-II, exhibit solid solution ranges described by a substitution mechanism also found in the binary spinel phase, MgAl 2O 4: 3 Mg 2+ = 2 Al 3+ + □. Thus the ternary phases can be expressed by the chemical formulas. Ca 2Mg 2-3 xAl 28+2 x□ xO 46 for CAM-I with 0 ≤ x ≤ 0.30 and CaMg 2-3 yAl 16+2 y□ yO 27 for CAM-II with 0 ≤ y ≤ 0.2.

  9. 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.

  10. 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.

  11. 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.

  12. 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

  13. Sensory network dysfunction, behavioral impairments, and their reversibility in an Alzheimer’s β-amyloidosis mouse model

    PubMed Central

    Wesson, Daniel W.; Borkowski, Anne H.; Landreth, Gary E.; Nixon, Ralph A.; Levy, Efrat; Wilson, Donald A.

    2012-01-01

    The unique vulnerability of the olfactory system to Alzheimer’s disease (AD) provides a quintessential translational tool for understanding mechanisms of synaptic dysfunction and pathological progression in the disease. Using the Tg2576 mouse model of β-amyloidosis, we show aberrant, hyperactive olfactory network activity begins early in life, prior to detectable behavioral impairments or comparable hippocampal dysfunction and at a time when Aβ deposition is restricted to the olfactory bulb (OB). Hyperactive odor-evoked activity in the piriform cortex (PCX) and increased OB-PCX functional connectivity emerged at a time coinciding with olfactory behavior impairments. This hyperactive activity persisted until later-life when the network converted to a hyporesponsive state. This conversion was Aβ-dependent, as liver-x-receptor agonist treatment to promote Aβ degradation, rescued the hyporesponsive state and olfactory behavior. These data lend evidence to a novel working model of olfactory dysfunction in AD and, complimentary to other recent works, suggest that disease-relevant network dysfunction is highly dynamic and region specific, yet with lasting effects on cognition and behavior. PMID:22049439

  14. Thermal explosion in Al-Ni system: influence of mechanical activation.

    PubMed

    White, Jeremiah D E; Reeves, Robert V; Son, Steven F; Mukasyan, Alexander S

    2009-12-03

    The influence of short-term (5-15 min) highly energetic ball milling on the ignition characteristics of a gasless heterogeneous Ni-Al reactive system has been investigated. By using Al-Ni clad particles (30-40 microm diameter Al spheres coated by a 3-3.5 microm layer of Ni, that corresponds to a 1:1 Ni/Al atomic ratio), it was shown that such mechanical treatment leads to a significant decrease in the self-ignition temperature of the system. For example, after 15 min of ball milling, the ignition temperature appears to be approximately 600 K, well below the eutectic (913 K) in the considered binary system, which is the ignition temperature for the initial clad particles. Thus, it was demonstrated that the thermal explosion process for mechanically treated reactive media can be solely defined by solid-state reactions. Additionally, thermal analysis measurements revealed that mechanical activation results in a substantial decrease in the effective activation energy (from 84 to 28 kcal/mol) of interaction between Al and Ni. This effect, that is, mechanical activation of chemical reaction, is connected to a substantial increase of contact area between reactive particles and fresh interphase boundaries formed in an inert atmosphere during ball milling. It is also important that by varying the time of mechanical activation one can precisely control the ignition temperature in high-density energetic heterogeneous systems.

  15. 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.

  16. A multicenter report of biologic agents for the treatment of secondary amyloidosis in Turkish rheumatoid arthritis and ankylosing spondylitis patients.

    PubMed

    Pamuk, Ömer Nuri; Kalyoncu, Umut; Aksu, Kenan; Omma, Ahmet; Pehlivan, Yavuz; Çağatay, Yonca; Küçükşahin, Orhan; Dönmez, Salim; Çetin, Gözde Yıldırım; Mercan, Rıdvan; Bayındır, Özün; Çefle, Ayşe; Yıldız, Fatih; Balkarlı, Ayşe; Kılıç, Levent; Çakır, Necati; Kısacık, Bünyamin; Öksüz, Mustafa Ferhat; Çobankara, Veli; Onat, Ahmet Mesut; Sayarlıoğlu, Mehmet; Öztürk, Mehmet Akif; Pamuk, Gülsüm Emel; Akkoç, Nurullah

    2016-07-01

    In this multicenter, retrospective study, we evaluated the efficacy and safety of biologic therapies, including anti-TNFs, in secondary (AA) amyloidosis patients with ankylosing spondylitis (AS) and rheumatoid arthritis (RA). In addition, the frequency of secondary amyloidosis in RA and AS patients in a single center was estimated. Fifty-one AS (39M, 12F, mean age: 46.7) and 30 RA patients (11M, 19F, mean age: 51.7) with AA amyloidosis from 16 different centers in Turkey were included. Clinical and demographical features of patients were obtained from medical charts. A composite response index (CRI) to biologic therapy-based on creatinine level, proteinuria and disease activity-was used to evaluate the efficacy of treatment. The mean annual incidence of AA amyloidosis in RA and AS patients was 0.23 and 0.42/1000 patients/year, respectively. The point prevalence in RA and AS groups was 4.59 and 7.58/1000, respectively. In RA group with AA amyloidosis, effective response was obtained in 52.2 % of patients according to CRI. RA patients with RF positivity and more initial disease activity tended to have higher response rates to therapy (p values, 0.069 and 0.056). After biologic therapy (median 17 months), two RA patients died and two developed tuberculosis. In AS group, 45.7 % of patients fulfilled the criteria of good response according to CRI. AS patients with higher CRP levels at the time of AA diagnosis and at the beginning of anti-TNF therapy had higher response rates (p values, 0.011 and 0.017). During follow-up after anti-TNF therapy (median 38 months), one patient died and tuberculosis developed in two patients. Biologic therapy seems to be effective in at least half of RA and AS patients with AA amyloidosis. Tuberculosis was the most important safety concern.

  17. 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)

  18. Structural and electronic properties of the graphene/Al/Ni(111) intercalation system

    NASA Astrophysics Data System (ADS)

    Voloshina, E. N.; Generalov, A.; Weser, M.; Böttcher, S.; Horn, K.; Dedkov, Yu S.

    2011-11-01

    Decoupling of the graphene layer from the ferromagnetic substrate via intercalation of sp metal has recently been proposed as an effective way to realize a single-layer graphene-based spin-filter. Here, the structural and electronic properties of the prototype system, graphene/Al/Ni(111), are investigated via a combination of electron diffraction and spectroscopic methods. These studies are accompanied by state-of-the-art electronic structure calculations. The properties of this prospective Al-intercalation-like system and its possible implementations in future graphene-based devices are discussed.

  19. Phase equilibria and structural investigations in the system Al-Fe-Si.

    PubMed

    Marker, Martin C J; Skolyszewska-Kühberger, Barbara; Effenberger, Herta S; Schmetterer, Clemens; Richter, Klaus W

    2011-12-01

    The Al-Fe-Si system was studied for an isothermal section at 800 °C in the Al-rich part and at 900 °C in the Fe-rich part, and for half a dozen vertical sections at 27, 35, 40, 50 and 60 at.% Fe and 5 at.% Al. Optical microscopy and powder X-ray diffraction (XRD) was used for initial sample characterization, and Electron Probe Microanalysis (EPMA) and Scanning Electron Microscopy (SEM) of the annealed samples was used to determine the exact phase compositions. Thermal reactions were studied by Differential Thermal Analysis (DTA). Our experimental results are generally in good agreement with the most recent phase diagram versions of the system Al-Fe-Si. A new ternary high-temperature phase τ12 (cF96, NiTi2-type) with the composition Al48Fe36Si16 was discovered and was structurally characterized by means of single-crystal and powder XRD. The variation of the lattice parameters of the triclinic phase τ1 with the composition Al2+x Fe3Si3-x (-0.3 < x < 1.3) was studied in detail. For the binary phase FeSi2 only small solubility of Al was found in the low-temperature modification LT-FeSi2 (ζβ ) but significant solubility in the high-temperature modification HT-FeSi2 (ζα ) (8.5 at.% Al). It was found that the high-temperature modification of FeSi2 is stabilized down to much lower temperature in the ternary, confirming earlier literature suggestions on this issue. DTA results in four selected vertical sections were compared with calculated sections based on a recent CALPHAD assessment. The deviations of liquidus values are significant suggesting the need for improvement of the thermodynamic models.

  20. Intense Uptake in Amyloidosis of the Seminal Vesicles on 68Ga-PSMA PET Mimicking Locally Advanced Prostate Cancer.

    PubMed

    Stephens, Maximilian; Kim, David Insoo; Shepherd, Benjamin; Gustafson, Sonja; Thomas, Paul

    2017-02-01

    We report a case of benign senile seminal vesicle amyloidosis demonstrating intense Ga-prostate-specific membrane antigen (PSMA) uptake on PET/CT. A 68-year-old man underwent staging PSMA PET/CT and MRI for biopsy-proven prostate adenocarcinoma. There was an intense focus of Ga-PSMA uptake in the primary malignancy, as well as symmetrical intense uptake in the seminal vesicles bilaterally that was reported as multifocal disease with local invasion. Final histology after radical prostatectomy showed amyloidosis of the seminal vesicles without any evidence of prostate cancer. Care should be taken in the interpretation of seminal vesicle PSMA uptake to avoid overstaging.

  1. Sequential cyclophosphamide-bortezomib-dexamethasone unmasks the harmful cardiac effect of dexamethasone in primary light-chain cardiac amyloidosis.

    PubMed

    Le Bras, Fabien; Molinier-Frenkel, Valerie; Guellich, Aziz; Dupuis, Jehan; Belhadj, Karim; Guendouz, Soulef; Ayad, Karima; Colombat, Magali; Benhaiem, Nicole; Tissot, Claire Marie; Hulin, Anne; Jaccard, Arnaud; Damy, Thibaud

    2017-03-20

    Chemotherapy combining cyclophosphamide, bortezomib and dexamethasone is widely used in light-chain amyloidosis. The benefit is limited in patients with cardiac amyloidosis mainly because of adverse cardiac events. Retrospective analysis of our cohort showed that 39 patients died with 42% during the first month. A new escalation-sequential regimen was set to improve the outcomes. Nine newly-diagnosed patients were prospectively treated with close monitoring of serum N-terminal pro-brain natriuretic peptide, troponin-T and free light chains. The results show that corticoids may destabilise the heart through fluid retention. Thus, a sequential protocol may be a promising approach to treat these patients.

  2. AlGaInN laser diode technology and systems for defence and security applications

    NASA Astrophysics Data System (ADS)

    Najda, Stephen P.; Perlin, Piotr; Suski, Tadek; Marona, Lujca; Boćkowski, Mike; Leszczyński, Mike; Wisniewski, Przemek; Czernecki, Robert; Kucharski, Robert; Targowski, Grzegorz; Watson, Scott; Kelly, Antony E.

    2015-10-01

    AlGaInN laser diodes is an emerging technology for defence and security applications such as underwater communications and sensing, atomic clocks and quantum information. The AlGaInN material system allows for laser diodes to be fabricated over a very wide range of wavelengths from u.v., ~380nm, to the visible ~530nm, by tuning the indium content of the laser GaInN quantum well. Thus AlGaInN laser diode technology is a key enabler for the development of new disruptive system level applications in displays, telecom, defence and other industries. Ridge waveguide laser diodes are fabricated to achieve single mode operation with optical powers up to 100mW with the 400-440nm wavelength range with high reliability. Visible free-space and underwater communication at frequencies up to 2.5GHz is reported using a directly modulated 422nm GaN laser diode. Low defectivity and highly uniform GaN substrates allow arrays and bars to be fabricated. High power operation operation of AlGaInN laser bars with up to 20 emitters have been demonstrated at optical powers up to 4W in a CS package with common contact configuration. An alternative package configuration for AlGaInN laser arrays allows for each individual laser to be individually addressable allowing complex free-space or optical fibre system integration with a very small form-factor.

  3. 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

  4. Application of confocal laser scanning microscopy for the diagnosis of amyloidosis.

    PubMed

    Castellani, Chiara; Fedrigo, Marny; Frigo, Anna Chiara; Barbera, Mila Della; Thiene, Gaetano; Valente, Marialuisa; Adami, Fausto; Angelini, Annalisa

    2017-02-20

    We analysed specificity and sensitivity of confocal laser microscopy (CLSM) on tissue sections for a diagnosis of amyloidosis, in an attempt to reduce technical errors and better standardise pathological diagnosis. We first set up a protocol for the use of CLSM on this type of specimen, using a group of 20 amyloid negative and 20 positive samples. Of all specimens, 2, 4 and 8-μm sections were cut. Sections were stained with Congo red (CR) and thioflavin-T (ThT) and observed by cross-polarised light microscopy (CR-PL), epifluorescence microscopy (CRF-epiFM and ThT-epiFM) and CLSM (CRF-CLSM and ThT-CLSM). To validate the method in a diagnostic setting, we examined tissue samples from 116 consecutive patients with clinical suspicion of amyloidosis, selected from the period 2005 to 2014 from the database of the Pathology Unit of the University of Padua. The results were compared with those of transmission electron microscopy (TEM), which we consider as reference. We found that with CRF-CLSM, the false negative rate was reduced from 17 to 5%, while the sensitivity of detection increased to 12%. The results were in complete agreement with those of TEM ThT-CLSM; both sensitivity and specificity were 100%. Finally, ThT-CLSM results did not vary with section thickness, and small amounts of amyloid could even be detected in 2-μm sections. In conclusion, we found ThT-CLSM to be more sensitive as a screening method for amyloidosis than CR and ThT epifluorescence optical imaging. The method was easier to standardise, provided images with better resolution and resulted in more consistent pathologist diagnoses.

  5. Amyloidosis and the serum amyloid A protein response to muramyl dipeptide analogs and different mycobacterial species.

    PubMed Central

    McAdam, K P; Foss, N T; Garcia, C; DeLellis, R; Chedid, L; Rees, R J; Wolff, S M

    1983-01-01

    Serum amyloid A protein (SAA) elevation accompanies induction of secondary amyloidosis in mice given Mycobacterium butyricum in Freund adjuvant. The synthesis of SAA by cultured hepatocytes is induced by a macrophage-derived mediator, which has been identified as interleukin 1. In these studies, SAA synthesis has been used as an index of macrophage activation to examine the in vivo response of mice to challenge with seven different mycobacteria and with synthetic analogs of the immunoadjuvant N-acetylmuramyl-L-alanyl-D-isoglutamine [MDP(L-D)]. SAA synthesis was stimulated by administration (by the intraperitoneal route) of the mycobacteria dissolved in saline, with Mycobacterium vaccae being the most active and Mycobacterium leprae being the least stimulatory. MDP(L-D), which is the minimal structure (molecular weight, 492) able to substitute for mycobacteria in Freund adjuvant, stimulated SAA synthesis, whereas the MDP(D-D) isomer was inactive. The butyl ester of MDP, which induces no detectable pyrogenicity but retains adjuvanticity, required a 100-fold greater dosage than MDP(L-D) in stimulating SAA synthesis. Amyloidosis was detected histologically only when active SAA inducers MDP(L-D), M. vaccae, and M. butyricum, were administered in incomplete Freund adjuvant, with amyloid-enhancing factor. These studies demonstrated that SAA elevation was a sensitive in vivo marker of the capacity of antigens to stimulate macrophages to produce interleukin 1. A point of considerable relevance to the human use of MDP was the observation that repeated injections of the adjuvant MDP in saline did not induce secondary amyloidosis. PMID:6601620

  6. 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.

  7. Pathological study on amyloidosis in Cygnus olor (mute swan) and other waterfowl.

    PubMed

    Tanaka, Shinsuke; Dan, Cui; Kawano, Hiroo; Omoto, Masatoshi; Ishihara, Tokuhiro

    2008-06-01

    Between 2004 and 2007, we examined a total of 70 waterfowl. Forty of 51 (78.4%) mute swans (Cygnus olor) had amyloidosis. Amyloid deposits were detected in the spleen of 39 of 49 birds (79.6%), liver of 37 of 47 birds (78.7%), intestine of 38 of 50 birds (76.0%), pancreas of 30 of 42 birds (71.4%), kidney of 32 of 47 birds (68.1%), thyroid gland of 20 of 30 birds (66.7%), heart of 26 of 49 birds (53.1%), and lung of 5 of 45 birds (11.1%). In some birds, there was a globular pattern of amyloid deposition or infiltration of foreign-body giant cells around amyloid nodules in the spleen. Immunostaining with anti-AA antibody and Western blotting revealed that all were cases of AA amyloidosis. In sections treated with potassium permanganate, which removes Congo red stain, the green refringence under polarized light had mostly vanished. However, staining was not completely eliminated in some areas. Electron microscopy confirmed that the star-shaped amyloid fibrils were 10 nm in diameter and lacked branching. We also demonstrated amyloid bundles and star-shaped amyloid fibrils. A high percentage (96.3%) of mute swans had an inflammatory condition known as bumblefoot. Swans are useful model for studies of animals that have high amounts of amyloid. This research may help in the elucidation of the mechanism of amyloidogenesis in humans, and more research regarding amyloidosis in birds that are consumed as food is necessary.

  8. 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

  9. Incidence of hereditary amyloidosis and autoinflammatory diseases in Sweden: endemic and imported diseases

    PubMed Central

    2013-01-01

    Background Amyloidoses are a heterogeneous group of progressive diseases caused by tissue deposition of misfolded proteins. According to the International Classification of Diseases, hereditary amyloidosis is divided into neuropathic and non-neuropathic forms. In Sweden, neuropathic heredofamilial amyloidosis has been identified as familial amyloidotic polyneuropathy (FAP), a fatal disease that is treated by liver transplantation. The non-neuropathic form includes familial autoinflammatory diseases. As no incidence data on these hereditary diseases are available and as even diagnostic data on non-neuropathic forms are lacking we determined the incidence of these diseases and characterized non-neuropathic conditions. Methods Patients were identified using data from the Swedish Hospital Discharge Register and from the Outpatient Register for 2001 through 2008. All patients discharged with hereditary amyloidosis diagnoses were included and standardized incidence rates were calculated. Results Non-neuropathic disease was diagnosed in 210 patients, with an incidence of 2.83 per million. FAP was diagnosed in 221 patients, with an incidence of 2.02 per million. Two northern provinces that are home to 5% of the Swedish population accounted for 77% of FAP cases; the incidence in one of them, West Bothnia, was 100 times that in the rest of Sweden. Approximately 98% of non-neuropathic disease patients were immigrants, most of whom were from the Eastern Mediterranean area. Young Syrian descendants had the highest incidence rate, which was over 500-fold higher than that in individuals with Swedish parents. Even the early onset of these conditions identified them as familial autoinflammatory diseases. Conclusions FAP cases were highly concentrated in the two northernmost provinces. Non-neuropathic familial autoinflammatory diseases were of early-onset and immigrant origin most likely related to periodic fever syndromes. Paradoxically, FAP has remained endemic, in spite of

  10. Identification of proteins associated with amyloidosis by polarity index method.

    PubMed

    Polanco, Carlos; Samaniego, José Lino; Uversky, Vladimir N; Castañón-González, Jorge Alberto; Buhse, Thomas; Leopold-Sordo, Marili; Madero-Arteaga, Alejandro; Morales-Reyes, Alicia; Tavera-Sierra, Lourdes; González-Bernal, Jesus A; Arias-Estrada, Miguel

    2015-01-01

    There is a natural protein form, insoluble and resistant to proteolysis, adopted by many proteins independently of their amino acid sequences via specific misfolding-aggregation process. This dynamic process occurs in parallel with or as an alternative to physiologic folding, generating toxic protein aggregates that are deposited and accumulated in various organs and tissues. These proteinaceous deposits typically represent bundles of β-sheet-enriched fibrillar species known as the amyloid fibrils that are responsible for serious pathological conditions, including but not limited to neurodegenerative diseases, grouped under the term amyloidoses. The proteins that might adopt this fibrillar conformation are some globular proteins and natively unfolded (or intrinsically disordered) proteins. Our work shows that intrinsically disordered and intrinsically ordered proteins can be reliably identified, discriminated, and differentiated by analyzing their polarity profiles generated using a computational tool known as the polarity index method (Polanco & Samaniego, 2009; Polanco et al., 2012; 2013; 2013a; 2014; 2014a; 2014b; 2014c; 2014d). We also show that proteins expressed in neurons can be differentiated from proteins in these two groups based on their polarity profiles, and also that this computational tool can be used to identify proteins associated with amyloidoses. The efficiency of the proposed method is high (i.e. 70%) as evidenced by the analysis of peptides and proteins in the APD2 database (2012), AVPpred database (2013), and CPPsite database (2013), the set of selective antibacterial peptides from del Rio et al. (2001), the sets of natively unfolded and natively folded proteins from Oldfield et al. (2005), the set of human revised proteins expressed in neurons, and non-human revised proteins expressed in neurons, from the Uniprot database (2014), and also the set of amyloidogenic proteins from the AmyPDB database (2014).

  11. The Imaging Diagnosis of Less Advanced Cases of Cardiac Amyloidosis: The Relative Apical Sparing Pattern

    PubMed Central

    Ono, Koya; Ishimaru, Go; Hayashi, Miho; Bae, Yuan; Ito, Takashi; Izumo, Toshiyuki; Murata, Ken

    2017-01-01

    An early diagnosis is important for improving the prognosis of cardiac amyloidosis (CA). We herein describe the utility of two-dimensional speckle tracking echocardiography (2-D STE) in diagnosing CA at a less advanced stage. A 63-year-old woman with exertional dyspnea was suspected of having CA based on her echocardiographic and electrocardiographic findings. A myocardial biopsy was negative for amyloid deposits, while the relative apical sparing pattern was detected on 2-D STE, which was highly suggestive of CA. Chemotherapy was initiated as a treatment for CA, and the patient's symptoms were immediately relieved. Thereafter, amyloid deposits were detected in a skin biopsy specimen. PMID:28154276

  12. Primary localized tracheobronchial amyloidosis presenting with massive hemoptysis: a case report and literature review.

    PubMed

    Zhang, Li-Qin; Zhao, Yi-Chao; Wang, Xiao-Wei; Yang, Jian; Lu, Zhi-Wei; Cheng, Yu-Sheng

    2017-01-01

    Primary localized tracheobronchial amyloidosis (TBA) is a rare respiratory tract dysfunction, which is a heterogeneous group of diseases involving abnormal extracellular deposition of amyloid and autologous fibrillar protein material in β-pleated sheets. A 64-year-old man was referred to our hospital because of hemoptysis. Physical examination showed decreased breath sounds in the right lung on auscultation. Chest computed tomography scan displayed multiple nodules with varied size in main bronchia around bilateral hilus of the lung. After admission, bronchoscopy was performed for this patient, and roughness of mucosa in trachea and multiple nodules in respiratory tract were observed. Through further tissue biopsy, the diagnosis of primary TBA was confirmed.

  13. Wettability and phase formation in the TiC{sub x}/Al system

    SciTech Connect

    Frumin, N.; Frage, N.; Polak, M.; Dariel, M.P.

    1997-10-15

    The introduction of reinforcements such as carbide or oxide ceramics into a metallic matrix improves high temperature strength, creep resistance and low temperature toughness. Modification of the ceramic phase can also be used in order to improve wetting in a metal/ceramic system. The wettability of ceramic surfaces by metallic melts is strongly dependent on the stoichiometry of the ceramic compound (oxide or carbide). Titanium carbide has a wide range of stability (0.55 < C/Ti {le} 1), and its chemical, physical and mechanical properties largely depend on the composition. For instance, chemical inertness and hardness increase with increasing C/Ti ratio. The use of TiC as a reinforcing phase in aluminum alloys or in TiC/Al cermets has not been exploited fully. Titanium carbide exhibits adequate wetting by Al{sub (L)} due to chemical interactions at high temperatures that may also lead to the formation of the Al{sub 4}C{sub 3} phase. The presence of this phase at the interface is undesirable because it is weak and brittle. Moreover, Al{sub 4}C{sub 3} decomposes in the presence of water to form Al{sub 2}O{sub 3} and methane gas. The present work was aimed at finding the proper conditions and the Ti/C ratio in non-stoichiometric TiC{sub x} that would improve wetting and prevent the formation of Al{sub 4}C{sub 3}.

  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. Critical Assessment and Thermodynamic Modeling of the Al-Fe-O System

    NASA Astrophysics Data System (ADS)

    Shishin, Denis; Prostakova, Viktoria; Jak, Evgueni; Decterov, Sergei A.

    2016-02-01

    A complete literature review, critical evaluation, and thermodynamic modeling of the phase diagrams and thermodynamic properties of phases in the Al-Fe-O system at 1 atm total pressure are presented. Optimized model equations for the thermodynamic properties of all phases are obtained, which reproduce all available thermodynamic and phase-equilibrium data within experimental error limits from 298.15 K (25 °C) to above the liquidus temperatures at all compositions and oxygen partial pressures from metal saturation to 1 atm. The complex phase relationships in the system have been elucidated, and discrepancies among the data have been resolved. The database of the model parameters can be used along with software for Gibbs-energy minimization in order to calculate all thermodynamic properties and any type of phase diagram section. The modified quasichemical model was used for the liquid oxide phase. A sublattice model, based upon the Compound Energy Formalism, was developed for spinel, which expands from magnetite, Fe3O4, to hercynite, FeAl2O4. The distribution of cations between octahedral and tetrahedral sites and oxygen nonstoichiometry in spinel are taken into account. The model for metallic liquid assumes random mixing of associates: Fe, Al, O, AlO, and Al2O. It describes well the minimum that is observed on the solubility of oxygen in liquid iron as a function of the Al content. The solid solution between hematite and corundum exhibiting a miscibility gap, as well as a small solubility of Al2O3 in wüstite are quantitatively described by a simple Bragg-Williams model.

