Sample records for manifestations rhumatologiques revelatrices

  1. Manifestations rhumatologiques paranéoplasiques en dehors de l'ostéoarthropathie hypertrophiante

    Microsoft Academic Search

    C. Masson; B. Bouvard; M. Audran

    2005-01-01

    Paraneoplastic rheumatic syndromes (PRS), as any paraneoplastic syndrome, induce signs or symptoms related to a known or an unrecognized tumour, cancer, or haemopathy, often malignant but not always. The chronologic relationship between the neoplastic process and the symptomatology is essential. PRS is not due to a tumour-induced compression or a synovial or bone peri-articular metastatic invasion. They differ from pre-neoplastic

  2. What patients want from the treatment of Dupuytren's disease--is the Unité Rhumatologique des Affections de la Main (URAM) scale relevant?

    PubMed

    Rodrigues, J N; Zhang, W; Scammell, B E; Davis, T R C

    2015-02-01

    Patients awaiting surgery for Dupuytren's contractures listed the problems from their Dupuytren's disease that had led them to request surgery and their relative priorities of the results of surgery. The listed problems were compared with the nine items comprising the Unité Rhumatologique des Affections de la Main (URAM) Dupuytren's disease-specific outcome measure. One hundred and ten patients reported 278 problems. Ninety-four different combinations of problems were provided by patients, and over half (55%) of the problems were not captured by items in the URAM scale. The long-term outcome from surgery was relatively more important to patients than speed of recovery. The URAM tool may not be relevant to patients in the UK, and further assessment of its face validity is required before it is used. PMID:24563499

  3. 40 CFR 262.21 - Manifest tracking numbers, manifest printing, and obtaining manifests.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... Manifest tracking numbers, manifest printing, and obtaining manifests. 262.21... Manifest tracking numbers, manifest printing, and obtaining manifests. ...the registrant plans to undertake in printing, distributing, and using its...

  4. Paraneoplastic manifestations in children

    Microsoft Academic Search

    Jan H. de Graaf; Rienk Y. J. Tamminga; Willem A. Kamps

    1994-01-01

    Paraneoplastic manifestations are signs and symptoms observed in patients with cancer, distant from the tumour or its metastases and not caused by invasion, obstruction or bulk mass. In children with cancer, paraneoplastic manifestations are rare and distinct from those observed in adults. Knowledge about paraneoplastic manifestations can be of great clinical importance because they may be the presenting sign of

  5. Heart Manifestations in TSC

    MedlinePLUS

    ... the inheritance pattern of TSC is autosomal dominant (meaning the offspring of an affected person has a 50% chance of inheriting TSC) with variable expression (meaning that the manifestations may differ between affected persons), ...

  6. Thermodynamics in 'Manifest Reality'

    SciTech Connect

    Hankey, Alex [Meru Research Institute, Bucks, 1985 (United Kingdom)

    2010-12-22

    D'Espagnat's proof that the universe is not a 'strongly objective reality' demands that all physical processes are reconsidered in that light. D'Espagnat suggests a 'Veiled Reality' as a suitable alternative. The most economical way to achieve that is to demand that 'information production' at a quantum level creates the basis for self-consistent perception of a world of macroscopic, 'manifest' entities, as opposed to self-existent objects. Such a 'manifest reality' fulfils both Wheeler's attempt at an 'IT-from-BIT' programme, and Zeilinger's suggestion that 'information is primary'.