  16. Synthesis of MAX Phases in the Zr-Ti-Al-C System.

    PubMed

    Tunca, Bensu; Lapauw, Thomas; Karakulina, Olesia M; Batuk, Maria; Cabioc'h, Thierry; Hadermann, Joke; Delville, Rémi; Lambrinou, Konstantina; Vleugels, Jozef

    2017-03-20

    This study reports on the synthesis and characterization of MAX phases in the (Zr,Ti)n+1AlCn system. The MAX phases were synthesized by reactive hot pressing and pressureless sintering in the 1350-1700 °C temperature range. The produced ceramics contained large fractions of 211 and 312 (n = 1, 2) MAX phases, while strong evidence of a 413 (n = 3) stacking was found. Moreover, (Zr,Ti)C, ZrAl2, ZrAl3, and Zr2Al3 were present as secondary phases. In general, the lattice parameters of the hexagonal 211 and 312 phases followed Vegard's law over the complete Zr-Ti solid solution range, but the 312 phase showed a non-negligible deviation from Vegard's law around the (Zr0.33,Ti0.67)3Al1.2C1.6 stoichiometry. High-resolution scanning transmission electron microscopy combined with X-ray diffraction demonstrated ordering of the Zr and Ti atoms in the 312 phase, whereby Zr atoms occupied preferentially the central position in the close-packed M6X octahedral layers. The same ordering was also observed in 413 stackings present within the 312 phase. The decomposition of the secondary (Zr,Ti)C phase was attributed to the miscibility gap in the ZrC-TiC system.

  17. 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.

  18. New investigation of phase equilibria in the system Al-Cu-Si.

    PubMed

    Ponweiser, Norbert; Richter, Klaus W

    2012-01-25

    The phase equilibria and invariant reactions in the system Al-Cu-Si were investigated by a combination of optical microscopy, powder X-ray diffraction (XRD), differential thermal analysis (DTA) and electron probe micro analysis (EPMA). Isothermal phase equilibria were investigated within two isothermal sections. The isothermal section at 500 °C covers the whole ternary composition range and largely confirms the findings of previous phase diagram investigations. The isothermal section at 700 °C describes phase equilibria only in the complex Cu-rich part of the phase diagram. A new ternary compound τ was found in the region between (Al,Cu)-γ(1) and (Cu,Si)-γ and its solubility range was determined. The solubility of Al in κ-CuSi was found to be extremely high at 700 °C. In contrast, no ternary solubility in the β-phase of Cu-Al was found, although this phase is supposed to form a complete solid solution according to previous phase diagram assessments. Two isopleths, at 10 and 40 at.% Si, were investigated by means of DTA and a partial ternary reaction scheme (Scheil diagram) was constructed, based on the current work and the latest findings in the binary systems Al-Cu and Cu-Si. The current study shows that the high temperature equilibria in the Cu-rich corner are still poorly understood and additional studies in this area would be favorable.

  19. A Study of Phase Composition and Structure of Alloys of the Al - Mg - Si - Fe System

    NASA Astrophysics Data System (ADS)

    Mailybaeva, A. D.; Zolotorevskii, V. S.; Smagulov, D. U.; Islamkulov, K. M.

    2017-03-01

    The Thermo-Calc software is used to compute the phase transformations occurring during cooling of alloys. Polythermal and isothermal sections of the phase diagram of the Al - Mg - Si - Fe system are plotted. The phase composition and the structure of aluminum alloys in cast condition and after a heat treatment are studied experimentally.

  20. 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

  1. 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.

  2. Direct Evidence of Nanometric Invasionlike Grain Boundary Penetration in the Al/Ga System

    SciTech Connect

    Pereiro-Lopez, E.; Cloetens, P.; Ludwig, W.; Bellet, D.; Lemaignan, C.

    2005-11-18

    We report the first in situ results of deformation during grain boundary penetration in the Al/Ga system, obtained with a novel, nondestructive hard x-ray synchrotron projection microscopy technique. Focusing the beam to a state-of-the-art spot size of 90x90 nm{sup 2}, we demonstrate that penetration is accompanied by continuous relative separation of the Al grains of the same final amplitude as the final Ga layer thickness in the absence of external stress. The formation of nanometric intergranular liquid layers is originated by a crack propagation process and inherently implies the presence of weak stress levels.

  3. 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

  4. Anti-fibrillation propensity of a flavonoid baicalein against the fibrils of hen egg white lysozyme: potential therapeutics for lysozyme amyloidosis.

    PubMed

    Fazili, Naveed Ahmad; Bhat, Imtiyaz Ahmad; Bhat, Waseem Feeroze; Naeem, Aabgeena

    2016-10-01

    More than 20 human diseases involve the fibrillation of a specific protein/peptide which forms pathological deposits at various sites. Hereditary lysozyme amyloidosis is a systemic disorder which mostly affects liver, spleen and kidney. This conformational disorder is featured by lysozyme fibril formation. In vivo lysozyme fibrillation was simulated under in vitro conditions using a strong denaturant GdHCl at 3 M concentration. Sharp decline in the ANS fluorescence intensity compared to the partially unfolded states, almost 20-fold increase in ThT fluorescence intensity, increase in absorbance at 450 nm suggesting turbidity, negative ellipticity peak in the far-UVCD at 217 nm, red shift of 50 nm compared to the native state in Congo red assay and appearance of a network of long rope-like fibrils in transmission electron microscope (TEM) analysis suggested HEWL fibrillation. Anti-fibrillation potency of baicalein against the preformed fibrils of HEWL was investigated following ThT assay in which there was a dose-dependent decrease in ThT fluorescence intensity compared to the fibrillar state of HEWL with the maximum effect observed at 150-μM baicalein concentration, loss of negative ellipticity peak in the far-UVCD region, dip in the Rayleigh scattering intensity and absorbance at 350 and 450 nm, respectively, together with a reduction in the density of fibrillar structure in TEM imaging. Thus, it could be suggested that baicalein could prove to be a positive therapeutics for hereditary human lysozyme amyloidosis.

  5. 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.

  6. 75 FR 36689 - United States, et al. v. Election Systems & Software, Inc.; Public Comments and Response on...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-06-28

    ... Antitrust Division United States, et al. v. Election Systems & Software, Inc.; Public Comments and Response... United States, et al. v. Election Systems & Software Inc., Case No. 1:10-00380-JDB, which were filed in.... Election Systems and Software, Inc., Defendant. Case No.: 1:10-cv-00380 Judge: Bates, John D. Deck...

  7. Natural Phenolic Compounds as Therapeutic and Preventive Agents for Cerebral Amyloidosis.

    PubMed

    Yamada, Masahito; Ono, Kenjiro; Hamaguchi, Tsuyoshi; Noguchi-Shinohara, Moeko

    2015-01-01

    Epidemiological studies have suggested that diets rich in phenolic compounds may have preventive effects on the development of dementia or Alzheimer's disease (AD). We investigated the effects of natural phenolic compounds, such as myricetin (Myr), rosmarinic acid (RA), ferulic acid (FA), curcumin (Cur) and nordihydroguaiaretic acid (NDGA) on the aggregation of amyloid β-protein (Aβ), using in vitro and in vivo models of cerebral Aβ amyloidosis. The in vitro studies revealed that these phenolic compounds efficiently inhibit oligomerization as well as fibril formation of Aβ through differential binding, whilst reducing Aβ oligomer-induced synaptic and neuronal toxicity. Furthermore, a transgenic mouse model fed orally with such phenolic compounds showed significant reduction of soluble Aβ oligomers as well as of insoluble Aβ deposition in the brain. These data, together with an updated review of the literature, indicate that natural phenolic compounds have anti-amyloidogenic effects on Aβ in addition to well-known anti-oxidative and anti-inflammatory effects, hence suggesting their potential as therapeutic and/or preventive agents for cerebral Aβ amyloidosis, including AD and cerebral amyloid angiopathy (CAA). Well-designed clinical trials or preventive interventions with natural phenolic compounds are necessary to establish their efficacy as disease-modifying agents.

  8. Nonfluent/agrammatic PPA with in-vivo cortical amyloidosis and Pick's disease pathology.

    PubMed

    Caso, Francesca; Gesierich, Benno; Henry, Maya; Sidhu, Manu; LaMarre, Amanda; Babiak, Miranda; Miller, Bruce L; Rabinovici, Gil D; Huang, Eric J; Magnani, Giuseppe; Filippi, Massimo; Comi, Giancarlo; Seeley, William W; Gorno-Tempini, Maria Luisa

    2013-01-01

    The role of biomarkers in predicting pathological findings in the frontotemporal dementia (FTD) clinical spectrum disorders is still being explored. We present comprehensive, prospective longitudinal data for a 66 year old, right-handed female who met current criteria for the nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA). She first presented with a 3-year history of progressive speech and language impairment mainly characterized by severe apraxia of speech. Neuropsychological and general motor functions remained relatively spared throughout the clinical course. Voxel-based morphometry (VBM) showed selective cortical atrophy of the left posterior inferior frontal gyrus (IFG) and underlying insula that worsened over time, extending along the left premotor strip. Five years after her first evaluation, she developed mild memory impairment and underwent PET-FDG and PiB scans that showed left frontal hypometabolism and cortical amyloidosis. Three years later (11 years from first symptom), post-mortem histopathological evaluation revealed Pick's disease, with severe degeneration of left IFG, mid-insula, and precentral gyrus. Alzheimer's disease (AD) (CERAD frequent/Braak Stage V) was also detected. This patient demonstrates that biomarkers indicating brain amyloidosis should not be considered conclusive evidence that AD pathology accounts for a typical FTD clinical/anatomical syndrome.

  9. Methylene Blue Modulates β-Secretase, Reverses Cerebral Amyloidosis, and Improves Cognition in Transgenic Mice*

    PubMed Central

    Mori, Takashi; Koyama, Naoki; Segawa, Tatsuya; Maeda, Masahiro; Maruyama, Nobuhiro; Kinoshita, Noriaki; Hou, Huayan; Tan, Jun; Town, Terrence

    2014-01-01

    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 amyloidosis 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 amyloidosis by modulating the amyloidogenic pathway. PMID:25157105

  10. Successful treatment of lichen amyloidosis using a CO2 surgical laser.

    PubMed

    Norisugi, Osamu; Yamakoshi, Takako; Shimizu, Tadamichi

    2014-01-01

    Lichen amyloidosis (LA) is a type of primary localized cutaneous amyloidosis characterized by multiple pruritic discrete hyperkeratotic papules with amyloid deposition in the papillary dermis. Two patients with LA had been treated with topical corticosteroids, but with no effect on the eruptions. The present authors then started treating the affected area by superficial ablation using a CO2 surgical laser (LASER 30C, Lumenis Inc., Yokneum, Israel) at a setting of 10-15 watts with a 0.12-second pulse duration, 0.36-second rest duration, and 5-mm laser spot size. The present authors treated the patients twice a month with the CO2 laser. The papules on the legs had flattened in both patients, with a great improvement in the severe itching after 6 months in Case 1 and after 10 months in Case 2. These cases indicate that the CO2 laser led to a good response in terms of the clinical manifestations, and may be useful for the treatment of LA.

  11. Brain inflammation and oxidative stress in a transgenic mouse model of Alzheimer-like brain amyloidosis

    PubMed Central

    Yao, Yuemang; Chinnici, Cinzia; Tang, Hanguan; Trojanowski, John Q; Lee, Virginia MY; Praticò, Domenico

    2004-01-01

    Background An increasing body of evidence implicates both brain inflammation and oxidative stress in the pathogenesis of Alzheimer's disease (AD). The relevance of their interaction in vivo, however, is unknown. Previously, we have shown that separate pharmacological targeting of these two components results in amelioration of the amyloidogenic phenotype of a transgenic mouse model of AD-like brain amyloidosis (Tg2576). Methods In the present study, we investigated the therapeutic effects of a combination of an anti-inflammatory agent, indomethacin, and a natural anti-oxidant, vitamin E, in the Tg2576 mice. For this reason, animals were treated continuously from 8 (prior to Aβ deposition) through 15 (when Aβ deposits are abundant) months of age. Results At the end of the study, these therapeutic interventions suppressed brain inflammatory and oxidative stress responses in the mice. This effect was accompanied by significant reductions of soluble and insoluble Aβ1-40 and Aβ1-42 in neocortex and hippocampus, wherein the burden of Aβ deposits also was significantly decreased. Conclusions The results of the present study support the concept that brain oxidative stress and inflammation coexist in this animal model of AD-like brain amyloidosis, but they represent two distinct therapeutic targets in the disease pathogenesis. We propose that a combination of anti-inflammatory and anti-oxidant drugs may be a useful strategy for treating AD. PMID:15500684

  12. Mechanism of Action and Clinical Application of Tafamidis in Hereditary Transthyretin Amyloidosis.

    PubMed

    Coelho, Teresa; Merlini, Giampaolo; Bulawa, Christine E; Fleming, James A; Judge, Daniel P; Kelly, Jeffery W; Maurer, Mathew S; Planté-Bordeneuve, Violaine; Labaudinière, Richard; Mundayat, Rajiv; Riley, Steve; Lombardo, Ilise; Huertas, Pedro

    2016-06-01

    Transthyretin (TTR) transports the retinol-binding protein-vitamin A complex and is a minor transporter of thyroxine in blood. Its tetrameric structure undergoes rate-limiting dissociation and monomer misfolding, enabling TTR to aggregate or to become amyloidogenic. Mutations in the TTR gene generally destabilize the tetramer and/or accelerate tetramer dissociation, promoting amyloidogenesis. TTR-related amyloidoses are rare, fatal, protein-misfolding disorders, characterized by formation of soluble aggregates of variable structure and tissue deposition of amyloid. The TTR amyloidoses present with a spectrum of manifestations, encompassing progressive neuropathy and/or cardiomyopathy. Until recently, the only accepted treatment to halt progression of hereditary TTR amyloidosis was liver transplantation, which replaces the hepatic source of mutant TTR with the less amyloidogenic wild-type TTR. Tafamidis meglumine is a rationally designed, non-NSAID benzoxazole derivative that binds with high affinity and selectivity to TTR and kinetically stabilizes the tetramer, slowing monomer formation, misfolding, and amyloidogenesis. Tafamidis is the first pharmacotherapy approved to slow the progression of peripheral neurologic impairment in TTR familial amyloid polyneuropathy. Here we describe the mechanism of action of tafamidis and review the clinical data, demonstrating that tafamidis treatment slows neurologic deterioration and preserves nutritional status, as well as quality of life in patients with early-stage Val30Met amyloidosis.

  13. A Clinico-Epidemiological Study of Macular Amyloidosis from North India

    PubMed Central

    Bandhlish, Anshu; Aggarwal, Asok; Koranne, Ravinder V

    2012-01-01

    Background: Macular amyloidosis (MA) is the most subtle form of cutaneous amyloidosis, characterized by brownish macules in a rippled pattern, distributed predominantly over the trunk and extremities. MA has a high incidence in Asia, Middle East, and South America. Its etiology has yet to be fully elucidated though various risk factors such as sex, race, genetic predisposition, exposure to sunlight, atopy and friction and even auto-immunity have been implicated. Aim: This study attempts to evaluate the epidemiology and risk factors in the etiology of MA. Materials and: Methods: Clinical history and risk factors of 50 patients with a clinical diagnosis of MA were evaluated. Skin biopsies of 26 randomly selected patients were studied for the deposition of amyloid. Results: We observed a characteristic female preponderance (88%) with a female to male ratio of 7.3:1, with a mean age of onset of MA being earlier in females. Upper back was involved in 80% of patients and sun-exposed sites were involved in 64% cases. Incidence of MA was high in patients with skin phototype III. Role of friction was inconclusive Conclusion: Lack of clear-cut etiological factors makes it difficult to suggest a reasonable therapeutic modality. Histopathology is not specific and amyloid deposits can be demonstrated only in a small number of patients. For want of the requisite information on the natural course and definitive etiology, the disease MA remains an enigma and a source of concern for the suffering patients. PMID:22837559

  14. A new ordered tetragonal phase in the Ti3Al+Nb system

    NASA Technical Reports Server (NTRS)

    Hsiung, L. M.; Wadley, H. N. G.

    1992-01-01

    Results are reported from an experimental study of phase stability in a plasma-sprayed Ti3Al+Nb alloy using TEM and electron diffraction techniques. The alloys was produced from Ti3Al+Nb powder via an inductively coupled plasma deposition process. Evidence was found for the ordering of the B2 phase to a structure similar to the DO3-type phase, but with a tetragonal distortion. An ordered tetragonal phase (T) is proposed to account for these observations, and the existence of the B2 - T ordering transformation in the Ti3Al+Nb system is suggested. The T phase can be regarded as a DO3-like phase with a tetragonal distortion. The tegragonality c0/a0 is approximately equal to 1.02, and a0 equals 0.65 +/-0.01 nm.

  15. Fabrication of NiAl intermetallic reactors for microtechnology-based energy chemical systems (MECS)

    SciTech Connect

    Alman, D.A.; Wilson, R.D.; Paul, B.K.

    2001-01-01

    Microtechnology-based energy chemical systems (MECS) offer opportunities for portable power generation, on-site waste remediation, point-of-use chemical synthesis, and heat-transfer. The material requirements for this application include chemical inertness and the ability to be fabricated into structures that contain internal features of complex geometries and small (<250 micrometer) dimensions. It has been recognized that materials with limited formability, like ceramics and intermetallics, may be required for high temperature applications. In this paper, a method for forming an array of internal microchannels in a NiAl device is presented. Microchannels are precision machined (via laser ablation) into elemental Ni and Al foils. During bonding, these foils are converted into NiAl. Results show that this is a viable method for producing aluminide-based structure containing complex, internal features.

  16. 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.

  17. Quasiparticle band structure for the Hubbard systems: Application to. alpha. -CeAl sub 2

    SciTech Connect

    Costa-Quintana, J.; Lopez-Aguilar, F. ); Balle, S. ); Salvador, R. Supercomputer Computations Research Institute, Florida State University, Tallahassee, Florida 32306-4052 )

    1990-04-01

    A self-energy formalism for determining the quasiparticle band structure of the Hubbard systems is deduced. The self-energy is obtained from the dynamically screened Coulomb interaction whose bare value is the correlation energy {ital U}. A method for integrating the Schroedingerlike equation with the self-energy operator is given. The method is applied to the cubic Laves phase of {alpha}-CeAl{sub 2} because it is a clear Hubbard system with a very complex electronic structure and, moreover, this system provides us with sufficient experimental data for testing our method.

  18. 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://ntrs.nasa.gov/search.jsp?R=19800047753&hterms=aloe&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D20%26Ntt%3Daloe','NASA-TRS'); return false;" href="https://ntrs.nasa.gov/search.jsp?R=19800047753&hterms=aloe&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D20%26Ntt%3Daloe"><span><span class="hlt">Al</span>-26 from red giants. [connections with anomalous Mg-26 content in meteorites and solar <span class="hlt">system</span> formation</span></a></p> <p><a target="_blank" href="http://ntrs.nasa.gov/search.jsp">NASA Technical Reports Server (NTRS)</a></p> <p>Norgaard, H.</p> <p>1980-01-01</p> <p>Simplified models of thermally pulsing red giants are investigated, with particular emphasis on predicting the extent to which nuclear processing at the base of the convective envelope in conjunction with processing in the thermally unstable He shell can synthesize <span class="hlt">Al</span>-26 (tau/1/2/ = 7.2 x 10 to the 5th yr). Values of <span class="hlt">Al</span>-26/<span class="hlt">Al</span>-27 of about 0.5-1, with <span class="hlt">Al</span>-27/<span class="hlt">Al</span>-27(solar) of about 1-2, are predicted in some cases. It is pointed out that such results can lead to isotope shifts in the absorption lines of <span class="hlt">Al</span>H and <span class="hlt">Al</span>O, which should be observationally identifiable in some late-type supergiants. The possible connection with the anomalous Mg-26 content (assigned to the decay of <span class="hlt">Al</span>-26) detected in some meteorites and the connection with formation of the solar <span class="hlt">system</span> are also touched on.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/scitech/biblio/21494107','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/21494107"><span>A new phase in the <span class="hlt">system</span> lithium-aluminum: Characterization of orthorhombic Li{sub 2}<span class="hlt">Al</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Puhakainen, Kati; Bostroem, Magnus; Groy, Thomas L.; Haeussermann, Ulrich</p> <p>2010-11-15</p> <p>Investigation of the Li rich part of the binary Li-<span class="hlt">Al</span> <span class="hlt">system</span> revealed the existence of a new phase, orthorhombic Li{sub 2}<span class="hlt">Al</span>, which is isostructural to Li{sub 2}Ga and Li{sub 2}In. The crystal structure was determined from single crystal X-ray diffraction data (Cmcm, a=4.658(2) A, b=9.767(4) A, c=4.490(2) A, Z=4). Refinement of atomic position site occupancies yielded a composition Li{sub 1.92}<span class="hlt">Al</span>{sub 1.08} (64 at% Li) indicating a small homogeneity range, Li{sub 2-x}<span class="hlt">Al</span>{sub 1+x}. Li{sub 2}<span class="hlt">Al</span> is the peritectic decomposition product of the stoichiometric compound Li{sub 9}<span class="hlt">Al</span>{sub 4}, which is stable below 270{+-}2 {sup o}C. Li{sub 2}<span class="hlt">Al</span> itself decomposes peritectically to Li{sub 3}<span class="hlt">Al</span>{sub 2} and Li rich melt at 335{+-}2 {sup o}C. The discovery of Li{sub 2}<span class="hlt">Al</span> (Li{sub 2-x}<span class="hlt">Al</span>{sub 1+x}) settles a long standing inconsistency in the Li-<span class="hlt">Al</span> phase diagram which was based on the assumption that Li{sub 9}<span class="hlt">Al</span>{sub 4} possesses a high temperature modification. - Graphical abstract: A new phase, Li{sub 2}<span class="hlt">Al</span>, has been discovered in the binary Li-<span class="hlt">Al</span> <span class="hlt">system</span>. The structure of orthorhombic Li{sub 2}<span class="hlt">Al</span> is closely related to that of the established monoclinic phase Li{sub 9}<span class="hlt">Al</span>{sub 4}.</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://www.dtic.mil/docs/citations/ADA601698','DTIC-ST'); return false;" href="http://www.dtic.mil/docs/citations/ADA601698"><span>A <span class="hlt">Systemic</span> Assessment Model for <span class="hlt">Al</span>-Qaida’s Global Jihad</span></a></p> <p><a target="_blank" href="https://publicaccess.dtic.mil/psm/api/service/search/search">DTIC Science & Technology</a></p> <p></p> <p>2013-04-11</p> <p>infrastructure, boycotts of Western goods and services, and “ depriv [ing] the western crusader from stealing the Muslims’ oil which is being drained as the...strategy. The subsequent demonstration employs the latter approach, examining the <span class="hlt">systemic</span> impact of Operation IRAQI FREEDOM (OIF) on <span class="hlt">Al</span>-Qaida’s...Prevent exploitation of Muslim resources by the infidels. MoP: Do the infidels reap unfair benefits from Muslim oil production? MoP: Do the</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://ntrs.nasa.gov/search.jsp?R=19760050746&hterms=frictions+metal&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D40%26Ntt%3Dfrictions%2Bmetal','NASA-TRS'); return false;" href="https://ntrs.nasa.gov/search.jsp?R=19760050746&hterms=frictions+metal&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D40%26Ntt%3Dfrictions%2Bmetal"><span>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> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/27761255','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/27761255"><span>Congenital insensitivity to pain and anhydrosis due to a rare mutation and that is complicated by inflammatory bowel disease and <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>Bakri, Faris G; Wahbeh, Ayman; Abu Sneina, Awni; Al Khader, Ali; Obeidat, Fatima; AlAwwa, Izzat; Buni, Maryam; Ki, Chang-Seok; Masri, Amira</p> <p>2016-10-01</p> <p>Patients with congenital insensitivity to pain and anhydrosis syndrome are at risk for renal <span class="hlt">amyloidosis</span> and inflammatory bowel disease. Physicians caring for such patients should be aware of these complications.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2006PSSAR.203.2581J','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2006PSSAR.203.2581J"><span>Real-time investigations of selenization reactions in the <span class="hlt">system</span> Cu-In-<span class="hlt">Al</span>-Se</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Jost, Stefan; Hergert, Frank; Hock, Rainer; Purwins, Michael; Enderle, Ralph</p> <p>2006-09-01</p> <p>In this article we present results of a detailed study of selenization reactions in the quaternary <span class="hlt">system</span> Cu-In-<span class="hlt">Al</span>-Se and of the binary subsystem aluminum-selenium. The investigation of solid-state reactions involved in the formation of the compound semiconductor Cu(In,<span class="hlt">Al</span>)Se2 was performed using real-time X-ray diffraction (XRD) with a time resolution of 22.5 s while annealing an elemental layer stack of the metals covered with selenium. A temperature-resolved phase analysis shows that the formation of the semiconductor takes place via metal-selenides. Ex-situ XRD measurements of the processed thin films show a phase segregation concerning the aluminum content of the formed chalcopyrite. Subsequent Rietveld-refinement of real-time measurements reveals a formation reaction of the quaternary semiconductor Cu(In,<span class="hlt">Al</span>)Se2 from the -In2Se3 related crystal structure of (<span class="hlt">Al</span>,In)2Se3 and Cu2Se as educts.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016JAP...120t5304L','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016JAP...120t5304L"><span>Structural and thermoelectric properties of SiGe/<span class="hlt">Al</span> multilayer <span class="hlt">systems</span> during metal induced crystallization</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Lindorf, M.; Rohrmann, H.; Span, G.; Raoux, S.; Jordan-Sweet, J.; Albrecht, M.</p> <p>2016-11-01</p> <p>While the process of metal induced crystallization (MIC) is widely used in the fabrication of thin film electronic devices, its application to the field of thermoelectrics is fairly new. Especially, its implementation in the field of the classic thermoelectric material SiGe could lead to a low cost approach by combining the benefits of low thermal budget, self-doping, and thin film and sputter deposition compatibility. In this work, samples consisting of SiGe/<span class="hlt">Al</span> multilayers deposited on aluminum oxide based substrates have been prepared. Special emphasis was put on the ratio of <span class="hlt">Al</span> to SiGe and the resulting changes in transport properties during annealing. On one hand, a certain amount of <span class="hlt">Al</span> is needed to ensure a complete MIC process for the SiGe, but on the other hand, an excess of <span class="hlt">Al</span> results in a metallic <span class="hlt">system</span> with low thermoelectric efficiency. In-situ characterization during annealing of the samples was carried out via x-ray diffraction, electrical resistivity, and Seebeck measurements.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/scitech/biblio/22255054','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/22255054"><span>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://adsabs.harvard.edu/abs/2011ApPhL..98i1905M','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2011ApPhL..98i1905M"><span>Influence of sample processing parameters on thermal boundary conductance value in an <span class="hlt">Al/Al</span>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>Monachon, Christian; Hojeij, Mohamad; Weber, Ludger</p> <p>2011-02-01</p> <p>The influence of sample processing parameters on the thermal boundary conductance (TBC) between aluminum and aluminum nitride has been investigated by transient thermoreflectance. An evaporated <span class="hlt">Al</span> layer on the polished substrate yielded a TBC at ambient of roughly 47 MW m-2 K-1. The largest improvement (by a factor of 5) was obtained by plasma-etching of the substrate and subsequent evaporation of the metal layer. Electron microscopy suggests that the differences in TBC were mainly due to the (partial) elimination of the native oxide layer on the substrate. The importance of an adequate model for data extraction on measured TBC is highlighted.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2014SPIE.8973E..0IK','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2014SPIE.8973E..0IK"><span>Optimal microelectromechanical <span class="hlt">systems</span> (MEMS) device for achieving high pyroelectric response of <span class="hlt">Al</span>N</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Kebede, Bemnnet; Coutu, Ronald A.; Starman, LaVern</p> <p>2014-03-01</p> <p>This paper discusses research being conducted on aluminum nitride (<span class="hlt">Al</span>N) as a pyroelectric material for use in detecting applications. <span class="hlt">Al</span>N is being investigated because of its high pyroelectric coefficient, thermal stability, and high Curie temperature. In order to determine suitability of the pyroelectric properties of <span class="hlt">Al</span>N for use as a detector, testing of several devices was conducted. These devices were fabricated using microelectromechanical <span class="hlt">systems</span> (MEMS) fabrication processes; the devices were also designed to allow for voltage and current measurements. The deposited <span class="hlt">Al</span>N films used were 150 nm - 300 nm in thickness. Thin-films were used to rapidly increase the temperature response after the thermal stimulus was applied to the pyroelectric material. This is important because the pyroelectric effect is directly proportional to the rate of temperature change. The design used was a face-electrode bridge that provides thermal isolation which minimizes heat loss to the substrate, thereby increasing operation frequency of the pyroelectric device. A thermal stimulus was applied to the pyroelectric material and the response was measured across the electrodes. A thermal imaging camera was used to monitor the changes in temperature. Throughout the testing process, the annealing temperatures, type of layers, and thicknesses were also varied. These changes resulted in improved MEMS designs, which were fabricated to obtain an optimal design configuration for achieving a high pyroelectric response. A pyroelectric voltage response of 38.9 mVp-p was measured without filtering, 12.45 mVp-p was measured in the infrared (IR) region using a Si filter, and 6.38 mVp-p was measured in the short wavelength IR region using a long pass filter. The results showed that <span class="hlt">Al</span>N's pyroelectric properties can be used in detecting applications.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/15179309','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/15179309"><span>[BIG-H3 protein: mutation of codon 124 and corneal <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>Schmitt-Bernard, C-F; Pouliquen, Y; Argilès, A</p> <p>2004-05-01</p> <p>In 1997, a group of hereditary corneal dystrophies was related to mutations in the TGFBI (BIGH3) gene. Within this group, some corneal dystrophies present particular biochemical features in that they are characterized by corneal amyloid deposition. Contrary to clinical and genetic knowledge, the biochemical characteristics of the encoded protein (Big-h3) and the mechanisms of its amyloid conversion remain unclear. We review the current knowledge on the Big-h3 protein and focus on the behavior of the codon 124 region. We discuss this protein's mechanisms of amyloid conversion from our results and previous reports as well as from other types of <span class="hlt">amyloidosis</span>. These data provide a better understanding of the putative processes leading to the phenotypic variations linked with their respective codon 124 mutation.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/1373079','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/1373079"><span>Topographic relationship between senile plaques and cerebrovascular <span class="hlt">amyloidosis</span> in the brain of aged dogs.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Shimada, A; Kuwamura, M; Awakura, T; Umemura, T; Takada, K; Ohama, E; Itakura, C</p> <p>1992-02-01</p> <p>The distributions of senile plaques (SP) and cerebrovascular <span class="hlt">amyloidosis</span> (CA) were studied by employing thioflavin S and modified Bielschowsky stains, and beta-protein immunohistochemistry on serial sections of the brains of aged dogs older than 10 years. Mature and perivascular plaques, both of which contained compact amyloid deposits, always showed a close topographic relationship to CA. In contrast, the majority of diffuse plaques showed no topographic relationship to CA. Cell bodies of neurons and/or glia were almost always involved in the diffuse plaques. In addition, beta-protein immunohistochemistry demonstrated amyloid deposits on the periphery of occasional neurons. These findings suggest that different mechanisms may be involved in the development of the different subtypes of SP in the brains of aged dogs.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/14639586','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/14639586"><span>Gelsolin-related familial <span class="hlt">amyloidosis</span>, Finnish type, in a Portuguese family: clinical and neurophysiological studies.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Conceição, Isabel; Sales-Luis, Maria Lurdes; De Carvalho, Mamede; Evangelista, Teresinha; Fernandes, Rui; Paunio, Tiina; Kangas, Hannele; Coutinho, Paula; Neves, Carlos; Saraiva, Maria João</p> <p>2003-12-01</p> <p>We report a Portuguese family with familial amyloid polyneuropathy related to gelsolin. There were no known Finnish ancestors, but the same mutation as described in Finnish patients (G654A) was carried. Clinical and neurophysiological investigations were performed in four patients. Corneal lattice dystrophy affected all four patients; an axonal lesion of the facial nerve occurred in three patients; visual tract involvement was documented in one case; and corticospinal and posterior column dysfunction was present in one patient. Polarizing microscopy of skin and muscle samples demonstrated amyloid deposits in two patients; anti-gelsolin immunohistochemistry was positive for amyloidogenic gelsolin. The Finnish mutation of gelsolin protein (G654A) was detected in five family members. The utility of neurophysiological testing in the evaluation and follow-up of this type of <span class="hlt">amyloidosis</span> is discussed.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2012PhPro..39..382S','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2012PhPro..39..382S"><span>Direct Metal Deposition of Functional Graded Structures in 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>Shishkovsky, I.; Missemer, F.; Smurov, I.</p> <p></p> <p>A direct laser metal deposition (DLMD) technology with co-axial powder injection is used to fabricate a complex functional graded structure (FGS) fabrication. The aim of the study is to demonstrate the possibility to produce intermetallic phases in the Ti-<span class="hlt">Al</span> powder <span class="hlt">systems</span> in the course of a single-step DMD process. Besides, relationships between the main laser cladding parameters and the intermetallic phase structures of the built-up objects have been studied. In our research we applied the optical microscopy, X-ray analysis, microhardness measurement and SEM with EDX analysis of the laser-fabricated intermetallics. The discussion of the mechanisms of Ti x <span class="hlt">Al</span> y (x,y = 1.3) intermetallic transformations in exothermal reactions is also offered in the report.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/scitech/biblio/22111950','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/22111950"><span>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('https://www.osti.gov/scitech/biblio/1327114','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/1327114"><span>FORMATION MECHANISM FOR THE NANOSCALE AMORPHOUS INTERFACE IN PULSE-WELDED <span class="hlt">AL</span>/FE BIMETALLIC <span class="hlt">SYSTEM</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Li, Jingjing; Yu, Qian; Zhang, Zijiao; Xu, Wei; Sun, Xin</p> <p>2016-05-20</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 resulted recrystallization occurred on the aluminum side of the interface.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2017MSHT...58..515K','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2017MSHT...58..515K"><span>Strength of "Light" Ferritic and Austenitic Steels Based on the Fe - Mn - <span class="hlt">Al</span> - C <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>Kaputkina, L. M.; Svyazhin, A. G.; Smarygina, I. V.; Kindop, V. E.</p> <p>2017-01-01</p> <p>The phase composition, the hardness, the mechanical properties at room temperature, and the resistance to hot (950 - 1000°C) and warm (550°C) deformation are studied for cast deformable "light" ferritic and austenitic steels of the Fe - (12 - 25)% Mn - (0 - 15)% <span class="hlt">Al</span> - (0 - 2)% C <span class="hlt">system</span> alloyed additionally with about 5% Ni. The high-aluminum high-manganese low-carbon and carbonless ferritic steels at a temperature of about 0.5 T melt have a specific strength close to that of the austenitic steels and may be used as weldable scale-resistant and wear-resistant materials. The high-carbon Fe - (20 - 24)% Mn - (5 - 9)% <span class="hlt">Al</span> - 5% Ni - 1.5% C austenitic steels may be applied as light high-strength materials operating at cryogenic temperatures after a solution treatment and as scale- and heat-resistant materials in an aged condition.</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>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://adsabs.harvard.edu/abs/2016JSSCh.243..168M','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016JSSCh.243..168M"><span>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/8589295','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/8589295"><span>Deposition of kappa and lambda light chains in amyloid filaments of 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>Brancaccio, D; Ghiggeri, G M; Braidotti, P; Garberi, A; Gallieni, M; Bellotti, V; Zoni, U; Gusmano, R; Coggi, G</p> <p>1995-10-01</p> <p>beta 2-Microglobulin (beta 2m) is considered to be the amyloidogenic precursor in dialysis-related <span class="hlt">amyloidosis</span>, although the implication of other relevant cofactors in the pathogenesis of this disease has also been hypothesized. It is conceivable that substances found in amyloid deposits might represent something more than simple codeposition, possibly playing a pathogenic role in amyloidogenesis. Along these lines, a detailed analysis of the protein composition of amyloid fibrils purified from synovial material surgically obtained from nine patients on long-term dialysis was carried out. By the use of sodium dodecyl sulfate-polyacrylamide gel electrophoresis, several other protein components, in addition to beta 2m, were found. These were characterized by NH2 amino-terminal sequencing and immunoblotting. In fibrils obtained by water extraction, which fulfill the electron microscopy criteria of highly pure amyloid material, polyclonal kappa and lambda light chains were detected with a concentration of 15 micrograms/mL in the water extraction material; the beta 2m concentration was 200 micrograms/mL. Light microscopy immunohistochemistry was performed on samples from five patients. Amyloid deposits reacted with anti-beta 2m, and anti-light (kappa, lambda), chain antibodies. The immunoreaction of amyloid filaments to anti-beta 2m, anti-lambda, and anti-kappa light chain antibodies was also tested by electron microscopy by use of the immunogold staining procedure. Amyloid filaments were labeled by the three antibodies and showed a different intensity of immunostaining apparently related to their different aggregation pattern. These observations demonstrate that polyclonal immunoglobulin light chains (kappa and lambda) are not contaminants but, together with beta 2m, represent a major constituent of amyloid deposits in dialysis-related osteoarticular <span class="hlt">amyloidosis</span>, thus indicating their possible role in amyloidogenesis.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/23113696','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/23113696"><span>Amyloid fibril protein nomenclature: 2012 recommendations from the Nomenclature Committee of the International Society of <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>Sipe, Jean D; Benson, Merrill D; Buxbaum, Joel N; Ikeda, Shu-ichi; Merlini, Giampaolo; Saraiva, Maria J M; Westermark, Per</p> <p>2012-12-01</p> <p>The Nomenclature Committee of the International Society of <span class="hlt">Amyloidosis</span> (ISA) met during the XIIIth International Symposium, May 6-10, 2012, Groningen, The Netherlands, to formulate recommendations on amyloid fibril protein nomenclature and to consider newly identified candidate amyloid fibril proteins for inclusion in the ISA Amyloid Fibril Protein Nomenclature List. The need to promote utilization of consistent and up to date terminology for both fibril chemistry and clinical classification of the resultant disease syndrome was emphasized. Amyloid fibril nomenclature is based on the chemical identity of the amyloid fibril forming protein; clinical classification of the <span class="hlt">amyloidosis</span> should be as well. Although the importance of fibril chemistry to the disease process has been recognized for more than 40 years, to this day the literature contains clinical and histochemical designations that were used when the chemical diversity of amyloid diseases was poorly understood. Thus, the continued use of disease classifications such as familial amyloid neuropathy and familial amyloid cardiomyopathy generates confusion. An amyloid fibril protein is defined as follows: the protein must occur in body tissue deposits and exhibit both affinity for Congo red and green birefringence when Congo red stained deposits are viewed by polarization microscopy. Furthermore, the chemical identity of the protein must have been unambiguously characterized by protein sequence analysis when so is practically possible. Thus, in nearly all cases, it is insufficient to demonstrate mutation in the gene of a candidate amyloid protein; the protein itself must be identified as an amyloid fibril protein. Current ISA Amyloid Fibril Protein Nomenclature Lists of 30 human and 10 animal fibril proteins are provided together with a list of inclusion bodies that, although intracellular, exhibit some or all of the properties of the mainly extracellular amyloid fibrils.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/20555144','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/20555144"><span>GM-CSF upregulated in rheumatoid arthritis reverses cognitive impairment and <span class="hlt">amyloidosis</span> in Alzheimer mice.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Boyd, Tim D; Bennett, Steven P; Mori, Takashi; Governatori, Nicholas; Runfeldt, Melissa; Norden, Michelle; Padmanabhan, Jaya; Neame, Peter; Wefes, Inge; Sanchez-Ramos, Juan; Arendash, Gary W; Potter, Huntington</p> <p>2010-01-01</p> <p>Rheumatoid arthritis (RA) is a negative risk factor for the development of Alzheimer's disease (AD). While it has been commonly assumed that RA patients' usage of non-steroidal anti-inflammatory drugs (NSAIDs) helped prevent onset and progression of AD, NSAID clinical trials have proven unsuccessful in AD patients. To determine whether intrinsic factors within RA pathogenesis itself may underlie RA's protective effect, we investigated the activity of colony-stimulating factors, upregulated in RA, on the pathology and behavior of transgenic AD mice. 5 microg bolus injections of macrophage, granulocyte, and granulocyte-macrophage colony-stimulating factors (M-CSF, G-CSF, or GM-CSF) were administered unilaterally into the hippocampus of aged cognitively-impaired AD mice and the resulting amyloid load reductions determined one week later, using the artificial cerebrospinal fluid-injected contralateral sides as controls. G-CSF and more significantly, GM-CSF reduced <span class="hlt">amyloidosis</span> throughout the treated brain hemisphere one week following bolus administration to AD mice. 20 daily subcutaneous injections of 5 microg of GM-CSF (the most amyloid-reducing CSF in the bolus experiment) were administered to balanced cohorts of AD mice after assessment in a battery of cognitive tests. Reductions in amyloid load and improvements in cognitive function were assessed. Subcutaneous GM-CSF administration significantly reduced brain <span class="hlt">amyloidosis</span> and completely reversed the cognitive impairment, while increasing hippocampal synaptic area and microglial density. These findings, along with two decades of accrued safety data using Leukine, recombinant human GMCSF, in elderly leukopenic patients, suggest that Leukine should be tested as a treatment to reverse cerebral amyloid pathology and cognitive impairment in AD.</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('https://medlineplus.gov/ency/article/000193.htm','NIH-MEDLINEPLUS'); return false;" href="https://medlineplus.gov/ency/article/000193.htm"><span>Cardiac <span class="hlt">amyloidosis</span></span></a></p> <p><a target="_blank" href="http://medlineplus.gov/">MedlinePlus</a></p> <p></p> <p></p> <p>... RO, Mann DL, Zipes DP, Libby P, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine . 10th ed. ... Updated by: Michael A. Chen, MD, PhD, Associate Professor of Medicine, ...</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://circ.ahajournals.org/content/126/12/e178.full','NIH-MEDLINEPLUS'); return false;" href="http://circ.ahajournals.org/content/126/12/e178.full"><span>Cardiac <span class="hlt">Amyloidosis</span></span></a></p> <p><a target="_blank" href="http://medlineplus.gov/">MedlinePlus</a></p> <p></p> <p></p> <p>... In the normal heart, the muscle fibers (stained pink in this slide) are close together with little ... amount of amyloid deposited between them (staining light pink-purple). (Images courtesy of Dr Paul VanderLaan, Brigham ...</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/scitech/biblio/22342232','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/22342232"><span><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://www.osti.gov/scitech/servlets/purl/67710','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/servlets/purl/67710"><span>The amplitude and phase control of the <span class="hlt">ALS</span> Storage Ring RF <span class="hlt">System</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Lo, C.C.; Taylor, B.; Baptiste, K.</p> <p>1995-03-01</p> <p>A 500MHz, 300KW Klystron power amplifier provides RF power to the <span class="hlt">ALS</span> Storage Ring. In order to accommodate the amplitude and phase changes during beam stacking and decay, which demand continuously varying power levels from the Klystron, four loops are used to keep the <span class="hlt">system</span> operating properly, with two of those loops dedicated to keeping the two cavity tuners on tune. Description of the control loops and their performance data will be given. Using the modulation anode of the Klystron in the amplitude loop will be discussed.