  7. Immunologic manifestations of autophagy.

    PubMed

    Deretic, Vojo; Kimura, Tomonori; Timmins, Graham; Moseley, Pope; Chauhan, Santosh; Mandell, Michael

    2015-01-01

    The broad immunologic roles of autophagy span innate and adaptive immunity and are often manifested in inflammatory diseases. The immune effects of autophagy partially overlap with its roles in metabolism and cytoplasmic quality control but typically expand further afield to encompass unique immunologic adaptations. One of the best-appreciated manifestations of autophagy is protection against microbial invasion, but this is by no means limited to direct elimination of intracellular pathogens and includes a stratified array of nearly all principal immunologic processes. This Review summarizes the broad immunologic roles of autophagy. Furthermore, it uses the autophagic control of Mycobacterium tuberculosis as a paradigm to illustrate the breadth and complexity of the immune effects of autophagy. PMID:25654553

  8. Cutaneous Manifestations of Hyperthyroidism

    Microsoft Academic Search

    Clara-Dina Cokonis; Carrie W. Cobb; Warren R. Heymann; Chad M. Hivnor

    \\u000a The key to diagnosing hyperthyroidism from a dermatologic perspective is based on having a high index of suspicion that excess\\u000a thyroid hormone is responsible for the patient's signs and symptoms. As there are no definitive cutaneous manifestations of\\u000a hyperthyroidism, a careful review of systems may yield important clinical clues to the diagnosis: Is the patient intolerant\\u000a of heat? Has there

  9. Tubercular lymphadenitis: clinical manifestations.

    PubMed

    Seth, V; Kabra, S K; Jain, Y; Semwal, O P; Mukhopadhyaya, S; Jensen, R L

    1995-01-01

    One hundred and thirteen children suffering from tubercular lymphadenitis proven histopathologically, were studied for clinical and laboratory features. Age distribution was equal in all age groups except during infancy when it was rare. Sex ratio indicates a male preponderance with male to female in the ratio of 1.5:1 (67:46). Family history of contact with known tuberculous patient was positive in 19 (17%) children, 44 (40%) had received BCG, and 98 (88%) were either normal nourished or had mild malnutrition. Cervical, axillary and inguinal nodes were found in 90 (80%), 14 (12%), and 8 (7%) children respectively. Epitrochlear node was found in only 1 child. 11 (10%) children had discharging sinus, all being in cervical group. The consistency of nodes was firm in 98 (87%), fluctuation was present in 15 (13%). In 87 (77%) lymph nodes were matted. Hepatomegaly of more than 2 cm was present in 13 (11%) and spleen was enlarged (> 2 cm) in 4 (4%) only: Mantoux test was positive in 96 (85%) children and chest X-ray was abnormal in 25 (22%) cases. Findings suggest that tubercular adenitis occurs in all age groups with equal frequency. It can occur in vaccinated children also. It may be a sole manifestation of tubercular infection. The cervical nodes are predominantly involved. There is no typical location of nodes in individual groups but multiplicity and matting of nodes are characteristic features of tubercular adenitis in children. PMID:10829923

  10. Cutaneous Manifestations of Crohn Disease.

    PubMed

    Hagen, Joshua W; Swoger, Jason M; Grandinetti, Lisa M

    2015-07-01

    Awareness of the extraintestinal manifestations of Crohn disease is increasing in dermatology and gastroenterology, with enhanced identification of entities that range from granulomatous diseases recapitulating the underlying inflammatory bowel disease to reactive conditions and associated dermatoses. In this review, the underlying etiopathology of Crohn disease is discussed, and how this mirrors certain skin manifestations that present in a subset of patients is explored. The array of extraintestinal manifestations that do not share a similar pathology, but which are often seen in association with inflammatory bowel disease, is also discussed. Treatment and pathogenetic mechanisms, where available, are discussed. PMID:26143422

  11. Hepatic manifestations during amoebic dysentery

    Microsoft Academic Search

    S. Ramachandran; C. N. A. Rajapakse; R. de Saram; S. Sivalingam

    1973-01-01

    A variety of hepatic manifestations may occur during attacks of acute amoebic dysentery, conforming to clearly defined clinical groups.The incidence of definite hepatic involvement presenting as cases of hepatic amoebiasis with or without demonstrable pus or with tender hepatomegaly was 57·5%.While it is possible that the cases presenting with hepatic manifestations without demonstrable pus are due to small deep-seated abscesses,

  12. [Extraesophageal manifestations of gastroesophageal reflux].