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/scitech/biblio/186788','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/186788"><span>Combustion synthesis of metal-matrix composites. Part 2: The Ti-Ti{sub x}<span class="hlt">Al</span>{sub y}-<span class="hlt">Al</span>{sub 2}O{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>Kunrath, A.O.; Strohaecker, T.R.; Moore, J.J.</p> <p>1996-01-15</p> <p>The production of high performance materials (ceramics, intermetallics and composites) by combustion synthesis is receiving considerable attention since the process offers certain advantages with respect to simplicity and a relatively low energy requirement. The methods by which combustion synthesis (or SHS) can be used to produce metal matrix composites were outlined in an earlier paper. The use of excess liquid metal in the combustion synthesis reaction has already been successfully employed to achieve low porosity products. This metallic phase may be generated by an in-situ reduction of a metal oxide or by adding an excess of some metal to the reactants. Coupling a simultaneous consolidation (pressing) process with the SHS reaction has been found to produce dense bodies. This work discussed in this paper is concerned with the synthesis of a metallic/intermetallic matrix composite reaction <span class="hlt">system</span> which can be represented by equation. With this reaction, high volume fractions of metallic/intermetallic phases can be produced. The metal-matrix produced by this reaction is predominantly a mixture of Ti{sub 3}<span class="hlt">Al</span> + Ti as indicated in the appropriate area of the Ti-<span class="hlt">Al</span> phase diagram. Increasing x increases the volume fraction of Ti. Ti<span class="hlt">Al</span> was observed in only one of the four different stoichiometries studied, i.e., x = 1, as detected by XRD. Using this reaction, there is a constant amount of excess <span class="hlt">Al</span> and a variable excess of Ti. These stoichiometries produce composites with varying Ti-<span class="hlt">Al</span> ratios that allow the matrix composition of the composite to be varied along the concentration axis of the Ti-<span class="hlt">Al</span> phase diagram.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/scitech/biblio/21448880','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/21448880"><span>{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('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=442492','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=442492"><span>Identification of a new hereditary <span class="hlt">amyloidosis</span> prealbumin variant, Tyr-77, and detection of the gene by DNA analysis.</span></a></p> <p><a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pmc">PubMed Central</a></p> <p>Wallace, M R; Dwulet, F E; Williams, E C; Conneally, P M; Benson, M D</p> <p>1988-01-01</p> <p>In the last several years, five human plasma prealbumin (transthyretin) variants have been discovered in association with hereditary <span class="hlt">amyloidosis</span>, a late-onset fatal disorder. We recently studied a patient of German descent with peripheral neuropathy and bowel dysfunction. Biopsied rectal tissue contained amyloid that stained with anti-human prealbumin. Amino acid sequence analysis of the patient's plasma prealbumin revealed both normal and variant prealbumin molecules, with the variant containing a tyrosine at position 77 instead of serine. We predicted a single nucleotide change in codon 77 of the variant prealbumin gene, which we then detected in the patient's DNA using the restriction enzyme SspI and a specifically tailored genomic prealbumin probe. DNA tests of other family members identified several gene carriers. This is the sixth prealbumin variant implicated in <span class="hlt">amyloidosis</span>, and adds to the accumulating evidence that the prealbumin amyloidoses are more varied and prevalent than previously thought. Images PMID:2891727</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/scitech/biblio/922165','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/922165"><span>A Potentiometric, Spectrophotometric and Pitzer Ion-Interaction Study of Reaction Equilibria in the Aqueous H+-<span class="hlt">Al</span>3+, H+-Oxalate and H+-<span class="hlt">Al</span>3+-Oxalate <span class="hlt">Systems</span> up to 5 mol*dm-3 NaCl</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Boily, Jean F.; Qafoku, Odeta; Felmy, Andrew R.</p> <p>2007-12-01</p> <p>Aluminium-oxalate complexation was determined in acidic media of aqueous NaCl solutions ranging from 0.1-5.0 mol•dm-3. Complexation in the H+-<span class="hlt">Al</span>3+ and H+-Oxalate <span class="hlt">systems</span> was also studied to provide a set of internally consistent thermodynamic data. The ionic strength dependent formation constants describing the stabilities of the <span class="hlt">Al</span>3+, <span class="hlt">Al</span>OH2+, <span class="hlt">Al</span>3(OH)45+, <span class="hlt">Al</span>13O4(OH)247+, H2L, HL-, L2-, <span class="hlt">Al</span>L+, <span class="hlt">Al</span>L2- and <span class="hlt">Al</span>L33- species (where L is the oxalate ion) was also described using a Pitzer ion interaction model. The derived parameters can be used to predict chemical speciation in the H+-<span class="hlt">Al</span>3+-Oxalate <span class="hlt">system</span> in the 0.1-5.0 mol•dm-3 NaCl range.</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>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('https://www.osti.gov/scitech/biblio/21300663','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/21300663"><span>{sup 26}<span class="hlt">Al</span> AND THE FORMATION OF THE SOLAR <span class="hlt">SYSTEM</span> FROM A MOLECULAR CLOUD CONTAMINATED BY WOLF-RAYET WINDS</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Gaidos, Eric; Krot, Alexander N.; Williams, Jonathan P.; Raymond, Sean N. E-mail: sasha@higp.hawaii.edu E-mail: sean.raymond@colorado.edu</p> <p>2009-05-10</p> <p>In agreement with previous work, we show that the presence of the short-lived radionuclide (SLR) {sup 26}<span class="hlt">Al</span> in the early solar <span class="hlt">system</span> was unlikely (less than 2% a priori probability) to be the result of direct introduction of supernova (SN) ejecta into the gaseous disk during the Class II stage of protosolar evolution. We also show that Bondi-Hoyle accretion of any contaminated residual gas from the Sun's natal star cluster contributed negligible {sup 26}<span class="hlt">Al</span> to the primordial solar <span class="hlt">system</span>. Our calculations are consistent with the absence of the oxygen isotopic signature expected with any late introduction of SN ejecta into the protoplanetary disk. Instead, the presence of {sup 26}<span class="hlt">Al</span> in the oldest solar <span class="hlt">system</span> solids (calcium-aluminum-rich inclusions (CAIs)) and its apparent uniform distribution with the inferred canonical {sup 26}<span class="hlt">Al</span>/{sup 27}<span class="hlt">Al</span> ratio of (4.5-5) x 10{sup -5} support the inheritance of {sup 26}<span class="hlt">Al</span> from the Sun's parent giant molecular cloud. We propose that this radionuclide originated in a prior generation of massive stars that formed in the same molecular cloud and contaminated that cloud by Wolf-Rayet winds. We calculated the Galactic distribution of {sup 26}<span class="hlt">Al</span>/{sup 27}<span class="hlt">Al</span> ratios that arise from such contamination using the established embedded cluster mass and stellar initial mass functions, published nucleosynthetic yields from the winds of massive stars, and by assuming rapid and uniform mixing into the cloud. Although our model predicts that the majority of stellar <span class="hlt">systems</span> contain no {sup 26}<span class="hlt">Al</span> from massive stars, and that the a priori probability that the {sup 26}<span class="hlt">Al</span>/{sup 27}<span class="hlt">Al</span> ratio will reach or exceed the canonical solar <span class="hlt">system</span> value is only {approx}6%, the maximum in the distribution of nonzero values is close to the canonical {sup 26}<span class="hlt">Al</span>/{sup 27}<span class="hlt">Al</span> ratio. We find that the Sun most likely formed 4-5 million years (Myr) after the massive stars that were the source of {sup 26}<span class="hlt">Al</span>. Furthermore, our model can explain the initial solar <span class="hlt">system</span></p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/18925459','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/18925459"><span>Long-term treatment of anemia with recombinant human erythropoietin in familial <span class="hlt">amyloidosis</span> TTR V30M.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Beirão, Idalina; Lobato, Luísa; Moreira, Luciana; Mp Costa, Paulo; Fonseca, Isabel; Cabrita, António; Porto, Graça</p> <p>2008-09-01</p> <p>Familial <span class="hlt">amyloidosis</span> or familial amyloid polyneuropathy (FAP) TTR V30M is a hereditary disease presented, in most cases, as a sensorimotor and autonomic neuropathy. Normocytic and normochromic anaemia was found in 24.8% of symptomatic FAP patients associated to lower serum erythropoietin (Epo) levels. Erythropoietin has been reported as efficient in anaemia correction in this disease. To evaluate the tolerance and efficacy of this treatment, a retrospective longitudinal study with 24 patients was undertaken. Patients were followed for at least 6 months. Haemoglobin, hematocrit, iron status, serum creatinine and urea and r-HuEPO doses were monitored, at 0, 3 months, 6 months and at the end of the follow-up. Long-term use of r-HuEPO proved to be efficient in the treatment of anaemia in familial <span class="hlt">amyloidosis</span> TTR V30M and, despite the disease progression, no resistance cases to this treatment were observed. Positive side effects, like improvement on orthostatic hypotension symptoms and well-being sensation, contributing to confirm erythropoietin as a drug of choice to treat anaemia in <span class="hlt">amyloidosis</span> TTR V30M.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/scitech/biblio/186789','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/186789"><span>Combustion synthesis of metal-matrix composites. Part 3: The <span class="hlt">Al-TiC-Al</span>{sub 2}O{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>Kunrath, A.O.; Strohaecker, T.R.; Moore, J.J.</p> <p>1996-01-15</p> <p>The principle of combustion synthesis to produce metal matrix composites has been outlined in earlier papers. Applying pressure either during or immediately after the reaction is completed is the most commonly used method to achieve high densification of the synthesized products. Some advanced ceramics (TiC and TiB{sub 2}) have been reported to achieve up to 95% of theoretical density using this technique. The current research is a continuation of the work on the TiC-<span class="hlt">Al</span>{sub 2}O{sub 3}-<span class="hlt">Al</span> <span class="hlt">system</span>, in which an excess amount of liquid aluminum is generated by the exothermic reaction and infiltrates the pores of the ceramic matrix improving the densification of the product. The current research is aimed at synthesizing high volume fractions, i.e., > 50%, metal matrix composites using the SHS reaction. The stability of this reaction is inversely proportional to the excess amount of the metal phase added to the reactants, i.e. x<span class="hlt">Al</span>. The excess <span class="hlt">Al</span> acts as a diluent, taking heat from the reaction front, and making it difficult to ignite and/or sustain the reaction in pellets with stoichiometries of high volume fractions of metal. For this reason, the simultaneous combustion (thermal explosion) mode was chosen to perform the synthesis reaction and in which the whole pellet is heated to the ignition temperature.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/scitech/biblio/1164045','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/1164045"><span>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> <li> <p><a target="_blank" onclick="trackOutboundLink('https://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="https://ntrs.nasa.gov/search.jsp?R=19920041145&hterms=ALS&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D10%26Ntt%3DALS"><span>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>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/2015ApSS..355..930S','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2015ApSS..355..930S"><span>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.osti.gov/scitech/biblio/21370547','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/21370547"><span>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('https://www.gpo.gov/fdsys/pkg/FR-2010-03-15/pdf/2010-5519.pdf','FEDREG'); return false;" href="https://www.gpo.gov/fdsys/pkg/FR-2010-03-15/pdf/2010-5519.pdf"><span>75 FR 12256 - United States, et <span class="hlt">al</span>. v. Election <span class="hlt">Systems</span> and Software, Inc.; Proposed Final Judgment and...</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>2010-03-15</p> <p>... Antitrust Division United States, et <span class="hlt">al</span>. v. Election <span class="hlt">Systems</span> and Software, Inc.; Proposed Final Judgment and.... v. Election <span class="hlt">Systems</span> and Software Inc., Civil Action No. 10-00380. On March 8, 2010, the United States filed a Complaint alleging that the proposed acquisition by Election <span class="hlt">Systems</span> and Software,...</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>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('https://www.osti.gov/scitech/biblio/1347806','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/1347806"><span>An Icosahedral Quasicrystal and Its 1/0 Crystalline Approximant in the Ca–Au–<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>Pham, Joyce; Kreyssig, Andreas; Goldman, Alan I.; Miller, Gordon J.</p> <p>2016-10-17</p> <p>A new icosahedral quasicrystalline phase, CaAu4.5–x<span class="hlt">Al</span>1.5+x [0.11 ≤ x ≤ 0.40(6); CaAu4.4<span class="hlt">Al</span>1.6, aQC = 5.383(4) Å, and Pm35], and its lowest-order 1/0 cubic crystalline approximant phase, CaAu3+x<span class="hlt">Al</span>1–x [0 ≤ x ≤ 0.31(1); a = 9.0766(5)–9.1261(8) Å, Pa3(No. 205), and Pearson symbol cP40], have been discovered in the Ca-poor region of the Ca–Au–<span class="hlt">Al</span> <span class="hlt">system</span>. In the crystalline approximant, eight [Au3–x<span class="hlt">Al</span>1+x] tetrahedra fill the unit cell, and each tetrahedron is surrounded by four Ca atoms, thus forming a three-dimensional network of {Ca4/4[Au3–x<span class="hlt">Al</span>1+x]} tetrahedral stars. A computational study of Au and <span class="hlt">Al</span> site preferences concurs with the experimental results, which indicate a preference for near-neighbor Au–<span class="hlt">Al</span> interactions over Au–Au and Al–<span class="hlt">Al</span> interactions. Analysis of the electronic density of states and the associated crystal orbital Hamilton population curves was used to rationalize the descriptions of CaAu4.5–x<span class="hlt">Al</span>1.5+x [0.11 ≤ x ≤ 0.46(6)] and CaAu3+x<span class="hlt">Al</span>1–x [0 ≤ x ≤ 0.31(1)] as polar intermetallic species, whereby Ca atoms engage in polar covalent bonding with the electronegative, electron-deficient [Au3–x<span class="hlt">Al</span>1+x] tetrahedral clusters and the observed phase width of the crystalline approximant.</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('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4737858','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4737858"><span>Improved Outcomes After Autologous Hematopoietic Cell Transplantation for Light Chain <span class="hlt">Amyloidosis</span>: A Center for International Blood and Marrow Transplant Research 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>D'Souza, Anita; Dispenzieri, Angela; Wirk, Baldeep; Zhang, Mei-Jie; Huang, Jiaxing; Gertz, Morie A.; Kyle, Robert A.; Kumar, Shaji; Comenzo, Raymond L.; Peter Gale, Robert; Lazarus, Hillard M.; Savani, Bipin N.; Cornell, Robert F.; Weiss, Brendan M.; Vogl, Dan T.; Freytes, César O.; Scott, Emma C.; Landau, Heather J.; Moreb, Jan S.; Costa, Luciano J.; Ramanathan, Muthalagu; Callander, Natalie S.; Kamble, Rammurti T.; Olsson, Richard F.; Ganguly, Siddhartha; Nishihori, Taiga; Kindwall-Keller, Tamila L.; Wood, William A.; Mark, Tomer M.; Hari, Parameswaran</p> <p>2015-01-01</p> <p>Purpose Autologous hematopoietic cell transplantation, or autotransplantation, is effective in light-chain <span class="hlt">amyloidosis</span> (<span class="hlt">AL</span>), but it is associated with a high risk of early mortality (EM). In a multicenter randomized comparison against oral chemotherapy, autotransplantation was associated with 24% EM. We analyzed trends in outcomes after autologous hematopoietic cell transplantation for <span class="hlt">AL</span> in North America. Patients and Methods Between 1995 and 2012, 1,536 patients with <span class="hlt">AL</span> who underwent autotransplantation at 134 centers were identified in the Center for International Blood and Marrow Transplant Research database. EM and overall survival (OS) were analyzed in three time cohorts: 1995 to 2000 (n = 140), 2001 to 2006 (n = 596), and 2007 to 2012 (n = 800). Hematologic and renal responses and factors associated with EM, relapse and/or progression, progression-free survival and OS were analyzed in more recent subgroups from 2001 to 2006 (n = 197) and from 2007 to 2012 (n = 157). Results Mortality at 30 and 100 days progressively declined over successive time periods from 11% and 20%, respectively, in 1995 to 2000 to 5% and 11%, respectively, in 2001 to 2006, and to 3% and 5%, respectively, in 2007 to 2012. Correspondingly, 5-year OS improved from 55% in 1995 to 2000 to 61% in 2001 to 2006 and to 77% in 2007 to 2012. Hematologic response to transplantation improved in the latest cohort. Renal response rate was 32%. Centers performing more than four <span class="hlt">AL</span> transplantations per year had superior survival outcomes. In the multivariable analysis, cardiac <span class="hlt">AL</span> was associated with high EM and inferior progression-free survival and OS. Autotransplantation in 2007 to 2012 and use of higher dosages of melphalan were associated with a lowered relapse risk. A Karnofsky score less than 80 and creatinine levels 2 mg/m2 or greater were associated with worsened OS. Conclusion Post-transplantation survival in <span class="hlt">AL</span> has improved, with a dramatic reduction in early post-transplantation mortality</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/24286006','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/24286006"><span>Secondary <span class="hlt">amyloidosis</span> in a patient carrying mutations in the familial Mediterranean fever (FMF) and tumour necrosis factor receptor-1 syndrome (TRAPS) genes.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Clementi, Anna; Cruz, Dinna N; Granata, Antonio; Virzì, Grazia Maria; Battaglia, Giorgio</p> <p>2013-12-01</p> <p>Secondary <span class="hlt">amyloidosis</span> (AA) is characterized by the extracellular tissue deposition of fibrils composed of fragments of an acute-phase reactant protein, serum amyloid A (SAA), due to chronic inflammatory diseases, infections and several neoplasms. AA <span class="hlt">amyloidosis</span> may also complicate several hereditary diseases, where genetic factors play a pivotal role in the expression of <span class="hlt">amyloidosis</span>. Familial Mediterranean fever (FMF) and tumour necrosis factor receptor-1 syndrome (TRAPS) are the most frequently involved. We describe a case of a 21-year-old Romanian woman who presented at the 35th week of gestation with acute abdominal pain, nausea and vomiting. The laboratory workup performed after delivery showed proteinuria in the nephrotic range and increased SAA protein. Kidney amyloid deposits were detected and genetic testing for secondary <span class="hlt">amyloidosis</span> was performed identifying two mutations, one involving the gene of FMF (MEFV), and the other involving the tumour necrosis factor receptor-1 gene (TNFRSF1A). To our knowledge, this is the first case in the literature where secondary <span class="hlt">amyloidosis</span> develops in a patient carrying mutations involving the genes of both FMF and TRAPS.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2011RJPCA..85...55K','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2011RJPCA..85...55K"><span>Peculiarities of the iron reduction mechanism in Fe-<span class="hlt">Al</span>-K <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>Krylova, A. Yu.; Chernavskii, P. A.; Lyadov, A. S.; Sagitov, S. A.; Krylova, M. V.; Khadzhiev, S. N.</p> <p>2011-01-01</p> <p>Temperature-programmed reduction was used in combination with measurements of magnetization to determine the peculiarities of iron reduction in the Fe-K-<span class="hlt">Al</span> <span class="hlt">system</span>. It was found that reduction by hydrogen proceeds with the formation of metallic iron through the stage of magnetite formation (Fe3O4); the effective activation energies are 63 and 39 kJ/mol for the I and II stages, respectively. It was shown that substituting carbon oxide for hydrogen leads to iron reduction proceeding only to the stage of magnetite formation ( E Fe 3O4 = 94 kJ/mol). The magnetite interacts with CO to produce carbide (presumably Hegge carbide Fe2C). Iron reduction in the synthesis gas occurs with the preferential participation of hydrogen or carbon dioxide, depending on the rate of temperature rise.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/26035344','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/26035344"><span>NaF-KF-<span class="hlt">Al</span>F3 <span class="hlt">System</span>: Phase Transition in K2Na<span class="hlt">Al</span>3F12 Ternary Fluoride.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Kirik, Sergei D; Zaitseva, Yulia N; Leshok, Darya Yu; Samoilo, Alexandr S; Dubinin, Petr S; Yakimov, Igor S; Simakov, Dmitry A; Gusev, Alexandr O</p> <p>2015-06-15</p> <p>Phase formation in the NaF-KF-<span class="hlt">Al</span>F3 <span class="hlt">system</span>, in the vicinity of the K2Na<span class="hlt">Al</span>3F12 composition, has been studied. The samples have been prepared by melting the starting components at 650 °C. A new phase has been revealed, which appeared to be a low-temperature form of the well-known K2Na<span class="hlt">Al</span>3F12 ternary fluoride obtained by the hydrothermal synthesis method. The high-temperature form melts at 598 °C and is stable in a narrow temperature region of about 15 deg below the melting point. Thermal analysis, high temperature X-ray diffraction, IR-spectroscopy, X-ray fluorescence, and X-ray powder diffraction crystal structure analysis have been applied to study the composition, crystal structure, and thermal properties of the low-temperature phase. The crystal structure consists of trigonal-hexagonal two-dimensional (2D) grids built from the [<span class="hlt">Al</span>F6] octahedra connected via vertices. The 2D grids have a specific wave-like conformation with a wavelength of 11.88 Å and an amplitude of 0.46 Å. There is a shift of the adjacent grids relative to each other. Because of this shift, the space between the grids changes. The shift leads to the formation of pores adapted to potassium and sodium ions. The reasons for the wave-like structure of layers are discussed. It is shown that the two polymorphic forms differ in the order of cation occupations.</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>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('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2614732','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2614732"><span>Postperovskite phase equilibria in the MgSiO3–<span class="hlt">Al</span>2O3 <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>Tsuchiya, Jun; Tsuchiya, Taku</p> <p>2008-01-01</p> <p>We investigate high-P,T phase equilibria of the MgSiO3–<span class="hlt">Al</span>2O3 <span class="hlt">system</span> 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 Rh2O3(II) phase, present calculations demonstrate that (i) dissolving <span class="hlt">Al</span>2O3 tends to decrease the postperovskite transition pressure of MgSiO3 but the effect is not significant (≈-0.2 GPa/mol% <span class="hlt">Al</span>2O3); (ii) <span class="hlt">Al</span>2O3 produces the narrow perovskite+postperovskite coexisting P,T area (≈1 GPa) for the pyrolitic concentration (x<span class="hlt">Al</span>2O3 ≈6 mol%), which is sufficiently responsible to the deep-mantle D″ seismic discontinuity; (iii) the transition would be smeared (≈4 GPa) for the basaltic <span class="hlt">Al</span>-rich composition (x<span class="hlt">Al</span>2O3 ≈20 mol%), which is still seismically visible unless iron has significant effects; and last (iv) the perovskite structure spontaneously changes to the Rh2O3(II) with increasing the <span class="hlt">Al</span> concentration involving small displacements of the Mg-site cations. PMID:19036928</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/28241672','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/28241672"><span>Structural Behaviour of Solid Solutions in the Nd<span class="hlt">Al</span>O3-SrTiO3 <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>Ohon, Natalia; Stepchuk, Roman; Blazhivskyi, Kostiantyn; Vasylechko, Leonid</p> <p>2017-12-01</p> <p>Single-phase mixed aluminates-titanates Nd1-x Sr x <span class="hlt">Al</span>1-x Ti x O3 (x = 0.3 ÷ 0.9) were prepared from stoichiometric amounts of constituent oxides Nd2O3, <span class="hlt">Al</span>2O3, TiO2 and strontium carbonate SrCO3 by solid-state reaction technique in air at 1773 K. Crystal structure parameters of Nd1-x Sr x <span class="hlt">Al</span>1-x Ti x O3 were refined by full-profile Rietveld refinement in space groups R [Formula: see text] c (x = 0.3, 0.5, 0.7 and 0.8) and Pm [Formula: see text] m (x = 0.9). Comparison of the obtained structural parameters with the literature data for the end members of the <span class="hlt">system</span> Nd<span class="hlt">Al</span>O3 and SrTiO3 revealed formation of two kinds of solid solutions Nd1-xSrx<span class="hlt">Al</span>1-xTixO3 with the cubic and rhombohedral perovskite structure. Morphotropic rhombohedral-to-cubic phase transition in Nd1-xSrx<span class="hlt">Al</span>1-xTixO3 series occurs at x = 0.84. Based on the results obtained as well as the literature data for the parent compounds, the tentative phase diagram of the Nd<span class="hlt">Al</span>O3-SrTiO3 pseudo-binary <span class="hlt">system</span> have been constructed.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2017NRL....12..148O','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2017NRL....12..148O"><span>Structural Behaviour of Solid Solutions in the Nd<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>Ohon, Natalia; Stepchuk, Roman; Blazhivskyi, Kostiantyn; Vasylechko, Leonid</p> <p>2017-02-01</p> <p>Single-phase mixed aluminates-titanates Nd1- x Sr x <span class="hlt">Al</span>1- x Ti x O3 ( x = 0.3 ÷ 0.9) were prepared from stoichiometric amounts of constituent oxides Nd2O3, <span class="hlt">Al</span>2O3, TiO2 and strontium carbonate SrCO3 by solid-state reaction technique in air at 1773 K. Crystal structure parameters of Nd1- x Sr x <span class="hlt">Al</span>1- x Ti x O3 were refined by full-profile Rietveld refinement in space groups R overline{3} c ( x = 0.3, 0.5, 0.7 and 0.8) and Pm overline{3} m ( x = 0.9). Comparison of the obtained structural parameters with the literature data for the end members of the <span class="hlt">system</span> Nd<span class="hlt">Al</span>O3 and SrTiO3 revealed formation of two kinds of solid solutions Nd1-xSrx<span class="hlt">Al</span>1-xTixO3 with the cubic and rhombohedral perovskite structure. Morphotropic rhombohedral-to-cubic phase transition in Nd1-xSrx<span class="hlt">Al</span>1-xTixO3 series occurs at x = 0.84. Based on the results obtained as well as the literature data for the parent compounds, the tentative phase diagram of the Nd<span class="hlt">Al</span>O3-SrTiO3 pseudo-binary <span class="hlt">system</span> have been constructed.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2680652','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2680652"><span>Hereditary and Sporadic Forms of Aβ-Cerebrovascular <span class="hlt">Amyloidosis</span> and Relevant Transgenic Mouse Models</span></a></p> <p><a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pmc">PubMed Central</a></p> <p>Kumar-Singh, Samir</p> <p>2009-01-01</p> <p>Cerebral amyloid angiopathy (CAA) refers to the specific deposition of amyloid fibrils in the leptomeningeal and cerebral blood vessel walls, often causing secondary vascular degenerative changes. Although many kinds of peptides are known to be deposited as vascular amyloid, amyloid-β (Aβ)-CAA is the most common type associated with normal aging, sporadic CAA, Alzheimer’s disease (AD) and Down’s syndrome. Moreover, Aβ-CAA is also associated with rare hereditary cerebrovascular <span class="hlt">amyloidosis</span> due to mutations within the Aβ domain of the amyloid precursor protein (APP) such as Dutch and Flemish APP mutations. Genetics and clinicopathological studies on these familial diseases as well as sporadic conditions have already shown that CAA not only causes haemorrhagic and ischemic strokes, but also leads to progressive dementia. Transgenic mouse models based on familial AD mutations have also successfully reproduced many of the features found in human disease, providing us with important insights into the pathogenesis of CAA. Importantly, such studies have pointed out that specific vastopic Aβ variants or an unaltered Aβ42/Aβ40 ratio favor vascular Aβ deposition over parenchymal plaques, but higher than critical levels of Aβ40 are also observed to be anti-amyloidogenic. These data would be important in the development of therapies targeting amyloid in vessels. PMID:19468344</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/1362414','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/1362414"><span>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.</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>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('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=5288517','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=5288517"><span>Bronchoscopic Diagnosis and Treatment of Primary Tracheobronchial <span class="hlt">Amyloidosis</span>: A Retrospective Analysis from China</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, Bixiu; Wang, Ge; He, Baimei</p> <p>2017-01-01</p> <p>Objective. To assess the value of bronchoscopy in the diagnosis and treatment of primary tracheobronchial <span class="hlt">amyloidosis</span> (TBA), in order to reduce misdiagnosis rates and improve prognosis. Methods. Clinical data of 107 patients with TBA reported from 1981 to 2015 in China were retrospectively analyzed for clinical features, bronchoscopic manifestations, pathologies, treatments, and outcomes. Results. 105 of 107 TBA patients were pathologically confirmed by bronchoscopy. Main bronchoscopic manifestations of TBA were single or multiple nodules and masses within tracheobronchial lumens; local or diffuse luminal stenosis and obstruction; luminal wall thickening and rigidity; rough or uneven inner luminal walls; congestion and edema of mucosa, which was friable and prone to bleeding upon touch; and so forth. 53 patients were treated with bronchoscopic interventions, like Nd-YAG laser, high-frequency electrotome cautery, freezing, resection, clamping, argon plasma coagulation (APC), microwaving, stent implantation, drug spraying, and other treatments. 51 patients improved, 1 patient worsened, and 1 died. Conclusion. Bronchoscopic biopsy is the primary means of diagnosing TBA. A variety of bronchoscopic interventions have good short-term effects on TBA. Bronchoscopy has important value in the diagnosis, severity assessment, treatment, efficacy evaluation, and prognosis of TBA. PMID:28197412</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/28081655','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/28081655"><span>Marked biochemical difference in amyloid proportion between intra- and extraocular tissues in a liver-transplanted patient with hereditary ATTR <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>Yoshinaga, Tsuneaki; Yazaki, Masahide; Kametani, Fuyuki; Sekijima, Yoshiki; Iesato, Yasuhiro; Miyahara, Teruyoshi; Tsuchiya-Suzuki, Ayako; Sano, Kenji; Higuchi, Keiichi; Ikeda, Shu-Ichi</p> <p>2017-01-13</p> <p>In order to elucidate the pathomechanism of ocular amyloid formation in a liver-transplanted patient with hereditary ATTR <span class="hlt">amyloidosis</span>, we investigated detailed biochemical features of ocular amyloid. The patient was a 49-year-old woman with V30M transthyretin (TTR) variant (p.TTRV50M), who underwent ophthalmectomy due to corneal rupture 10 years after liver transplantation (LT). The amyloid was selectively isolated from several portions in intra- and extraocular tissues using a laser microdissection (LMD) <span class="hlt">system</span> and analyzed by liquid chromatography-tandem mass spectrometry to determine the composition percentage of wild-type and variant TTR in the isolated amyloid. Biochemical analysis revealed that the amyloid consisted mainly of variant TTR in intraocular tissues with a percentage > 80%. On the contrary in the extraocular muscles, wild-type TTR was the main component of the amyloid with a percentage of ∼70%. Our data indicate that intraocular amyloid formation strongly depends on locally synthesized variant TTR and the contribution of wild-type TTR to amyloid formation is quite limited.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=3789183','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=3789183"><span>Activated microglia mediate synapse loss and short-term memory deficits in a mouse model of transthyretin-related oculoleptomeningeal <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>Azevedo, E P; Ledo, J H; Barbosa, G; Sobrinho, M; Diniz, L; Fonseca, A C C; Gomes, F; Romão, L; Lima, F R S; Palhano, F L; Ferreira, S T; Foguel, D</p> <p>2013-01-01</p> <p>Oculoleptomeningeal <span class="hlt">amyloidosis</span> (OA) is a fatal and untreatable hereditary disease characterized by the accumulation of transthyretin (TTR) amyloid within the central nervous <span class="hlt">system</span>. The mechanisms underlying the pathogenesis of OA, and in particular how amyloid triggers neuronal damage, are still unknown. Here, we show that amyloid fibrils formed by a mutant form of TTR, A25T, activate microglia, leading to the secretion of tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6) and nitric oxide. Further, we found that A25T amyloid fibrils induce the activation of Akt, culminating in the translocation of NFκB to the nucleus of microglia. While A25T fibrils were not directly toxic to neurons, the exposure of neuronal cultures to media conditioned by fibril-activated microglia caused synapse loss that culminated in extensive neuronal death via apoptosis. Finally, intracerebroventricular (i.c.v.) injection of A25T fibrils caused microgliosis, increased brain TNF-α and IL-6 levels and cognitive deficits in mice, which could be prevented by minocycline treatment. These results indicate that A25T fibrils act as pro-inflammatory agents in OA, activating microglia and causing neuronal damage. PMID:24008733</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/1988MTA....19.1645A','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/1988MTA....19.1645A"><span>Microstructural characterization of the dispersed phases in <span class="hlt">Al</span>-Ce-Fe <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>Ayer, Raghavan; Angers, L. M.; Mueller, R. R.; Scanlon, J. C.; Klein, C. F.</p> <p>1988-07-01</p> <p>Analytical electron microscopy studies were conducted on a rapidly solidified <span class="hlt">Al</span>-8.8Fe-3.7Ce alloy and arc melted buttons of aluminum rich <span class="hlt">Al</span>-Fe-Ce alloys to determine the characteristics of the metastable and equilibrium phases. The rapidly solidified alloy consisted of binary and ternary metastable phases in the as-extruded condition. The binary metastable phase was identified to be <span class="hlt">Al</span>6Fe, while the ternary metastable phases were identified to be <span class="hlt">Al</span>10Fe2Ce and <span class="hlt">Al</span>20Fe5Ce. The <span class="hlt">Al</span>20Fe5Ce was a decagonal quasicrystal while the <span class="hlt">Al</span>10Fe2Ce phase was determined to have an orthorhombic crystal structure belonging to space group Cmmm, Cmm2, or C222. Microscopy studies of RS alloy and cast buttons annealed at 700 K established the equilibrium phases to be <span class="hlt">Al</span>13Fe4, <span class="hlt">Al</span>4Ce, and an <span class="hlt">Al</span>13Fe3Ce ternary phase which was first identified in the present study. The crystal structure of the equilibrium ternary phase was determined to be orthorhombic with a Cmcm or Cmc2 space group. The details of X-ray microanalysis and convergent beam electron diffraction analysis are described.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.dtic.mil/docs/citations/ADA568146','DTIC-ST'); return false;" href="http://www.dtic.mil/docs/citations/ADA568146"><span><span class="hlt">AL-Amyloidosis</span> Presenting with Negative Congo Red Staining in the Setting of High Clinical Suspicion: A Case Report</span></a></p> <p><a target="_blank" href="https://publicaccess.dtic.mil/psm/api/service/search/search">DTIC Science & Technology</a></p> <p></p> <p>2012-01-01</p> <p>1Department of Internal Medicine, William Beaumont Army Medical Center, 5005 N Piedras Street, Building 7777, 9th floor East, El Paso, TX 79920, USA...2Department of Nephrology, William Beaumont Army Medical Center, 5005 N Piedras Street, Building 7777, 12th floor, El Paso, TX 79920, USA 3Propath...NUMBER 7. PERFORMING ORGANIZATION NAME(S) AND ADDRESS(ES) William Beaumont Army Medical Center,Department of Nephrology,5005 N Piedras Street,Building</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/pages/biblio/1340510-sensitivity-polycrystal-local-plasticity-slip-system-kinematic-hardening-laws-al-t6','SCIGOV-DOEP'); return false;" href="https://www.osti.gov/pages/biblio/1340510-sensitivity-polycrystal-local-plasticity-slip-system-kinematic-hardening-laws-al-t6"><span>Sensitivity of polycrystal [local] plasticity to slip <span class="hlt">system</span> kinematic hardening laws for <span class="hlt">Al</span> 7075-T6</span></a></p> <p><a target="_blank" href="http://www.osti.gov/pages">DOE PAGES</a></p> <p>Hennessey, Conor; Castelluccio, Gustavo M.; McDowell, David L.</p> <p>2017-01-22</p> <p>The prediction of formation and early growth of microstructurally small fatigue cracks requires use of constitutive models that accurately estimate local states of stress, strain, and cyclic plastic strain. However, few research efforts have attempted to systematically consider the sensitivity of overall cyclic stress-strain hysteresis and higher order mean stress relaxation and plastic strain ratcheting responses introduced by the slip <span class="hlt">system</span> back-stress formulation in crystal plasticity, even for face centered cubic (FCC) crystal <span class="hlt">systems</span>. This paper explores the performance of two slip <span class="hlt">system</span> level kinematic hardening models using a finite element crystal plasticity implementation as a User Material Subroutine (UMAT)more » within ABAQUS, with fully implicit numerical integration. The two kinematic hardening formulations aim to reproduce the cyclic deformation of polycrystalline <span class="hlt">Al</span> 7075-T6 in terms of both macroscopic cyclic stress-strain hysteresis loop shape, as well as ratcheting and mean stress relaxation under strain- or stress-controlled loading with mean strain or stress, respectively. The first formulation is an Armstrong-Frederick type hardening-dynamic recovery law for evolution of the back stress. This approach is capable of reproducing observed deformation under completely reversed uniaxial loading conditions, but overpredicts the rate of cyclic ratcheting and associated mean stress relaxation. The second formulation corresponds to a multiple back stress Ohno-Wang type hardening law with nonlinear dynamic recovery. The adoption of this back stress evolution law greatly improves the capability to model experimental results for polycrystalline specimens subjected to cycling with mean stress or strain. As a result, the relation of such nonlinear dynamic recovery effects are related to slip <span class="hlt">system</span> interactions with dislocation substructures.« less</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/pages/biblio/1340510-sensitivity-polycrystal-plasticity-slip-system-kinematic-hardening-laws-al-t6','SCIGOV-DOEP'); return false;" href="https://www.osti.gov/pages/biblio/1340510-sensitivity-polycrystal-plasticity-slip-system-kinematic-hardening-laws-al-t6"><span>Sensitivity of polycrystal plasticity to slip <span class="hlt">system</span> kinematic hardening laws for <span class="hlt">Al</span> 7075-T6</span></a></p> <p><a target="_blank" href="http://www.osti.gov/pages">DOE PAGES</a></p> <p>Hennessey, Conor; Castelluccio, Gustavo M.; McDowell, David L.</p> <p>2017-02-01</p> <p>The prediction of formation and early growth of microstructurally small fatigue cracks requires use of constitutive models that accurately estimate local states of stress, strain, and cyclic plastic strain. However, few research efforts have attempted to systematically consider the sensitivity of overall cyclic stress-strain hysteresis and higher order mean stress relaxation and plastic strain ratcheting responses introduced by the slip <span class="hlt">system</span> back-stress formulation in crystal plasticity, even for face centered cubic (FCC) crystal <span class="hlt">systems</span>. This paper explores the performance of two slip <span class="hlt">system</span> level kinematic hardening models using a finite element crystal plasticity implementation as a User Material Subroutine (UMAT)more » within ABAQUS, with fully implicit numerical integration. The two kinematic hardening formulations aim to reproduce the cyclic deformation of polycrystalline <span class="hlt">Al</span> 7075-T6 in terms of both macroscopic cyclic stress-strain hysteresis loop shape, as well as ratcheting and mean stress relaxation under strain- or stress-controlled loading with mean strain or stress, respectively. The first formulation is an Armstrong-Frederick type hardening-dynamic recovery law for evolution of the back stress. This approach is capable of reproducing observed deformation under completely reversed uniaxial loading conditions, but overpredicts the rate of cyclic ratcheting and associated mean stress relaxation. The second formulation corresponds to a multiple back stress Ohno-Wang type hardening law with nonlinear dynamic recovery. The adoption of this back stress evolution law greatly improves the capability to model experimental results for polycrystalline specimens subjected to cycling with mean stress or strain. As a result, the relation of such nonlinear dynamic recovery effects are related to slip <span class="hlt">system</span> interactions with dislocation substructures.« less</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/scitech/biblio/22412999','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/22412999"><span>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://adsabs.harvard.edu/abs/2013NIMPA.721...21S','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2013NIMPA.721...21S"><span>Thick target neutron yield from 145 MeV 19F+27<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>Sunil, C.; Bandyopadhyay, T.; Nandy, M.; Suman, Vitisha; Paul, S.; Nanal, V.; Pillay, R. G.; Sarkar, P. K.</p> <p>2013-09-01</p> <p>The double differential neutron energy distribution has been measured for the 19F+27<span class="hlt">Al</span> <span class="hlt">system</span> at 145 MeV projectile energy. The time of flight technique was used to measure the energy while pulse shape discrimination has been used to separate the neutrons from photons. The results are compared with the statistical nuclear reaction model codes PACE and EMPIRE. The PACE code appears to predict the slope and the end point energy of the experimental spectra fairly well but over predicts the values. The slope obtained from the EMPIRE calculations appears to be harder while the values being closer to the experimental results. The yield from the Hauser-Feshbach based compound nucleus model calculations agree reasonably well with the experimental results at the backward angles but not in the forward directions. The energy integrated angular distribution from 145 MeV projectiles show an enhanced emission in the forward angles compared to the similar results from 110 MeV projectiles. This analysis suggests some contribution from the pre-equilibrium emissions from the <span class="hlt">system</span> at the higher projectile energy.</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://adsabs.harvard.edu/abs/2015JAP...117d4303M','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2015JAP...117d4303M"><span>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://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Malyshev, K. V.</p> <p>2015-01-01</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 F011(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('https://www.ncbi.nlm.nih.gov/pubmed/28375369','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/28375369"><span>Comparison of degradation and recaesiation between GaAs and <span class="hlt">Al</span>GaAs photocathodes in an unbaked vacuum <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>Feng, Cheng; Zhang, Yijun; Shi, Feng; Qian, Yunsheng; Cheng, Hongchang; Zhang, Junju; Liu, Xinxin; Zhang, Xiang</p> <p>2017-03-20</p> <p>The lifetime and reliability of a photocathode during operation are always raised problems and the photocathode performance depends on the vacuum condition. With the purpose of investigating the stability and reliability of a GaAs-based photocathode in a harsher vacuum environment, reflection-mode exponential-doped GaAs and <span class="hlt">Al</span>GaAs photocathodes are metalorganic vapor-phase epitaxial grown and then (Cs, O) activated inside an unbaked vacuum chamber. The degraded photocurrents are compared after activation and recaesiations between GaAs and <span class="hlt">Al</span>GaAs photocathdoes under illumination with an equal initial photocurrent and an equal optical flux, respectively. It is found that the performance on degradation and recaesiations between GaAs and <span class="hlt">Al</span>GaAs photocathodes are different. In the unbaked vacuum <span class="hlt">system</span>, the stability of an <span class="hlt">Al</span>GaAs photocathode after (Cs, O) activation is always better than that of a GaAs photocathode. After multiple recaesiations, the photocurrent decay curves of the <span class="hlt">Al</span>GaAs photocathode are nearly coincident, which means a nearly constant operational lifetime. Moreover, operational lifetime of an <span class="hlt">Al</span>GaAs photocathode is longer than that of a GaAs photocathode, which further illuminates that <span class="hlt">Al</span>GaAs photocathodes are superior to GaAs photocathodes in stability and repeatability under markedly harsher vacuum conditions.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/27956278','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/27956278"><span>The M694I/M694I genotype: A genetic risk factor of AA-<span class="hlt">amyloidosis</span> in a group of Algerian patients with familial Mediterranean fever.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Ait-Idir, Djouher; Djerdjouri, Bahia; Bouldjennet, Faiza; Taha, Rowaida Z; El-Shanti, Hatem; Sari-Hamidou, Rawda; Khellaf, Ghalia; Benmansour, Mustapha; Benabadji, Mohamed; Haddoum, Farid</p> <p>2017-03-01</p> <p>Familial Mediterranean fever (FMF, OMIM 249100) is the most common hereditary fever, resulting from mutations in MEFV. FMF is characterized by episodic febrile attacks and polyserositis. Renal AA-<span class="hlt">amyloidosis</span> is a major complication, which often leads to end-stage renal disease in untreated patients. The data about the renal AA-<span class="hlt">amyloidosis</span> secondary to FMF are scarce in North African countries and non-existent in Algeria. We aimed to investigate the MEFV mutations associated with this complication in an Algerian patient cohort. Molecular analysis included 28 unrelated Algerian FMF patients with ascertained <span class="hlt">amyloidosis</span>, 23 of them were symptomatic and 5 were asymptomatic. For this study, a group of 20 FMF patients without renal <span class="hlt">amyloidosis</span> were selected as controls according to their age, disease onset and disease duration. The mutations were detected by sequencing exon 10 of MEFV. A total of 87.5% (49/56) mutant alleles were identified in 27/28 analyzed patients; p.M694I was predominant and appeared with an allele frequency of 62.5%, followed by p.M694V (17.85%), p.M680I (5.35%) and p.I692Del (1.78%). Remarkably, only p.M694I mutation was observed among the asymptomatic patients. The M694I/M694I genotype, identified in 14/27 (52%) patients, was significantly associated with the development of <span class="hlt">amyloidosis</span> compared to group of controls (p = 0.022). This study did not link the M694V/M694V genotype to the renal complication despite the fact that it has been observed only in the patients with <span class="hlt">amyloidosis</span> (3/27; 11%) (p = 0.349). The association of other identified genotypes to this complication was statistically insignificant. The progression of <span class="hlt">amyloidosis</span> led to end-stage renal disease in 14 patients with 6 deaths. This study shows that p.M694I homozygosity is a potential genetic risk factor for the development of renal AA-<span class="hlt">amyloidosis</span> in Algerian FMF patients.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2017JEMat.tmp...19A','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2017JEMat.tmp...19A"><span>Effects of Codoping with Ga and P on Thermoelectric Properties of Ba8<span class="hlt">Al</span>16Si30 Clathrate <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>Anno, Hiroaki; Ueda, Takahiro; Okamoto, Kazuya</p> <p>2017-01-01</p> <p>We have investigated the effects of Codoping With Ga and P on the thermoelectric properties of the Ba8<span class="hlt">Al</span>16Si30 clathrate <span class="hlt">system</span>, attempting to optimize the carrier concentration. The elastic properties, which are important for design of thermoelectric devices, were investigated by ultrasonic testing. Ga/P-codoped specimens with nominal compositions Ba8<span class="hlt">Al</span>16Ga x Si30-2x P x (x = 1.0, 1.5, 2.0) were prepared by arc melting and spark plasma sintering and their Seebeck coefficient, electrical conductivity, and thermal conductivity were measured. Analytical studies revealed that the total content of <span class="hlt">Al</span> and Ga, expressed as atoms per formula unit, increased to 15.65 at nominal x = 2.0, exceeding the maximum content (y = 15.16) of <span class="hlt">Al</span> for the Ba8<span class="hlt">Al</span> y Si46-y clathrate <span class="hlt">system</span>. Ultrasonic tests determined the Young's modulus, shear modulus, bulk modulus, and Poisson's ratio to be 102.55 GPa, 40.14 GPa, 76.85 GPa, and 0.2775, respectively, for Ba8<span class="hlt">Al</span>16Ga x Si30-2x P x (x = 2.0). The Hall carrier concentration decreased from ˜1.0 × 1021 cm-3 for Ba8<span class="hlt">Al</span> y Si46-y to ˜6.3 × 1020 cm-3 for Ba8<span class="hlt">Al</span>16Ga x Si30-2x P x (x = 2.0), suggesting that Ga/P codoping may be useful for tuning the carrier concentration. The value of the Seebeck coefficient at ˜320 K increased from -46 μV K-1 for Ba8<span class="hlt">Al</span> y Si46-y to -67 μV K-1 for Ba8<span class="hlt">Al</span>16Ga x Si30-2x P x (x = 2.0). The dimensionless thermoelectric figure␣of merit ZT at 900 K improved from ˜0.4 for Ba8<span class="hlt">Al</span> y Si46-y to ˜0.47 for Ba8<span class="hlt">Al</span>16Ga x Si30-2x P x (x = 2.0).</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2017JEMat..46.1730A','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2017JEMat..46.1730A"><span>Effects of Codoping with Ga and P on Thermoelectric Properties of Ba8<span class="hlt">Al</span>16Si30 Clathrate <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>Anno, Hiroaki; Ueda, Takahiro; Okamoto, Kazuya</p> <p>2017-03-01</p> <p>We have investigated the effects of Codoping With Ga and P on the thermoelectric properties of the Ba8<span class="hlt">Al</span>16Si30 clathrate <span class="hlt">system</span>, attempting to optimize the carrier concentration. The elastic properties, which are important for design of thermoelectric devices, were investigated by ultrasonic testing. Ga/P-codoped specimens with nominal compositions Ba8<span class="hlt">Al</span>16Ga x Si30-2 x P x ( x = 1.0, 1.5, 2.0) were prepared by arc melting and spark plasma sintering and their Seebeck coefficient, electrical conductivity, and thermal conductivity were measured. Analytical studies revealed that the total content of <span class="hlt">Al</span> and Ga, expressed as atoms per formula unit, increased to 15.65 at nominal x = 2.0, exceeding the maximum content ( y = 15.16) of <span class="hlt">Al</span> for the Ba8<span class="hlt">Al</span> y Si46- y clathrate <span class="hlt">system</span>. Ultrasonic tests determined the Young's modulus, shear modulus, bulk modulus, and Poisson's ratio to be 102.55 GPa, 40.14 GPa, 76.85 GPa, and 0.2775, respectively, for Ba8<span class="hlt">Al</span>16Ga x Si30-2 x P x ( x = 2.0). The Hall carrier concentration decreased from ˜1.0 × 1021 cm-3 for Ba8<span class="hlt">Al</span> y Si46- y to ˜6.3 × 1020 cm-3 for Ba8<span class="hlt">Al</span>16Ga x Si30-2 x P x ( x = 2.0), suggesting that Ga/P codoping may be useful for tuning the carrier concentration. The value of the Seebeck coefficient at ˜320 K increased from -46 μV K-1 for Ba8<span class="hlt">Al</span> y Si46- y to -67 μV K-1 for Ba8<span class="hlt">Al</span>16Ga x Si30-2 x P x ( x = 2.0). The dimensionless thermoelectric figure of merit ZT at 900 K improved from ˜0.4 for Ba8<span class="hlt">Al</span> y Si46- y to ˜0.47 for Ba8<span class="hlt">Al</span>16Ga x Si30-2 x P x ( x = 2.0).</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/27126074','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/27126074"><span>Familial mutations in fibrinogen Aα (FGA) chain identified in renal <span class="hlt">amyloidosis</span> increase in vitro amyloidogenicity of FGA fragment.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Sivalingam, Vishwanath; Patel, Basant K</p> <p>2016-08-01</p> <p>Amyloidoses are clinical disorders where deposition of β-sheet rich, misfolded protein aggregates called amyloid occurs in vital organs like brain, kidney, liver or heart etc. Aggregation of several proteins such as immunoglobulin light chain, fibrinogen Aα chain (FGA) and lysozyme have been found to be associated with renal <span class="hlt">amyloidosis</span>. Fibrinogen <span class="hlt">amyloidosis</span> (AFib) is predominantly familial and is associated with the deposition of mutant FGA amyloid, primarily in kidneys. Over ten substitution and frame-shift mutations in FGA have been identified from AFib patients. Whether wild-type FGA is also involved in AFib is yet unknown. The affected tissues from AFib patients usually show ∼10 kDA peptide from C-terminal 80 amino acid residues of mutant FGA. Notably, this region also encompasses all known disease-related mutations. Whether these point mutations increase the amyloidogenicity of FGA leading to disease progression, have not been studied yet. Here, we have investigated the role of two disease-related mutations in affecting amyloidogenic propensity of an FGA(496-581) fragment. We found that at physiological pH, the wild-type FGA(496-581) fragment remains monomeric, whereas its E540V mutant forms amyloid-like fibrils as observed by AFM. Also, FGA(496-581) harbouring another familial mutation, R554L, converts in vitro into globular, β-sheet rich aggregates, showing amyloid-like properties. These findings suggest that familial mutations in FGA may have role in renal <span class="hlt">amyloidosis</span> via enhanced amyloid formation.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/1997JSSCh.131...24T','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/1997JSSCh.131...24T"><span>An XRD and Electron Diffraction Study of Cristobalite-Related Phases in the Na<span class="hlt">Al</span>O 2-Na<span class="hlt">Al</span>SiO 4<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>Thompson, John G.; Melnitchenko, Alexandra; Palethorpe, Stephen R.; Withers, Ray L.</p> <p>1997-06-01</p> <p>A detailed study of the sodium aluminate-carnegieite <span class="hlt">system</span> (Na2-x<span class="hlt">Al</span>2-xSixO4, 0≤x≤1) at temperatures between 800 and 1300°C has revealed five previously unreported phases. All of the new phases can be described as modulated variants of an underlyingβ-cristobalite parent structure. Atx≈0.05 theγ-Na<span class="hlt">Al</span>O2-type structure (P41212,a=1/2ap,c=cp) (p=parent) is stabilized to room-temperature; atx≈0.2-0.45 an orthorhombic (Pbca,a=2ap,b=2bp,c=1/2cp) KGaO2-type structure is obtained, except atx≈0.35 where a new tetragonal (P41212,a=2ap,c=cp) phase is observed; atx≈0.5-0.6 a new cubic (P213,a=2ap) phase is obtained; atx≈0.7-0.9 a new orthorhombic (Pc21b,a=2ap,b=2bp,c=2cp) phase is obtained. XRD and electron diffraction data, refined unit cell dimensions, and the phase relationships at 1300°C are presented. The proposed space group symmetries are based on observed extinction conditions and constraints provided by a modulated structure description of the new phases. The materials are prepared by gel synthesis followed by solid state reaction in air.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/17237937','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/17237937"><span>Intraneuronal Abeta immunoreactivity is not a predictor of brain <span class="hlt">amyloidosis</span>-beta or neurofibrillary degeneration.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Wegiel, Jerzy; Kuchna, Izabela; Nowicki, Krzysztof; Frackowiak, Janusz; Mazur-Kolecka, Bozena; Imaki, Humi; Wegiel, Jarek; Mehta, Pankaj D; Silverman, Wayne P; Reisberg, Barry; Deleon, Mony; Wisniewski, Thomas; Pirttilla, Tuula; Frey, Harry; Lehtimäki, Terho; Kivimäki, Tarmo; Visser, Frank E; Kamphorst, Wouter; Potempska, Anna; Bolton, David; Currie, Julia R; Miller, David L</p> <p>2007-04-01</p> <p>Amyloid beta (Abeta) immunoreactivity in neurons was examined in brains of 32 control subjects, 31 people with Down syndrome, and 36 patients with sporadic Alzheimer's disease to determine if intraneuronal Abeta immunoreactivity is an early manifestation of Alzheimer-type pathology leading to fibrillar plaque formation and/or neurofibrillary degeneration. The appearance of Abeta immunoreactivity in neurons in infants and stable neuron-type specific Abeta immunoreactivity in a majority of brain structures during late childhood, adulthood, and normal aging does not support this hypothesis. The absence or detection of only traces of reaction with antibodies against 4-13 aa and 8-17 aa of Abeta in neurons indicated that intraneuronal Abeta was mainly a product of alpha- and gamma-secretases (Abeta(17-40/42)). The presence of N-terminally truncated Abeta(17-40) and Abeta(17-42) in the control brains was confirmed by Western blotting and the identity of Abeta(17-40) was confirmed by mass spectrometry. The prevalence of products of alpha- and gamma -secretases in neurons and beta- and gamma-secretases in plaques argues against major contribution of Abeta-immunopositive material detected in neuronal soma to amyloid deposit in plaques. The strongest intraneuronal Abeta(17-42) immunoreactivity was observed in structures with low susceptibility to fibrillar Abeta deposition, neurofibrillary degeneration, and neuronal loss compared to areas more vulnerable to Alzheimer-type pathology. These observations indicate that the intraneuronal Abeta immunoreactivity detected in this study is not a predictor of brain <span class="hlt">amyloidosis</span> or neurofibrillary degeneration. The constant level of Abeta immunoreactivity in structures free from neuronal pathology during essentially the entire life span suggests that intraneuronal amino-terminally truncated Abeta represents a product of normal neuronal metabolism.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/1986GeCoA..50.1911B','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/1986GeCoA..50.1911B"><span>Equilibrium aluminium hydroxo-oxalate phases during initial clay formation; H +-<span class="hlt">Al</span> 3+-oxalic acid-Na + <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>Bilinski, Halka; Horvath, Laszlo; Ingri, Nils; Sjöberg, Staffan</p> <p>1986-09-01</p> <p>The conditions necessary for initial clay formation have been studied in different model <span class="hlt">systems</span> comprising different organic acids besides Si and <span class="hlt">Al</span>. In the present paper the solid phases and the precipitation boundary characterizing the subsystem H +-<span class="hlt">Al</span> 3+-oxalic acid (H 2L) are discussed. pH and tyndallometric measurements were performed in an ionic medium of 0.6 M Na(Cl) at 25 °C. The two phases <span class="hlt">Al</span> 3(OH) 7(C 2O 4) · 3H 2O (phase I) and Na<span class="hlt">Al</span>(OH) 2(C 2O 4) · 3H 2O (phase II) determine the precipitation boundary. The following formation constants for the two phases were deduced: lgβ1 = lg([ <span class="hlt">Al</span>3+] -3[ H2C2O4] -1[ H+] 9 = -21.87 ± 0.08 and lgβ11 = lg([ <span class="hlt">Al</span>3+] -1[ H2C2O4] -1[ H+] 4 = -5.61 ± 0.06. Phase I exists in the range [ <span class="hlt">Al</span>] tot≥ 10 -4.4moldm-3,[ H2C2O4] tot ≥ 10 -4.9moldm-3 and at pH < 6.8, thus being a possible precipitate in oxalic-rich natural waters. The more soluble sodium phase is unlikely to exist in natural waters. The two phases are metastable relative to crystalline gibbsite and may be considered as the first precipitation step in the transition from aqueous <span class="hlt">Al</span> oxalates down to stable <span class="hlt">Al</span> hydroxide. Model calculations illustrating these competing hydrolysis-complexation reactions are discussed in terms of predominance and speciation diagrams. The solid phases have been characterized by X-ray analysis of powders, TGA and IR spectra, and tentative structures are proposed. Phase I seems to be an octahedral layer structure, in which 3/5 of the octahedral sites between two close packed oxygen sheets are occupied by <span class="hlt">Al</span> 3+ and the oxalate ion acts as a bridge ligand between two aluminium atoms. Phase II forms a more open sheet structure and has ion exchange properties. Powder data for a phase crystallized from the studied solution after a year are also presented. This phase, Na 4<span class="hlt">Al</span> 2(OH) 2(C 2O 4) 4 · 10H 2O, supports the results from the equilibrium analysis of recent solution data by SJöBERG and ÖHMAN (1985), who have found the dinuclear</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/scitech/biblio/565405','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/565405"><span>Observation, control, and modal analysis of longitudinal coupled-bunch instabilities in the <span class="hlt">ALS</span> via a digital feedback <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Fox, J.D.; Claus, R.; Hindi, H.; Linscott, I.; Prabhakar, S.; Ross, W.; Teytelman, D.; Drago, A.; Serio, M.; Byrd, J.; Corlett, J.; Stover, G.</p> <p>1997-01-01</p> <p>The operation of a longitudinal multibunch damping <span class="hlt">system</span> using digital signal processing (DSP) techniques is shown via measurements from the Lawrence Berkeley Laboratory (LBL) Advanced Light Source (<span class="hlt">ALS</span>). The feedback <span class="hlt">system</span> (developed for use by PEP-II, <span class="hlt">ALS</span>, and DA{Phi}NE) uses a parallel array of signal processors to implement a bunch-by-bunch feedback <span class="hlt">system</span> for sampling rates up to 500 MHz. The programmable DSP <span class="hlt">system</span> allows feedback control as well as accelerator diagnostics. A diagnostic technique is illustrated which uses the DSP <span class="hlt">system</span> to excite and then damp the beam. The resulting 12-ms time domain transient is Fourier analyzed to provide the simultaneous measurement of growth rates and damping rates of all unstable coupled-bunch beam modes. {copyright} {ital 1997 American Institute of Physics.}</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2013JaJAP..52hJF07D','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2013JaJAP..52hJF07D"><span>Enhanced Capability of Photoelectrochemical CO2 Conversion <span class="hlt">System</span> Using an <span class="hlt">Al</span>GaN/GaN Photoelectrode</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Deguchi, Masahiro; Yotsuhashi, Satoshi; Hashiba, Hiroshi; Yamada, Yuka; Ohkawa, Kazuhiro</p> <p>2013-08-01</p> <p>We report significantly improved photosynthesis <span class="hlt">system</span> based on <span class="hlt">Al</span>GaN/GaN photochemical process. The resultant energy conversion efficiency is 0.13% which is the same level as that of real plants. The capability of this <span class="hlt">system</span> is enhanced by high cathode potential due to the reduction of energy loss while utilizing the piezoelectric effect in the <span class="hlt">Al</span>GaN/GaN heterostructure. The Faradaic efficiency of the CO2 conversion to organic materials is enhanced, accompanied by an increment in photocurrent by modification of the <span class="hlt">Al</span>GaN/GaN photoelectrode structure and electrolytes. Furthermore, reaction products such as C2H4 and C2H5OH are generated by light illumination alone.</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>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/servlets/purl/1221954','SCIGOV-STC'); return false;" href="http://www.osti.gov/scitech/servlets/purl/1221954"><span>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('https://www.osti.gov/scitech/biblio/1130193','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/1130193"><span>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('http://hdl.handle.net/2060/20060051711','NASA-TRS'); return false;" href="http://hdl.handle.net/2060/20060051711"><span>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> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.gpo.gov/fdsys/pkg/FR-2010-10-01/pdf/2010-24624.pdf','FEDREG'); return false;" href="https://www.gpo.gov/fdsys/pkg/FR-2010-10-01/pdf/2010-24624.pdf"><span>75 FR 60820 - United States v. Adobe <span class="hlt">Systems</span>, Inc., et <span class="hlt">al</span>.; Proposed Final Judgment and Competitive Impact...</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>2010-10-01</p> <p>... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF JUSTICE Antitrust Division United States v. Adobe <span class="hlt">Systems</span>, Inc., et <span class="hlt">al</span>.; Proposed Final Judgment and Competitive Impact Statement Notice is hereby given pursuant to the Antitrust Procedures and Penalties Act, 15 U.S.C. 16(b)-(h), that a proposed Final...</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>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('http://adsabs.harvard.edu/abs/2009PhRvB..80t5412D','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2009PhRvB..80t5412D"><span>Complex metallic surface phases in the <span class="hlt">Al</span>/Cu(111) <span class="hlt">system</span>: An experimental and computational study</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Duguet, T.; Gaudry, E.; Deniozou, T.; Ledieu, J.; de Weerd, M. C.; Belmonte, T.; Dubois, J. M.; Fournée, V.</p> <p>2009-11-01</p> <p>The growth of complex intermetallics as surface alloys is investigated by annealing <span class="hlt">Al</span> thin films deposited on Cu(111) substrate in ultrahigh vacuum. Already at room temperature, the large lattice mismatch between <span class="hlt">Al</span> and Cu results in interfacial intermixing. Upon annealing, various phases are formed by diffusion depending on the thickness of the <span class="hlt">Al</span> films and the annealing temperature. The surface structures are characterized by scanning tunneling microscopy, low-energy electron diffraction, and x-ray photoelectron spectroscopy. Three different superlattice phases are identified as well as the complex Hume-Rothery γ-<span class="hlt">Al</span>4Cu9 phase. The epitaxial relationships between the surface phases and the Cu(111) substrate are determined. We further investigate the electronic structure of the γ phase by density functional calculations. Experimental valence bands are compared to calculated density of states and simulated STM images are used to identify possible bulk planes appearing as surface termination.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2008RuMet2008...46P','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2008RuMet2008...46P"><span>Rare-earth metals (REMs) in nickel aluminide-based alloys: I. Physicochemical laws of interaction in the Ni-<span class="hlt">Al</span>-REM and Ni x <span class="hlt">Al</span> y -REM-AE (alloying element) <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>Povarova, K. B.; Kazanskaya, N. K.; Drozdov, A. A.; Morozov, A. E.</p> <p>2008-02-01</p> <p>The data on the Ni-<span class="hlt">Al</span>- R ( R = REM Sc, Y, La, lanthanides) binary and ternary <span class="hlt">systems</span> and the interactions of three rare-earth metals (yttrium, lanthanum, cerium) with the main alloying elements (Ti (Zr, Hf), Cr (Mo, W) that are introduced into Ni3<span class="hlt">Al</span>-based VKNA alloys are analyzed. The binary aluminides of REMs in the Ni-<span class="hlt">Al</span>- R ternary <span class="hlt">systems</span> are shown to be in equilibrium with neither Ni<span class="hlt">Al</span> nor Ni3<span class="hlt">Al</span>. The solid solution of aluminum in RNi5, which penetrates deep into these ternary <span class="hlt">systems</span>, is the most stable phase in equilibrium with Ni3<span class="hlt">Al</span>. In the Ni<span class="hlt">Al</span> (Ni3<span class="hlt">Al</span>)-AE- R <span class="hlt">systems</span>, REM precipitation (segregation) on various defects and interfaces in nickel aluminides is likely to be the most probable, and REMs are thought to interact with the most active impurities in real alloys (C, O, N), since REMs have a large atomic radius and, thus, are virtually undissolved in nickel, aluminum, and nickel aluminides.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.dtic.mil/docs/citations/ADA575213','DTIC-ST'); return false;" href="http://www.dtic.mil/docs/citations/ADA575213"><span>Ignition and Combustion Characteristics of Nanoscale <span class="hlt">Al</span>/AgIO3: A Potential Energetic Biocidal <span class="hlt">System</span></span></a></p> <p><a target="_blank" href="https://publicaccess.dtic.mil/psm/api/service/search/search">DTIC Science & Technology</a></p> <p></p> <p>2011-01-01</p> <p>reaction of <span class="hlt">Al</span>/AgIO3 thermites for potential use in biocidal applications . Rapid-heating wire experiments were performed to measure the ignition...OF: This work investigates the ignition and reaction of <span class="hlt">Al</span>/AgIO3 thermites for potential use in biocidal applications . Rapid-heating wire experiments...were performed to measure the ignition temperature and investigate the thermal decomposition of the oxidizer using a T-Jump/TOF Mass Spectrometer</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('https://www.ncbi.nlm.nih.gov/pubmed/25736960','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/25736960"><span>Long-term kinetics of AA <span class="hlt">amyloidosis</span> and effects of inflammatory restimulation after disappearance of amyloid depositions in mice.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Muhammad, N; Murakami, T; Inoshima, Y; Ishiguro, N</p> <p>2015-07-01</p> <p>Amyloid A (AA) <span class="hlt">amyloidosis</span> is characterized by extracellular pathogenic deposition of insoluble fibril protein in various body organs. Deposited amyloid generally remains in a variety of organs for long periods, but its disappearance has been reported after the precursor protein is diminished. The kinetics of AA deposition are not completely understood and, in particular, the roles of cells and cytokines in the deposition and clearance of amyloid remain unclear. In this study, we investigated the disappearance of amyloid depositions in mice over a 1-year period. AA <span class="hlt">amyloidosis</span> was induced experimentally in mice by injecting amyloid-enhancing factor (AEF) and silver nitrate. Mice were killed at different time-points to examine the occurrence and disappearance of amyloid depositions. Maximum levels of amyloid depositions were observed at 20 days after inoculation. Clearance of amyloid depositions was observed from the 40th day onwards, with only minute traces of amyloid present by 240 days. A second inflammatory stimulus consisting of AEF and silver nitrate was given at 330 or 430 days, after amyloid depositions had disappeared almost completely. After that, serum amyloid A was overproduced and redeposition of amyloid was observed, indicating that all mice were primed for aggressive amyloid depositions. After administration of the inflammatory stimuli, the proinflammatory environment was found to have increased levels of interleukin (IL)-6, while anti-inflammatory conditions were established by IL-10 as regression of amyloid deposition occurred. These results suggest that the proinflammatory and anti-inflammatory status have key roles in both amyloid deposition and clearance.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/3599910','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/3599910"><span>Characterization of tissue and plasma glycosaminoglycans during experimental AA <span class="hlt">amyloidosis</span> and acute inflammation. Qualitative and quantitative analysis.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Snow, A D; Kisilevsky, R; Stephens, C; Anastassiades, T</p> <p>1987-06-01</p> <p>Qualitative and quantitative methods were used to determine changes in glycosaminoglycans (GAGs) in the spleen and plasma during initial stages of experimental <span class="hlt">amyloidosis</span> and acute inflammation. GAG deposition in the spleen during the early stages of <span class="hlt">amyloidosis</span> consists of a 16-fold heparin and heparan sulfate increase. Though splenic weights do increase during protracted inflammation only minor changes arise in splenic GAGs in the absence of amyloid deposition. An overall increase in plasma GAGs, consisting of a 4.5-fold chondroitin-4-sulphate increase, occurred at the time of GAG deposition in the tissues (spleen, liver) and probably accounts for the minor GAG changes seen in the spleen during inflammation. The time course of splenic heparin/heparan sulfate increase during amyloid deposition coincides with the histochemical changes previously described. Plasma GAG changes follow a pattern similar to that of acute phase protein reactants. The results suggest that GAG metabolism, in particular heparin/heparan sulfate, are intimately involved in the process of AA amyloidogenesis.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1683143','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1683143"><span>Finnish type of familial <span class="hlt">amyloidosis</span>: cosegregation of Asp187----Asn mutation of gelsolin with the disease in three large families.</span></a></p> <p><a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pmc">PubMed Central</a></p> <p>Hiltunen, T; Kiuru, S; Hongell, V; Heliö, T; Palo, J; Peltonen, L</p> <p>1991-01-01</p> <p>Familial <span class="hlt">amyloidosis</span> of Finnish type (FAF) is one of the familial amyloidotic polyneuropathy (FAP) syndromes, a group of inherited disorders characterized by extracellular accumulation of amyloid and by clinical symptoms and signs of polyneuropathy. FAF, an autosomal dominant trait, belongs to those rare monogenic disorders which occur with increased frequency in the Finnish population: only single FAF cases have been reported from other populations. In most types of FAP syndromes the accumulating protein is a transthyretin variant. However, recent evidence has suggested that the amyloid peptides in FAF are related to gelsolin, an actin modulating protein. The gelsolin fragments isolated from at least one patient with <span class="hlt">amyloidosis</span> have been reported to have an amino acid substitution, with asparagine replacing aspartic acid at position 187 of the plasma gelsolin. In this study allele-specific oligonucleotides were used to analyze three large FAF families with multiple affected individuals as well as healthy family members. We found the corresponding G-A mutation in nucleotide 654 of the plasma gelsolin gene to cosegregate with the disease. The result was confirmed by sequencing and strongly suggests that the mutation has caused all the FAF cases of these families. Since the disease is clustered in restricted areas on the southern coast of Finland, this mutation most probably causes the majority, if not all, of FAF cases in Finland. Images Figure 2 PMID:1652889</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4428403','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4428403"><span>Chaperone Nanobodies Protect Gelsolin Against MT1-MMP Degradation and Alleviate Amyloid Burden in the Gelsolin <span class="hlt">Amyloidosis</span> Mouse Model</span></a></p> <p><a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pmc">PubMed Central</a></p> <p>Van Overbeke, Wouter; Verhelle, Adriaan; Everaert, Inge; Zwaenepoel, Olivier; Vandekerckhove, Joël; Cuvelier, Claude; Derave, Wim; Gettemans, Jan</p> <p>2014-01-01</p> <p>Gelsolin <span class="hlt">amyloidosis</span> is an autosomal dominant incurable disease caused by a point mutation in the GSN gene (G654A/T), specifically affecting secreted plasma gelsolin. Incorrect folding of the mutant (D187N/Y) second gelsolin domain leads to a pathological proteolytic cascade. D187N/Y gelsolin is first cleaved by furin in the trans-Golgi network, generating a 68 kDa fragment (C68). Upon secretion, C68 is cleaved by MT1-MMP-like proteases in the extracellular matrix, releasing 8 kDa and 5 kDa amyloidogenic peptides which aggregate in multiple tissues and cause disease-associated symptoms. We developed nanobodies that recognize the C68 fragment, but not native wild type gelsolin, and used these as molecular chaperones to mitigate gelsolin amyloid buildup in a mouse model that recapitulates the proteolytic cascade. We identified gelsolin nanobodies that potently reduce C68 proteolysis by MT1-MMP in vitro. Converting these nanobodies into an albumin-binding format drastically increased their serum half-life in mice, rendering them suitable for intraperitoneal injection. A 12-week treatment schedule of heterozygote D187N gelsolin transgenic mice with recombinant bispecific gelsolin-albumin nanobody significantly decreased gelsolin buildup in the endomysium and concomitantly improved muscle contractile properties. These findings demonstrate that nanobodies may be of considerable value in the treatment of gelsolin <span class="hlt">amyloidosis</span> and related diseases. PMID:25023329</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2017JQSRT.187..167S','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2017JQSRT.187..167S"><span>The new investigation of the b3Σ- -a3 Π <span class="hlt">system</span> of <span class="hlt">Al</span>H</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Szajna, Wojciech; Hakalla, Rafał; Kolek, Przemysław; Zachwieja, Mirosław</p> <p>2017-01-01</p> <p>The b3Σ- -a3 Π visible <span class="hlt">system</span> of <span class="hlt">Al</span>H was observed at high resolution by using a high accuracy, dispersive optical spectroscopy technique. The emission spectrum was excited in an aluminum hollow-cathode lamp with two anodes, filled with a static Ne/NH3 gas mixture. In the 25 , 900 - 26 , 500cm-1 spectral region, the rotational structure of the two overlapped 0-0 and 1-1 bands was clearly observed and precisely measured. In total, 260 transition wavenumbers have been assigned with an estimated accuracy of about 0.005 cm-1. The open rotational structure of the Q branches in both bands has been measured for the first time, and the Λ-doubling in the a3 Π , v = 0 , 1 levels has been described by o , p and q parameters. For example, the values for the v = 0 level are p0 = 1.754 (14) ×10-2cm-1, q0 = 3.264 (28) ×10-3cm-1 and o0 = 9.34 (20) ×10-2cm-1. Moreover, the spin-orbit interaction constants for the a3 Π state have been obtained experimentally as follows: A0 = 40.6040 (42)cm-1 and A1 = 40.419 (59)cm-1. The a3 Π , v = 0 , 1 levels are considered as regular, while for the b3Σ- , v = 0 , 1 levels considerable perturbations in the rotational structure have been observed. Consequently, these states have been represented in a different way in our least-squares treatment: a3 Π state by molecular constants and b3Σ- state by term values. The observed irregularities in the b3Σ- state have been graphically described by plotting experimental minus calculated term values versus quantum number N for the v = 0 level, as well as by plotting reduced term values versus N (N + 1) for the v = 1 level.</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>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('https://www.ncbi.nlm.nih.gov/pubmed/27103761','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/27103761"><span>Re-investigation of phase equilibria in the <span class="hlt">system</span> <span class="hlt">Al</span>-Cu and structural analysis of the high-temperature phase η1-<span class="hlt">Al</span>1-δCu.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Ponweiser, Norbert; Lengauer, Christian L; Richter, Klaus W</p> <p>2011-11-01</p> <p>The phase equilibria and reaction temperatures in the <span class="hlt">system</span> <span class="hlt">Al</span>-Cu were re-investigated by a combination of optical microscopy, powder X-ray diffraction (XRD) at ambient and elevated temperature, differential thermal analysis (DTA) and scanning electron microscopy (SEM). A full description of the phase diagram is given. The phase equilibria and invariant reactions in the Cu-poor part of the phase diagram could be confirmed. The Cu-rich part shows some differences in phase equilibria and invariant reactions compared to the known phase diagram. A two phase field was found between the high temperature phase η1 and the low temperature phase η2 thus indicating a first order transition. In the ζ1/ζ2 region of the phase diagram recent findings on the thermal stability could be widely confirmed. Contrary to previous results, the two phase field between δ and γ1 is very narrow. The results of the current work indicate the absence of the high temperature β0 phase as well as the absence of a two phase field between γ1 and γ0 suggesting a higher order transition between γ1 and γ0. The structure of γ0 (I-43m, Cu5Zn8-type) was confirmed by means of high-temperature XRD. Powder XRD was also used to determine the structure of the high temperature phase η1-<span class="hlt">Al</span>1-δCu. The phase is orthorhombic (space group Cmmm) and the lattice parameters are a = 4.1450(1) Å, b = 12.3004(4) Å and c = 8.720(1) Å; atomic coordinates are given.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/scitech/biblio/22492517','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/22492517"><span>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://adsabs.harvard.edu/abs/2016MSHT...58..400N','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016MSHT...58..400N"><span>Shortening the Time of Heat Treatment of Silumins of the <span class="hlt">Al</span> - Si - Cu <span class="hlt">System</span> by Modifying their Structure</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Nikitin, K. V.; Chikova, O. A.; Amosov, E. A.; Nikitin, V. I.</p> <p>2016-11-01</p> <p>The possibility of shortening the heat treatment hold of silumins of the <span class="hlt">Al</span> - Si - Cu <span class="hlt">system</span> by changing the parameters of the structure under chill casting is considered and a criterion for their evaluation is suggested. Alloys AK6M2 and AK8M3ch are used to demonstrate experimentally that decrease in the sizes of the crystals of primary silicon and in the transverse size of the α-<span class="hlt">Al</span> secondary dendrite arms can halve the time of holding for quenching and aging at a guaranteed margin of the strength properties.</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>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://adsabs.harvard.edu/abs/2007SSSci...9..531A','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2007SSSci...9..531A"><span>[H 4tren] 3/2·(<span class="hlt">Al</span> 6F 24)·3H 2O, the most condensed fluoride in the <span class="hlt">Al</span>(OH) 3- tren-HF aq.-ethanol <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>Adil, K.; Marrot, J.; Leblanc, M.; Maisonneuve, V.</p> <p>2007-06-01</p> <p>The most condensed crystalline fluoride that appears in the <span class="hlt">Al</span>(OH) 3- tren-HF aq.-ethanol <span class="hlt">system</span> at 190 °C is found to be [H 4tren] 3/2·(<span class="hlt">Al</span> 6F 24)·3H 2O. The structure is monoclinic, P2 1/ c, with a = 21.939(1) Å, b = 6.7180(2) Å, c = 23.329(1) Å, β = 111.324(2)°. ∞(<span class="hlt">Al</span> 6F 24) chains result from the connection of (<span class="hlt">Al</span> 7F 30) 9- polyanions by opposite <span class="hlt">Al</span>F 6 octahedra. Hydrogen bonds are established between the ∞(<span class="hlt">Al</span> 6F 24) chains and ordered or disordered [H 4tren] 4+ cations and water molecules.</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>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://adsabs.harvard.edu/abs/2014ChPhL..31b8502X','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2014ChPhL..31b8502X"><span>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://adsabs.harvard.edu/abs/2009JAP...106d4510T','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2009JAP...106d4510T"><span>Influence of Ni silicide phases on effective work function modulation with <span class="hlt">Al</span>-pileup in the Ni fully silicided gate/HfSiON <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>Tsuchiya, Yoshinori; Yoshiki, Masahiko; Koga, Junji; Nishiyama, Akira; Koyama, Masato</p> <p>2009-08-01</p> <p>Influences of Ni silicide phases on the effective work function (Φeff) modulation effect with <span class="hlt">Al</span> incorporation has been investigated in the Ni silicide/HfSiON <span class="hlt">systems</span>. We formed metal-insulator-semiconductor capacitors with <span class="hlt">Al</span> incorporated Ni silicide (NiSi, Ni2Si, and Ni3Si) gates on HfSiON by <span class="hlt">Al</span> solid-phase diffusion (<span class="hlt">Al</span>-SPD) process or <span class="hlt">Al</span> ion implantation (I/I) process. In the <span class="hlt">Al</span>-SPD process, <span class="hlt">Al</span> is deposited on Ni silicide gate. In the <span class="hlt">Al</span>-I/I process, <span class="hlt">Al</span> ions were doped in the upper part of Ni silicide layer. In both cases, we performed <span class="hlt">Al</span> drive-in annealing under the condition of 450 °C for 30 min in N2 ambient. It is found that the flat-band voltage (Vfb) values of <span class="hlt">Al</span> incorporated NiSi and Ni2Si gates shift negatively and identical independent of <span class="hlt">Al</span> incorporation processes. A highly concentrated <span class="hlt">Al</span> piled-up layer, which induces Φeff modulation to <span class="hlt">Al</span>-Φeff value, seems to correspond to the Vfb modulation. On the other hand, <span class="hlt">Al</span> incorporation has little influence on Φeff at the Ni3Si/HfSiON interface. We revealed that a lower <span class="hlt">Al</span> diffusion coefficient in Ni3Si phase reduces the <span class="hlt">Al</span> interface density at the Ni3Si/HfSiON interface. In addition, <span class="hlt">Al</span> piled-up layer is inherently unstable at the Ni3Si/HfSiON interface, which is confirmed from the detailed investigation about thermal stability of <span class="hlt">Al</span> piled-up layer by using phase change process from NiSi to Ni3Si phase.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016SuScT..29g5001L','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016SuScT..29g5001L"><span>Phase formation and superconducting properties of mechanically alloyed Nb3(<span class="hlt">Al</span>1-x Ge x ) <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>Li, Pingyuan; Chen, Yongliang; Xu, Liyuan; Zhang, Yun; Pan, Xifeng; Yan, Guo; Zhang, Yong; Cheng, C. H.; Feng, Yong; Zhao, Yong</p> <p>2016-07-01</p> <p>Mechanically alloyed Nb3(<span class="hlt">Al</span>1-x Ge x ) compounds with nominal composition x = 0%, 10%, 15%, 20%, 25% and 30% were synthesized using high-energy ball milling. The effects of Ge content and sintering temperature on the formation of the Nb3<span class="hlt">Al</span> superconducting phase at a relatively low temperature without the extremely high-temperature rapid heating, quenching and transformation process were studied. The results revealed that Ge doping in Nb3<span class="hlt">Al</span> improved the formation of the A15 phase at low temperatures, enhanced the superconducting transition temperature (T c), and refined the grain structure, thus improving the overall superconducting properties. The pinning behavior was also studied for the optimized sample.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/pages/biblio/1221954-simulation-atomic-diffusion-fcc-nial-system-kinetic-monte-carlo-study','SCIGOV-DOEP'); return false;" href="https://www.osti.gov/pages/biblio/1221954-simulation-atomic-diffusion-fcc-nial-system-kinetic-monte-carlo-study"><span>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 PAGES</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>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('https://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="https://ntrs.nasa.gov/search.jsp?R=20050168081&hterms=Copper&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D70%26Ntt%3DCopper"><span>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('https://www.osti.gov/scitech/biblio/22261726','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/22261726"><span>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/2016PhSS...58.1930A','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016PhSS...58.1930A"><span>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> </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('https://www.ncbi.nlm.nih.gov/pubmed/16876842','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/16876842"><span>Interdiffusion of <span class="hlt">Al</span>-Ni <span class="hlt">system</span> enhanced by ultrasonic vibration at ambient temperature.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Li, Mingyu; Ji, Hongjun; Wang, Chunqing; Bang, Han Sur; Bang, Hee Seon</p> <p>2006-12-01</p> <p>At ambient temperature, <span class="hlt">Al</span>-1%Si wire of 25 microm diameter was bonded successfully onto the Au/Ni/Cu pad by ultrasonic wedge bonding technology. Physical process of the bond formation and the interface joining essence were investigated. It is found that the wire was softened by ultrasonic vibration, at the same time, pressure was loaded on the wire and plastic flow was generated in the bonding wire, which promoted the diffusion for Ni into <span class="hlt">Al</span>. Ultrasonic vibration enhanced the interdiffusion that resulted from the inner defects such as dislocations, vacancies, voids and so on, which ascribed to short circuit diffusion.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/22240231','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/22240231"><span>The FTIR studies of gels and thin films of <span class="hlt">Al</span>2O3-TiO2 and <span class="hlt">Al</span>2O3-TiO2-SiO2 <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>Adamczyk, Anna; Długoń, Elżbieta</p> <p>2012-04-01</p> <p>In this work, samples in form of bulk ones and thin films were obtained using the sol-gel method. The bulk samples were heated at different temperatures (500 °C, 850 °C and 1100 °C) corresponding to the annealing process of coatings, deposited on different substrates by dipping and pulling out samples from the proper sol with the stable speed. Thin films of both <span class="hlt">Al</span>2O3-TiO2 and <span class="hlt">Al</span>2O3-TiO2-SiO2 <span class="hlt">systems</span> were deposited on carbon, steel and titanium substrates in two different ways: as single layers obtained from <span class="hlt">Al</span>2O3 sol, TiO2 sol and <span class="hlt">Al</span>2O3 sol or deposited as mixed coatings from <span class="hlt">Al</span>2O3-TiO2 sol as well as <span class="hlt">Al</span>2O3-TiO2-SiO2 one. All bulk samples were studied by the FTIR spectroscopy and the X-ray diffractometry while thin films were also investigated by the electron microscopy. In the IR spectra of <span class="hlt">Al</span>2O3-TiO2 samples, as well as gels and coatings, bands due to the vibrations of <span class="hlt">Al</span>O bonds of the octahedrally and tetrahedrally coordinated aluminum were observed. The IR spectra of samples of <span class="hlt">Al</span>2O3-TiO2-SiO2 <span class="hlt">system</span> differ from that of <span class="hlt">Al</span>2O3-TiO2 ones in presence of bands assigned to the SiO bond vibrations and in positions of bands due to <span class="hlt">Al</span>O bond vibrations. In all spectra of bulk samples and coatings, the positions of TiO bond vibrations were ascribed basing on the IR spectra of the pure anatase and rutile.</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>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('http://adsabs.harvard.edu/abs/2012TePhL..38..780R','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2012TePhL..38..780R"><span>Effect of conditions of pulsed electron-beam melting for <span class="hlt">Al</span> (film)/Ti (substrate) <span class="hlt">systems</span> on phase formation and properties of Ti-<span class="hlt">Al</span> surface alloys</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Rotshtein, V. P.; Kolubaeva, Yu. A.; Mei, X.; Markov, A. B.; Naiden, E. P.; Oskomov, K. V.; Pryadko, E. L.; Teresov, A. D.; Shulepov, I. A.; Shulov, V. A.</p> <p>2012-09-01</p> <p>Findings of comparative investigations are presented for phase formation, characteristics of hardening, tribological properties, and oxidation resistance of Ti-<span class="hlt">Al</span> intermetallic surface alloys formed by multiple alternation of deposition of an <span class="hlt">Al</span> film (0.1-1 μm) on a Ti substrate and pulsed liquid-phase mixing of <span class="hlt">Al</span> and Ti by intense low-energy (˜15 keV) electron beams with microsecond (˜3 μs) and submillisecond (100 μs) duration. It is found that microsecond synthesis is effective for formation of γ(Ti<span class="hlt">Al</span>) oxidation-resistant surface alloys. In turn, submillisecond synthesis allows one to form Ti3<span class="hlt">Al</span> surface alloys with enhanced wear resistance.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/scitech/biblio/6133740','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/6133740"><span>The evolution of interface microstructure in a ZrO[sub 2]/Ag-Cu-<span class="hlt">Al</span>-Ti <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Lee, Youngmin; Yu, Jin )</p> <p>1993-08-01</p> <p>Among ceramic/metal (C/M) joining technologies, the active filler metal method has been studied extensively due to the simple brazing process and excellent joint strength. Active metal elements, typically Ti, are intentionally added to braze alloys to enhance the formation of reaction products between the ceramic and the braze metal at the C/M interface. In the brazing of <span class="hlt">Al</span>[sub 2]O[sub 3] with the Ag-Cu-Ti filler metal, reaction products such as [gamma]-TiO, Cu[sub 2](Ti, <span class="hlt">Al</span>)[sub 4]O, Ti[sub 3](Cu[sub 0.76]<span class="hlt">Al</span>[sub 0.18]Sn[sub 0.06])[sub 3]O were found, while products such as Ti[sub 5]Si[sub 3] and TiN formed in the brazing of Si[sub 3]N[sub 4]. The presence of reaction layers at the C/M interface influences the interface strength in a complex way. In Cu/<span class="hlt">Al</span>[sub 2]O[sub 3], Co/<span class="hlt">Al</span>[sub 2]O[sub 3], Ni/<span class="hlt">Al</span>[sub 2]O[sub 3], and Cu/diamond <span class="hlt">systems</span>, maxima of joint strength were observed at some intermediate Ti addition, while the flexural strength decreased substantially with the thickening of the TiO layer in a ZrO[sub 2]/Ag-Cu-Sn-Ti <span class="hlt">system</span>. Thus, composition of the braze alloy (particularly, the content of the active metal), process conditions such as brazing temperature and time, microstructure and mechanical properties of reaction products at the C/M interfaces, interfacial chemistry, and residual stress are primary factors to be studied in order to understand the strengths of the C/M interfaces systematically. In the present and the following papers, evolutions of interfacial microstructures at various brazing conditions, and corresponding interface strengths are reported, respectively, for a ZrO[sub 2]/Ag-Cu-<span class="hlt">Al</span>-Ti <span class="hlt">system</span>.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2017MSHT...58..527A','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2017MSHT...58..527A"><span>Effect of Annealing on the Electrical Resistivity and Strengthening of Low-Alloy Alloys of the <span class="hlt">Al</span> - Zr - Si <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>Alabin, A. N.; Belov, N. A.; Korotkova, N. O.; Samoshinal, M. E.</p> <p>2017-01-01</p> <p>The effect of annealing at up to 550°C on the electrical resistivity ρ and HB hardness of low-alloy alloys of the <span class="hlt">Al</span> - Zr - Si <span class="hlt">system</span> containing up to 0.3% Zr and 0.3% Si is studied. The Thermo-Calc software is used to analyze the phase composition of the <span class="hlt">system</span>. The computed and experimental data are used to determine the lower and upper limits for heating of cast preforms from <span class="hlt">Al</span> - Zr alloys (shaped castings and ingots). It is shown that heating below 400°C and above 450°C is not expedient, because it increases the duration of the annealing in the former case and lowers the hardening effect due to coarsening of the Zr-containing particles in the latter case.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/scitech/biblio/22051137','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/22051137"><span>High rate reactive magnetron sputter deposition of <span class="hlt">Al</span>-doped ZnO with unipolar pulsing and impedance control <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Nishi, Yasutaka; Hirohata, Kento; Tsukamoto, Naoki; Sato, Yasushi; Oka, Nobuto; Shigesato, Yuzo</p> <p>2010-07-15</p> <p><span class="hlt">Al</span>-doped ZnO (AZO) films were deposited on quartz glass substrates, unheated and heated to 200 deg. C, using reactive sputtering with a special feedback <span class="hlt">system</span> of discharge impedance combined with midfrequency pulsing. A planar Zn-<span class="hlt">Al</span> alloy target was connected to the switching unit, which was operated in a unipolar pulse mode. The oxidation of the target surface was precisely controlled by a feedback <span class="hlt">system</span> for the entire O{sub 2} flow ratio including ''the transition region''. The deposition rate was about 10-20 times higher than that for films deposited by conventional sputtering using an oxide target. A deposition rate of AZO films of 390 nm/min with a resistivity of 3.8x10{sup -4} {Omega} cm and a transmittance in the visible region of 85% was obtained when the films were deposited on glass substrates heated to 200 deg. C with a discharge power of 4 kW.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.alsa.org/2015-non-responsive-pages/about-als/diagnosing-als.html','NIH-MEDLINEPLUS'); return false;" href="http://www.alsa.org/2015-non-responsive-pages/about-als/diagnosing-als.html"><span>Diagnosing <span class="hlt">ALS</span></span></a></p> <p><a target="_blank" href="http://medlineplus.gov/">MedlinePlus</a></p> <p></p> <p></p> <p>... that a person diagnosed with <span class="hlt">ALS</span> seek a second opinion from an <span class="hlt">ALS</span> "expert" - someone who diagnoses and treats many <span class="hlt">ALS</span> patients and has training in this medical specialty. The <span class="hlt">ALS</span> Association maintains a list of recognized experts in the field of <span class="hlt">ALS</span>. See <span class="hlt">ALS</span> Association Certified Centers of ...</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://www.dtic.mil/docs/citations/ADA630952','DTIC-ST'); return false;" href="http://www.dtic.mil/docs/citations/ADA630952"><span>Kinetic Metallization. Repair of IVD <span class="hlt">Al</span> Coatings and Mg Alloys Aircraft Components Using Portable Kinetic Metallization <span class="hlt">Systems</span></span></a></p> <p><a target="_blank" href="https://publicaccess.dtic.mil/psm/api/service/search/search">DTIC Science & Technology</a></p> <p></p> <p>2008-02-27</p> <p>person shall be subject to a penalty for failing to comply with a collection of information if it does not display a currently valid OMB control number...aircraft carriers & depots Environmentally sustainable Meets Navy JTP-2003 Portable <span class="hlt">system</span> & Handheld spray gun Robotic deployment for OEM Applications...Problem - Field & Depot Repair of Damaged IVD-<span class="hlt">Al</span> & Mg Alloys NADEP Facilities PEO(T) F/A-18, EA-18G PMA -271 E-6B</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/scitech/biblio/69689','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/69689"><span>Flow strength and size effect of an <span class="hlt">Al</span>-Si-Mg composite model <span class="hlt">system</span> under multiaxial loadings</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Zhu, H.T.; Zbib, H.M.; Khraisheh, M.K.</p> <p>1995-06-01</p> <p>In the present work, using an <span class="hlt">Al</span>-Si-Mg composite model <span class="hlt">system</span>, a series of experiments were conducted to further investigate the effect of microstructural parameters on the flow strength of particulate reinforced MMCs. In addition to the simple tension and torsion tests, biaxial tension-torsion tests were performed to obtain the yield surface, showing the isotropic nature of plastic deformation in these materials. Experimental results are also compared with the prediction of a recently developed theoretical model.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://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="https://ntrs.nasa.gov/search.jsp?R=19810054114&hterms=Free+energy&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D30%26Ntt%3DFree%2Benergy"><span>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('https://www.osti.gov/scitech/biblio/21208697','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/21208697"><span>Synthesis of Waste Form in the Gd-Fe-<span class="hlt">Al</span>-Ni-Mn-Cr-O <span class="hlt">System</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Chae, S.C.; Jang, Y.N.; Bae, I.K.; Ryu, K.W.</p> <p>2006-07-01</p> <p>Poly-phase waste form which was the mixture of Gd{sub 3}Fe{sub 2}<span class="hlt">Al</span>{sub 3}O{sub 12} and (Ni{sub x}Mn{sub 1-x})(Fe{sub y}Cr{sub 1-y}){sub 2}O{sub 4} was synthesized. Also, we are intended to examine phase relation and physicochemical properties of coexisted phases in the compositions and to confirm accommodation relation of elements and phases. Two types of phase series were observed: Garnet-perovskite-spinel and Garnet-spinel. The compositions of garnets and spinels were nonstoichiometric, and especially, this poly-phase ceramics may be in a good waste form. The excessive Gd in garnets indicated the immobilization of higher content of actinides. The nonstoichiometric compositions of garnet and spinel were attributed to the formation of perovskite in that perovskite contained Gd, Fe and <span class="hlt">Al</span> from garnet and Cr from spinel. (authors)</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>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/biblio/7209323','DOE-PATENT-XML'); return false;" href="http://www.osti.gov/scitech/biblio/7209323"><span>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>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('https://www.ncbi.nlm.nih.gov/pubmed/22907449','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/22907449"><span>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('https://www.osti.gov/pages/biblio/1321965-structural-ordering-solid-liquid-interfaces-al-sm-system-molecular-dynamics-study','SCIGOV-DOEP'); return false;" href="https://www.osti.gov/pages/biblio/1321965-structural-ordering-solid-liquid-interfaces-al-sm-system-molecular-dynamics-study"><span>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 PAGES</a></p> <p>Sun, Yang; Zhang, Feng; Ye, Zhuo; ...</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('http://adsabs.harvard.edu/abs/2010ApPhA..99..607V','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2010ApPhA..99..607V"><span>Investigation of the ɛ phase in the Fe-<span class="hlt">Al</span> <span class="hlt">system</span> by high-temperature neutron diffraction</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Vogel, Sven C.; Stein, Frank; Palm, Martin</p> <p>2010-06-01</p> <p>In the central part of the Fe-<span class="hlt">Al</span> <span class="hlt">system</span> between about 58 and 65 at.% <span class="hlt">Al</span>, a high-temperature phase denoted as ɛ occurs with a hitherto unknown crystallographic structure. The phase is stable between 1231°C and 1095°C. In order to study the crystallographic structure of the ɛ phase, in situ high-temperature neutron time-of-flight diffraction experiments have been performed at the HIPPO instrument at the Los Alamos Neutron Science Center (LANSCE). The ɛ phase was found to have the formula Fe5<span class="hlt">Al</span>8 with a body-centred cubic structure of the Hume-Rothery Cu5Zn8 type (Ibar{4}3m (No. 217), Z=4, cI52) and 52 atoms in the unit cell. Its lattice parameter is a=8.9756(2) Å at 1120°C, which is 3.02 times that of cubic Fe<span class="hlt">Al</span> (B2) at the same temperature. We report here the evolution of the crystallographic parameters over the temperature range between 1080°C and 1120°C.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/scitech/biblio/1321965','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/1321965"><span>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/scitech">SciTech Connect</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 with 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.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/28381753','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/28381753"><span>The Coexistence of Multiple Myeloma-associated Amyloid Light-chain <span class="hlt">Amyloidosis</span> and Fabry Disease in a Hemodialysis Patient.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Taguchi, Kensei; Moriyama, Atsuo; Kodama, Goh; Nakayama, Yosuke; Fukami, Kei</p> <p>2017-01-01</p> <p>Fabry disease (FD) is an inherited lysosomal disorder caused by an X-linked α-galactosidase A deficiency. We report the case of a 50-year-old male FD patient on hemodialysis who presented with macroglossia-related speaking difficulty and gastrointestinal symptoms. An endoscopic analysis revealed multiple gastric ulcers, and a histological examination led to a diagnosis of amyloid light-chain <span class="hlt">amyloidosis</span>. Serum free light-chain and bone marrow analyses detected multiple myeloma (MM). Treatment with bortezomib and dexamethasone significantly improved the patient's symptoms. This is the first case to demonstrate a potential pathogenic relationship between FD and MM. The similar gastrointestinal manifestations might have contributed to the diagnostic difficulty.</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('https://www.ncbi.nlm.nih.gov/pubmed/25824838','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/25824838"><span>The Cp<span class="hlt">AL</span> quorum sensing <span class="hlt">system</span> regulates production of hemolysins CPA and PFO to build Clostridium perfringens biofilms.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Vidal, Jorge E; Shak, Joshua R; Canizalez-Roman, Adrian</p> <p>2015-06-01</p> <p>Clostridium perfringens strains produce severe diseases, including myonecrosis and enteritis necroticans, in humans and animals. Diseases are mediated by the production of potent toxins that often damage the site of infection, e.g., skin epithelium during myonecrosis. In planktonic cultures, the regulation of important toxins, such as CPA, CPB, and PFO, is controlled by the C. perfringens Agr-like (Cp<span class="hlt">AL</span>) quorum sensing (QS) <span class="hlt">system</span>. Strains also encode a functional LuxS/AI-2 <span class="hlt">system</span>. Although C. perfringens strains form biofilm-like structures, the regulation of biofilm formation is poorly understood. Therefore, our studies investigated the role of Cp<span class="hlt">AL</span> and LuxS/AI-2 QS <span class="hlt">systems</span> and of QS-regulated factors in controlling the formation of biofilms. We first demonstrate that biofilm production by reference strains differs depending on the culture medium. Increased biomass correlated with the presence of extracellular DNA in the supernatant, which was released by lysis of a fraction of the biofilm population and planktonic cells. Whereas ΔagrB mutant strains were not able to produce biofilms, a ΔluxS mutant produced wild-type levels. The transcript levels of Cp<span class="hlt">AL</span>-regulated cpa and pfoA genes, but not cpb, were upregulated in biofilms compared to planktonic cultures. Accordingly, Δcpa and ΔpfoA mutants, in type A (S13) or type C (CN3685) backgrounds, were unable to produce biofilms, whereas CN3685Δcpb made wild-type levels. Biofilm formation was restored in complemented Δcpa/cpa and ΔpfoA/pfoA strains. Confocal microscopy studies further detected CPA partially colocalizing with eDNA on the biofilm structure. Thus, Cp<span class="hlt">AL</span> regulates biofilm formation in C. perfringens by increasing levels of certain toxins required to build biofilms.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4432755','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=4432755"><span>The Cp<span class="hlt">AL</span> Quorum Sensing <span class="hlt">System</span> Regulates Production of Hemolysins CPA and PFO To Build Clostridium perfringens Biofilms</span></a></p> <p><a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pmc">PubMed Central</a></p> <p>Shak, Joshua R.; Canizalez-Roman, Adrian</p> <p>2015-01-01</p> <p>Clostridium perfringens strains produce severe diseases, including myonecrosis and enteritis necroticans, in humans and animals. Diseases are mediated by the production of potent toxins that often damage the site of infection, e.g., skin epithelium during myonecrosis. In planktonic cultures, the regulation of important toxins, such as CPA, CPB, and PFO, is controlled by the C. perfringens Agr-like (Cp<span class="hlt">AL</span>) quorum sensing (QS) <span class="hlt">system</span>. Strains also encode a functional LuxS/AI-2 <span class="hlt">system</span>. Although C. perfringens strains form biofilm-like structures, the regulation of biofilm formation is poorly understood. Therefore, our studies investigated the role of Cp<span class="hlt">AL</span> and LuxS/AI-2 QS <span class="hlt">systems</span> and of QS-regulated factors in controlling the formation of biofilms. We first demonstrate that biofilm production by reference strains differs depending on the culture medium. Increased biomass correlated with the presence of extracellular DNA in the supernatant, which was released by lysis of a fraction of the biofilm population and planktonic cells. Whereas ΔagrB mutant strains were not able to produce biofilms, a ΔluxS mutant produced wild-type levels. The transcript levels of Cp<span class="hlt">AL</span>-regulated cpa and pfoA genes, but not cpb, were upregulated in biofilms compared to planktonic cultures. Accordingly, Δcpa and ΔpfoA mutants, in type A (S13) or type C (CN3685) backgrounds, were unable to produce biofilms, whereas CN3685Δcpb made wild-type levels. Biofilm formation was restored in complemented Δcpa/cpa and ΔpfoA/pfoA strains. Confocal microscopy studies further detected CPA partially colocalizing with eDNA on the biofilm structure. Thus, Cp<span class="hlt">AL</span> regulates biofilm formation in C. perfringens by increasing levels of certain toxins required to build biofilms. PMID:25824838</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/scitech/biblio/22345239','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/22345239"><span>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('https://www.ncbi.nlm.nih.gov/pubmed/24010694','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/24010694"><span>[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('http://adsabs.harvard.edu/abs/2015AIPC.1665e0175J','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2015AIPC.1665e0175J"><span>Influence of <span class="hlt">Al</span>2O3 nano-filler on dielectric properties and conductivity of two different PVA-PEO blend <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>Joge, Prajakta; Kanchan, D. K.; Dave, Gargi</p> <p>2015-06-01</p> <p><span class="hlt">System</span>-1: PVA-PEO-PEG-AgNO3 and <span class="hlt">System</span>-2: PVA-PEO-EC-LiCF3SO3, are two blend <span class="hlt">systems</span> prepared for different concentrations of <span class="hlt">Al</span>2O3 nano-filler ranging from 2 to 10 wt%. The effect of <span class="hlt">Al</span>2O3 nano filler on the conductivity (σdc) and dielectric properties such as dielectric constant (ɛ') and dielectric loss (ɛ") of the <span class="hlt">systems</span> is thoroughly investigated using impedance spectroscopic analysis technique.</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>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('https://www.osti.gov/scitech/biblio/22273493','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/22273493"><span>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/abs/2012PhDT.......215L','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2012PhDT.......215L"><span>Approche thermodynamique de la corrosion des refractaires aluminosiliceux par le bain cryolithique : modelisation thermodynamique du <span class="hlt">systeme</span> quaternaire reciproque <span class="hlt">Al</span>F3-NaF-SiF 4-<span class="hlt">Al</span>2O3-Na2O-SiO2</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Lambotte, Guillaume</p> <p></p> <p>The main objective of this thesis is the thermodynamic modeling of liquid and solid solutions, the stoichiometric compounds and the gaseous phase of the chemical <span class="hlt">system</span> which represent the species involved in the corrosion of the refractory lining of the electrolysis cell. This chemical <span class="hlt">system</span> is the quaternary reciprocal <span class="hlt">system</span> <span class="hlt">Al</span>F3-NaF-SiF4-<span class="hlt">Al</span> 2O3-Na2O-SiO2 with aluminum and carbon. The modeling of the entire reciprocal <span class="hlt">system</span> has never been realized before and is very challenging due to the nature of the oxyfluoride solution. The thermodynamic modeling is based on Gibbs free energy functions coming from the theory of solutions. The central hypothesis of this project is that, if it is possible to reproduce the strong short-range ordering observed between the ions of the liquid solution, then reasonable results will be obtained for the phase equilibria involved in this chemical <span class="hlt">system</span>. The thermodynamic model used in this thesis is the Modified Quasichemical Model in the Quadruplet Approximation (MQMQA) which takes into account the short-range ordering between first- and also second-nearest-neighbors, and is the best suited to model the oxyfluoride liquid solution. First, the reciprocal <span class="hlt">system</span> with the most negative Gibbs free energy change for the exchange reaction between the end-members, NaF-SiF4-Na 2O-SiO2, was modeled, allowing thus the validation of the recent modifications of the MQMQA aimed at improving the modeling of the thermodynamic properties of reciprocal solutions presenting a strong short-range order among first-nearest neighbors as well as second-nearest neighbors. In order to model this <span class="hlt">system</span>, an estimate of the thermodynamic properties of the hypothetical SiF4 liquid was necessary. The experimental data in the binary <span class="hlt">systems</span> NaF-SiF4 and Na2O-SiO2 are reproduced within the experimental error limits. In the reciprocal <span class="hlt">system</span>, a group of data in conflict with all others could not be reproduced. The data considered reliable are well reproduced. A</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>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('http://adsabs.harvard.edu/abs/2016JMiMi..26h4007B','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016JMiMi..26h4007B"><span>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.alsa.org/','NIH-MEDLINEPLUS'); return false;" href="http://www.alsa.org/"><span><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('https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=5282474','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=5282474"><span>Increase of TREM2 during Aging of an Alzheimer’s Disease Mouse Model Is Paralleled by Microglial Activation and <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>Brendel, Matthias; Kleinberger, Gernot; Probst, Federico; Jaworska, Anna; Overhoff, Felix; Blume, Tanja; Albert, Nathalie L.; Carlsen, Janette; Lindner, Simon; Gildehaus, Franz Josef; Ozmen, Laurence; Suárez-Calvet, Marc; Bartenstein, Peter; Baumann, Karlheinz; Ewers, Michael; Herms, Jochen; Haass, Christian; Rominger, Axel</p> <p>2017-01-01</p> <p>Heterozygous missense mutations in the triggering receptor expressed on myeloid cells 2 (TREM2) have been reported to significantly increase the risk of developing Alzheimer’s disease (AD). Since TREM2 is specifically expressed by microglia in the brain, we hypothesized that soluble TREM2 (sTREM2) levels may increase together with in vivo biomarkers of microglial activity and <span class="hlt">amyloidosis</span> in an AD mouse model as assessed by small animal positron-emission-tomography (μPET). In this cross-sectional study, we examined a strong amyloid mouse model (PS2APP) of four age groups by μPET with [18F]-GE180 (glial activation) and [18F]-florbetaben (<span class="hlt">amyloidosis</span>), followed by measurement of sTREM2 levels and amyloid levels in the brain. Pathology affected brain regions were compared between tracers (dice similarity coefficients) and pseudo-longitudinally. μPET results of both tracers were correlated with terminal TREM2 levels. The brain sTREM2 levels strongly increased with age of PS2APP mice (5 vs. 16 months: +211%, p < 0.001), and correlated highly with μPET signals of microglial activity (R = 0.89, p < 0.001) and <span class="hlt">amyloidosis</span> (R = 0.92, p < 0.001). Dual μPET enabled regional mapping of glial activation and <span class="hlt">amyloidosis</span> in the mouse brain, which progressed concertedly leading to a high overlap in aged PS2APP mice (dice similarity 67%). Together, these results substantiate the use of in vivo μPET measurements in conjunction with post mortem sTREM2 in future anti-inflammatory treatment trials. Taking human data into account sTREM2 may increase during active amyloid deposition. PMID:28197095</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/26986678','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/26986678"><span>Concomitant use of Congo red staining and confocal laser scanning microscopy to detect <span class="hlt">amyloidosis</span> in oral biopsy: A clinicopathological study of 16 patients.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Scivetti, Michele; Favia, Gianfranco; Fatone, Laura; Maiorano, Eugenio; Crincoli, Vito</p> <p>2016-01-01</p> <p>Twenty oral biopsies from 16 patients were analyzed both by traditional microscopy and by confocal laser scanning microscopy. Using conventional histopathological techniques, the diagnosis of <span class="hlt">amyloidosis</span> was confirmed only in 15 biopsies. Using confocal laser scanning microscopy, amyloid deposits were detected in all of the samples. The current study shows that confocal laser scanning analysis helps to identify minimal amyloid deposits that could be overlooked using traditional microscopy, thus raising the sensitivity of oral biopsy up to 100%.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/scitech/biblio/77962','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/77962"><span>Interdiffusion in the <span class="hlt">system</span> MgO-<span class="hlt">Al</span>{sub 2}O{sub 3} and the effect of some</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Zhang, P.; Seetharaman, S.; DebRoy, T.</p> <p>1995-08-01</p> <p>With a view to seek an improved understanding of the DIMOX{trademark} process, interdiffusion of polycrystalline MgO and <span class="hlt">Al</span>{sub 2}O{sub 3} in the temperature range 1,473 K to 1,873 K was studied by diffusion couple experiments. The interdiffusivities in the spinel layer were calculated as functions of composition and temperature. The diffusivities in the spinel phase decrease with the concentration of <span class="hlt">Al</span>{sup 3+} ions at temperatures above 1,773 K and are constant below this temperature. The spinel portion of the phase diagram in the <span class="hlt">system</span> MgO-<span class="hlt">Al</span>{sub 2}O{sub 3} was determined from carefully measured compositions at the phase boundaries and the low temperature spinel region of the phase diagram was confirmed from the present results. For Zn{sup 2+} as dopant in alumina, the growth rate of spinel thickness seems to increase when compared with that of the diffusion couples without dopant. The samples containing Si{sup 4+} as dopant reveal the formation of a glass phase and the effect of Si{sup 4+} on the diffusion process appears to be negligible.</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>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('https://www.osti.gov/scitech/biblio/21090724','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/21090724"><span>Gamma-resonance study of nanopowders with different dispersion and quasicrystalline phases in the <span class="hlt">Al</span>-Cu-Fe <span class="hlt">system</span></span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Frolov, K. V. Mikheeva, M. N.; Lyubutin, I. S.; Nikonov, A. A.; Teplov, A. A.; Shaitura, D. S.; Abuzin, Yu. A.</p> <p>2007-11-15</p> <p>{sup 57}Fe Moessbauer spectroscopy has been used to monitor synthesis of quasicrystals in the <span class="hlt">Al</span>-Cu-Fe <span class="hlt">system</span> and study the influence of the size of quasicrystalline particles in powder samples of the <span class="hlt">Al</span>{sub 63.1}Cu{sub 25.6}Fe{sub 11.3} alloy on the properties of synthesized materials. Quasicrystalline samples of different dispersion with particle sizes from 0.3 to 15 {mu}m have been studied in the temperature range 80-295 K. It is established that iron atoms in an <span class="hlt">Al</span>{sub 63.1}Cu{sub 25.6}Fe{sub 11.3} quasicrystals occupy four types of structural positions, which differ in the atomic composition of the nearest environment. The results of the analysis suggest the dependence of the hyperfine-interaction parameters on the degree of sample dispersion. The components corresponding to iron atoms in both the surface layer and bulk of microparticles are isolated in the Moessbauer spectra. No magnetic hyperfine splitting has been found in the Moessbauer spectra in the entire temperature range. This fact suggests that a localized magnetic moment is absent in iron atoms.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2017E%26PSL.463..171P','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2017E%26PSL.463..171P"><span>Stability of phase H in the MgSiO4H2-<span class="hlt">Al</span>OOH-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>Panero, Wendy R.; Caracas, Razvan</p> <p>2017-04-01</p> <p>The newly described hydrous phase H, MgSiO4H2, is stable to the pressures and temperatures of the Earth's lower mantle, suggesting that the Earth's lower mantle may participate in the cycling of water. We present the results of ab initio calculations on the stability of this phase within the phase H-δ<span class="hlt">Al</span>OOH-SiO2 <span class="hlt">system</span> between 20 and 60 GPa, exploring the wide pressure- and temperature stability field of this mineral. We find that hydrogen in phase H is bonded in a single well at all pressures, and only the pure phase results in pressure-induced symmetrization, while the solid solution maintains asymmetric bonding to high pressure. Substitutions on octahedral sites are locally charge-balanced by H. Furthermore, small amounts of <span class="hlt">Al</span> + H in SiO2 stabilize the CaCl2-structure of SiO2 to pressures as low as 20 GPa while phase H and δ<span class="hlt">Al</span>OOH form an ideal mixture at all pressures considered. The resulting structure is stable relative to the anhydrous assemblage of the Earth's lower mantle by 16-19 kJ/mol. In alumina-rich phase H this increases the stability of the mineral by ∼800 K relative to the pure substance, making it potentially stable under lower mantle temperatures along typical geotherms.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.ncbi.nlm.nih.gov/pubmed/18661740','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/18661740"><span>Catalytic oxidation of toluene in contaminant emission control <span class="hlt">systems</span> using Mn-Ce/gamma-<span class="hlt">Al</span>2O3.</span></a></p> <p><a target="_blank" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed">PubMed</a></p> <p>Kim, H-J; Choi, S-W; Inyang, H I</p> <p>2008-05-01</p> <p>Toluene, the alkyl benzene, is a common constituent of contaminant streams emitted by hydrocarbon fuel combustion <span class="hlt">systems</span>. The oxidation of toluene to less toxic compounds can be enhanced through catalysis. The capacity of Mn-Ce/gamma-<span class="hlt">Al</span>2O3 to catalyze toluene oxidation was investigated using a fixed bed flow reactor, operating within a temperature range of 160-400 degrees C. Mono-metallic catalysts were prepared with the manganese and cerium contents of 1-21 wt% on gamma-<span class="hlt">Al</span>2O3, support and bi-metallic catalysts were prepared with cerium (0.5-21 wt%/) on 18.2 wt% manganese. The results indicate that the 18.2 wt% Mn-10.0 wt% Ce catalyst combination had the best catalytic efficiency for toluene oxidation. Increase in cerium loading reduces the surface area of catalytic materials measured by BET, but increases catalytic activity. Data obtained through TGA (Thermogravimetric analysis), XRD (X-ray diffraction) and toluene-TPR (Temperature Programmed Reduction) measurements show that the reduction of the catalysts in the process of toluene oxidation is directly proportional to observed weight loss under hydrogen flow. From these results, it is concluded that cerium improves the catalytic role of manganese in toluene oxidation. Oxygen mobility is also promoted in a redox mechanism in which MnO2 serves as the active sites. These results are useful in the development of toluene emission control <span class="hlt">systems</span> for hydrocarbon fuel combustion <span class="hlt">systems</span>.</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>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/12484038','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/12484038"><span>[Treatment of tracheobronchial malignant tumors using a new high power diode contact laser (Ga<span class="hlt">Al</span>As) <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>Ishiguro, Takashi; Sawa, Toshiyuki; Yoshida, Tsutomu; Yokoyama, Mitsuru; Murakawa, Shinji; Azuma, Kenichirou; Tomida, Yoshiteru</p> <p>2002-11-01</p> <p>We treated ten patients with tracheobronchial malignant tumors using a new high power diode contact laser (Ga<span class="hlt">Al</span>As) <span class="hlt">system</span> (DIOMED 25, OLYMPUS) with a flexible bronchofiberscope (OLYMPUS BF IT200 or BF IT240). The total energy of the high power diode laser was 811 J, with a range of 64-3,960 J. With this method 85.7 percent of the symptoms such as dyspnea and hemoptysis were improved, and there was no incidence of massive hemorrhage or serious respiratory failure. The results confirmed the usefulness and safety of this method of treatment for obstructive lesions due to tracheobronchial polypoid malignant tumor and bleeding of the tracheobronchial tree.</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://adsabs.harvard.edu/abs/2017RJPCA..91..468K','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2017RJPCA..91..468K"><span>Catalytic properties of Fe/SiO2-<span class="hlt">Al</span>2O3 <span class="hlt">systems</span>, obtained via sol-gel method</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Kon'kova, T. V.; Gordienko, M. G.; Alekhina, M. B.; Men'shutina, N. V.</p> <p>2017-03-01</p> <p>The high efficacy of iron-containing catalysts based on SiO2-<span class="hlt">Al</span>2O3 <span class="hlt">systems</span> obtained via sol-gel method in the oxidative destruction of carmoisine azo dye in aqueous solutions is demonstrated. It is found that the stability of the catalysts with respect to the leaching of iron ions into a solution during catalysis grows along with the aluminum content in the composition of aluminosilicate supports. It is concluded that the synthesized catalysts are promising materials for purifying wastewaters contaminated with organic dyes.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016InJPh..90..917H','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016InJPh..90..917H"><span>Electronic structure, mechanical, and optical properties of CaO·<span class="hlt">Al</span>2O3 <span class="hlt">system</span>: a first principles approach</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Hussain, A.; Mehmood, S.; Rasool, M. N.; Aryal, S.; Rulis, P.; Ching, W. Y.</p> <p>2016-08-01</p> <p>A comprehensive study of the structure, bonding, mechanical and optical properties of five stable phases within the calcium aluminate (Ca-<span class="hlt">Al</span>-O) series with different CaO to <span class="hlt">Al</span>2O3 proportions has been carried out using the density functional theory based orthogonalized linear combination of atomic orbitals (OLCAO) method. The phases are C3A, C12A7-crystal, CA, CA2, and CA6 and the oxygen deficient C12A7-electride phase. These five stable phases are wide band gap insulators with energy gap values ranging from 3.85 to 4.62 eV. The charge neutral C12A7-crystal has localized defective states in the gap whereas the C12A7-electride phase has a region of metallic bands of about 2 eV wide in the gap. Effective charge and bond order calculations reveal intimate details of electronic structure and bonding in relation to the aluminate contents in the series. It is shown that <span class="hlt">Al</span>-O bonds dominate the Ca-O bonds in determining the crystal strength with CA6 having the highest and C12A7 having the lowest bond order density. Calculations of elastic coefficients and mechanical properties in these crystals show a high degree of diversity and anisotropic behavior consistent with the bond order calculations. The refractive index values from optical properties calculations are in good agreement with available literature. Other results furnish more insights for the Ca-<span class="hlt">Al</span>-O series and provide the opportunity for further investigations on similar or more complicated quaternary <span class="hlt">systems</span> with potential novel properties.</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>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>2016-04-07</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>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=3982257','PMC'); return false;" href="https://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=3982257"><span>Preparation of Tween 80-Zn/<span class="hlt">Al</span>-Levodopa-Layered Double Hydroxides Nanocomposite for Drug Delivery <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>Kura, Aminu Umar; Hussein-Al-Ali, Samer Hasan; Hussein, Mohd Zobir; Fakurazi, Sharida</p> <p>2014-01-01</p> <p>We incorporated anti-Parkinsonian drug, levodopa (dopa), in Zn/<span class="hlt">Al</span>-LDH by coprecipitation method to form dopa-LDH nanocomposite. Further coating of Tween-80 on the external surfaces of dopa-LDH nanocomposite was achieved through the oxygen of C=O group of Tween-80 with the layer of dopa-LDH nanocomposite. The final product is called Tween-dopa-LDH nanocomposite. The X-ray diffraction indicates that the Tween-dopa-LDH nanocomposite was formed by aggregation structure. From the TGA data, the Tween-80 loading on the surface of LDH and dopa-LDH was 8.6 and 7.4%, respectively. The effect of coating process on the dopa release from Tween-dopa-LDH nanocomposite was also studied. The release from Tween-dopa-LDH nanocomposite shows slower release compared to the release of the drug from dopa-LDH nanocomposite as done previously in our study, presumably due to the retarding shielding effect. The cell viability study using PC12 showed improved viability with Tween-80 coating on dopa-LDH nanocomposite as studied by mitochondrial dehydrogenase activity (MTT assay). PMID:24782658</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2016AIPC.1698c0004K','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016AIPC.1698c0004K"><span>Phase transformations in the <span class="hlt">system</span> Cu-Zn-<span class="hlt">Al</span> under conditions far from equilibrium</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Klopotov, Anatolii; Ivanov, Yuri; Vlasov, Viktor; Dedov, Nikolai; Loskutov, Oleg</p> <p>2016-01-01</p> <p>It is shown that the alloy Cu-Zn-<span class="hlt">Al</span> is a multiphase material. Under equilibrium conditions this alloy can form an α-phase (FCC crystalline lattice) and a β-phase (simple cubic crystalline lattice) based on copper. The possibility of formation of a γ-phase due to a three-component alloy composition is revealed. It is established that different chemical composition of the copper-based solid solution (alloys with zinc or alloys with aluminum), different concentration of the second element in a solid solution leads to the fact that within the same type of the crystalline lattice there is a certain amount of α- and γ-phases, differing in the parameter value of the crystalline lattice. The possibility of formation of powder alloys with an x-ray amorphous and a nanocrystalline structure using the plasma chemical synthesis methods is demonstrated. A wide variety of binary phases, each with different concentrations of zinc and aluminum in a solid copper-based solution is revealed. These results indicate that plasma-chemical synthesis of metal alloy powders is accompanied by separation of elements. Powders of the ternary composition are not detected.</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://www.osti.gov/scitech/biblio/5689128','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/5689128"><span>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('https://www.ncbi.nlm.nih.gov/pubmed/24782658','PUBMED'); return false;" href="https://www.ncbi.nlm.nih.gov/pubmed/24782658"><span>Preparation of Tween 80-Zn/<span class="hlt">Al</span>-levodopa-layered double hydroxides nanocomposite for drug delivery <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>Kura, Aminu Umar; Hussein-Al-Ali, Samer Hasan; Hussein, Mohd Zobir; Fakurazi, Sharida</p> <p>2014-01-01</p> <p>We incorporated anti-Parkinsonian drug, levodopa (dopa), in Zn/<span class="hlt">Al</span>-LDH by coprecipitation method to form dopa-LDH nanocomposite. Further coating of Tween-80 on the external surfaces of dopa-LDH nanocomposite was achieved through the oxygen of C=O group of Tween-80 with the layer of dopa-LDH nanocomposite. The final product is called Tween-dopa-LDH nanocomposite. The X-ray diffraction indicates that the Tween-dopa-LDH nanocomposite was formed by aggregation structure. From the TGA data, the Tween-80 loading on the surface of LDH and dopa-LDH was 8.6 and 7.4%, respectively. The effect of coating process on the dopa release from Tween-dopa-LDH nanocomposite was also studied. The release from Tween-dopa-LDH nanocomposite shows slower release compared to the release of the drug from dopa-LDH nanocomposite as done previously in our study, presumably due to the retarding shielding effect. The cell viability study using PC12 showed improved viability with Tween-80 coating on dopa-LDH nanocomposite as studied by mitochondrial dehydrogenase activity (MTT assay).</p> </li> <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>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.dtic.mil/docs/citations/ADA119295','DTIC-ST'); return false;" href="http://www.dtic.mil/docs/citations/ADA119295"><span>Formation and Structure of <span class="hlt">AL</span>(PO3)3 <span class="hlt">System</span> Glasses,</span></a></p> <p><a target="_blank" href="https://publicaccess.dtic.mil/psm/api/service/search/search">DTIC Science & Technology</a></p> <p></p> <p>1982-08-06</p> <p>only the <span class="hlt">systems</span> con- taining LiF or MgF2 that formed glass. 3) In the four-component <span class="hlt">systems</span> that only contained NaF, KF, Ca?2 , SrF2 , or BaF2...a joining effect upon networks. Other single valued and dual valued chemicals such as NaF, KF, CaF2, SrF2 9 and BaF2 , belong to the out- er part of</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>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('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>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://adsabs.harvard.edu/abs/2016JSSCh.237...48C','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2016JSSCh.237...48C"><span>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.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>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('https://ntrs.nasa.gov/search.jsp?R=19840031712&hterms=blue+laser&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D10%26Ntt%3Dblue%2Blaser','NASA-TRS'); return false;" href="https://ntrs.nasa.gov/search.jsp?R=19840031712&hterms=blue+laser&qs=Ntx%3Dmode%2Bmatchall%26Ntk%3DAll%26N%3D0%26No%3D10%26Ntt%3Dblue%2Blaser"><span>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('http://hdl.handle.net/2060/19960054113','NASA-TRS'); return false;" href="http://hdl.handle.net/2060/19960054113"><span>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('https://www.osti.gov/scitech/biblio/22570085','SCIGOV-STC'); return false;" href="https://www.osti.gov/scitech/biblio/22570085"><span>WE-AB-BRB-08: Progress Towards a 2D OSL Dosimetry <span class="hlt">System</span> Using <span class="hlt">Al</span>2O3:C Films</span></a></p> <p><a target="_blank" href="http://www.osti.gov/scitech">SciTech Connect</a></p> <p>Ahmed, M F; Yukihara, E; Schnell, E; Ahmad, S; Akselrod, M; Brons, S; Greilich, S; Jakel, O; Osinga, J</p> <p>2015-06-15</p> <p>Purpose: To develop a 2D dosimetry <span class="hlt">system</span> based on the optically stimulated luminescence (OSL) of <span class="hlt">Al</span>{sub 2}O{sub 3}:C films for medical applications. Methods: A 2D laser scanning OSL reader was built for readout of newly developed <span class="hlt">Al</span>2O3:C films (Landauer Inc.). An image reconstruction algorithm was developed to correct for inherent effects introduced by reader design and detector properties. The <span class="hlt">system</span> was tested using irradiations with photon and carbon ion beams. A calibration was obtained using a 6 MV photon beam from clinical accelerator and the dose measurement precision was tested using a range of doses and different dose distributions (flat field and wedge field). The dynamic range and performance of the <span class="hlt">system</span> in the presence of large dose gradients was also tested using 430 MeV/u {sup 12}C single and multiple pencil beams. All irradiations were performed with Gafchromic EBT3 film for comparison. Results: Preliminary results demonstrate a near-linear OSL dose response to photon fields and the ability to measure dose in dose distributions such as flat field and wedge field. Tests using {sup 12}C pencil beam demonstrate ability to measure doses over four orders of magnitude. The dose profiles measured by the OSL film generally agreed well with that measured by the EBT3 film. The OSL image signal-to-noise ratio obtained in the current conditions require further improvement. On the other hand, EBT3 films had large uncertainties in the low dose region due to film-to-film or intra-film variation in the background. Conclusion: A 2D OSL dosimetry <span class="hlt">system</span> was developed and initial tests have demonstrated a wide dynamic range as well as good agreement between the delivered and measured doses. The low background, wide dynamic range and wide range of linearity in dose response observed for the <span class="hlt">Al</span>{sub 2}O{sub 3}:C OSL film can be beneficial for dosimetry in radiation therapy applications, especially for small field dosimetry. This work has been funded by Landauer Inc. Dr</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('https://ntrs.nasa.gov/search.jsp?R=19840044028&hterms=M2c&qs=N%3D0%26Ntk%3DAll%26Ntx%3Dmode%2Bmatchall%26Ntt%3DM2c','NASA-TRS'); return false;" href="https://ntrs.nasa.gov/search.jsp?R=19840044028&hterms=M2c&qs=N%3D0%26Ntk%3DAll%26Ntx%3Dmode%2Bmatchall%26Ntt%3DM2c"><span>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.dtic.mil/docs/citations/ADA616578','DTIC-ST'); return false;" href="http://www.dtic.mil/docs/citations/ADA616578"><span>Fundamental Investigation of the Microstructural Parameters to Improve Dynamic Response in <span class="hlt">Al</span>-Cu Model <span class="hlt">System</span></span></a></p> <p><a target="_blank" href="https://publicaccess.dtic.mil/psm/api/service/search/search">DTIC Science & Technology</a></p> <p></p> <p>2014-05-01</p> <p>better engineer and optimize the properties of aluminum alloys for high strain rate applications.The composition of these alloys are shown in Table 4...rate application aluminum alloys . In A. Bajaj, P. Zavattieri, M. Koslowski, & T. Siegmund (Eds.). Proceedings of the Society of Engineering Science...chemistry on deformation in a series of model aluminum alloy <span class="hlt">systems</span> were investigated in an attempt to understand the dominant features responsible for the</p> </li> <li> <p><a target="_blank" onclick="trackOutboundLink('http://adsabs.harvard.edu/abs/2015GeCoA.158..245D','NASAADS'); return false;" href="http://adsabs.harvard.edu/abs/2015GeCoA.158..245D"><span>Isotopic mass fractionation laws for magnesium and their effects on 26<span class="hlt">Al</span>-26Mg systematics in solar <span class="hlt">system</span> materials</span></a></p> <p><a target="_blank" href="http://adsabs.harvard.edu/abstract_service.html">NASA Astrophysics Data System (ADS)</a></p> <p>Davis, Andrew M.; Richter, Frank M.; Mendybaev, Ruslan A.; Janney, Philip E.; Wadhwa, Meenakshi; McKeegan, Kevin D.</p> <p>2015-06-01</p> <p>Magnesium isotope ratios are known to vary in solar <span class="hlt">system</span> objects due to the effects of 26<span class="hlt">Al</span> decay to 26Mg and mass-dependent fractionation, but anomalies of nucleosynthetic origin must also be considered. In order to infer the amount of enhancement of 26Mg/24Mg due to 26<span class="hlt">Al</span> decay or to resolve small nucleogenetic anomalies, the exact relationship between 26Mg/24Mg and 25Mg/24Mg ratios due to mass-dependent fractionation, the mass-fractionation "law", must be accurately known so that the 25Mg/24Mg ratio can be used to correct the 26Mg/24Mg ratio for mass fractionation. Mass-dependent fractionation in mass spectrometers is reasonably well characterized, but not necessarily fully understood. It follows a simple power fractionation law, sometimes referred to as the "exponential law". In contrast, mass fractionation in nature, in particular that due to high temperature evaporation that likely caused the relatively large effects observed in calcium-, aluminum-rich inclusions (CAIs), is reasonably well understood, but mass-fractionation laws for magnesium have not been explored in detail. The magnesium isotopic compositions of CAI-like evaporation residues produced in a vacuum furnace indicate that the slope on a log 25Mg/24Mg vs. log 26Mg/24Mg plot is ∼0.5128, and different from those predicted by any of the commonly used mass-fractionation laws. Evaporation experiments on forsterite-rich bulk compositions give exactly the same slope, indicating that the measured mass-fractionation law for evaporation of magnesium is applicable to a wide range of bulk compositions. We discuss mass-fractionation laws and the implications of the measured fractionation behavior of magnesium isotopes for 26<span class="hlt">Al</span>-26Mg chronology.</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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