    PubMed

    Gribajcevi?, Mehmed

    2003-01-01

    The gastroesophageal reflux disease (GERB) is one from the most frequent disease of the upper gastrointestinal system. Extraesophageal manifestations GERB occur most often as lung manifestations, ORL discomforts, and as noncardial pain in breasts. GERB is associated with the various respiratory diseases and symptoms as are the bronchial asthma, cough, wheezing. The episodes of the sour reflux into the esophagus, specially in children, can provoke the symptoms discapper mainly in all patients who have used the antisecretoral therapy. PMID:12827964

  13. Manifestations dysimmunitaires associées aux lymphomes

    Microsoft Academic Search

    F. Jardin; H. Lévesque; H. Tilly

    2005-01-01

    Purpose. – A wide spectrum of auto-immune manifestations is frequently reported in non-Hodgkin’s lymphoma (NHL). The purpose of the review is to describe the immune manifestations observed in NHL, according to their histological subtype and to discuss the current physiopathological hypothesis with their therapeutic relevance.Current knowledge and key points. – Most of the organs can be targeted by an immune

  14. Neurological manifestations of HIV infection.

    PubMed

    Udgirkar, Vardhaman S; Tullu, Milind S; Bavdekar, Sandeep B; Shaharao, Vijayalaxmi B; Kamat, Jaishree R; Hira, Priya R

    2003-03-01

    Case records of HIV infected patients were analyzed for identifying neurological manifestations. Eight patients (7 males) were identified to have probable HIV encephalopathy (in a period of 24 months) as per the CDC revised classification system. Their ages ranged from one year to ten years. The neurological manifestations noted included-developmental delay (2 cases), seizures (6 cases), acute onset alteration of sensorium (4 cases), aphasia (2 cases), loss of vision (2 cases), focal neurological deficits (6 cases), brisk deep tendon reflexes (7 cases), extensor plantar responses (5 cases) and signs of cerebellar dysfunction (2 cases). Other clinical features included growth failure, microcephaly, fever, lymphadenopathy, hepatomegaly, splenomegaly, pneumonia, otorrhea and oral candidiasis. Cerebrospinal fluid studies were normal. The neuroimaging features included cerebral atrophy and ventricular dilatation, cerebral infarction, basal ganglia calcification and cerebellar atrophy. Childhood HIV infection may have a variety of neurological abnormalities. HIV infection should be suspected in children presenting with unexplained neurological manifestations and growth failure. PMID:12657756

  15. Pharyngoesophageal manifestations of epidermolysis bullosa.

    PubMed

    Kabakian, H A; Dahmash, N S

    1978-01-01

    Two cases of a rare hereditary disease, epidermolysis bullosa, congenital and acquired, are presented. The disease manifests by vesicular eruptions over the skin and mucous membrane of the oropharynx and oesophagus, alternating with sympton-free periods. The recurrent crops of vesicles heal by scarring and progressive stenosis of the pharynx and oesophagus. Although mucosal involvement is a less common and a late manifestation of the disease, it may occur in relatively mild cases and relatively early in the course of the disease. It is important that the radiologist be familiar with the pharyngoesophageal manifestations since the patient may present with dysphagia during an interval free of cutaneous lesions. The barium swallow appearance of stricturing with obstruction is illustrated. PMID:624207

  16. Cutaneous manifestations of viral hepatitis.

    PubMed

    Akhter, Ahmed; Said, Adnan

    2015-02-01

    There are several extrahepatic cutaneous manifestations associated with hepatitis B and hepatitis C virus infection. Serum sickness and polyarteritis nodosa are predominantly associated with hepatitis B infection, whereas mixed cryoglobulinemia associated vasculitis and porphyria cutanea tarda are more frequently seen in hepatitis C infection. The clinico-pathogenic associations of these skin conditions are not completely defined but appear to involve activation of the host immune system including the complement system. Management of the aforementioned cutaneous manifestations of viral hepatitis is often similar to that done in cases without viral hepatitis, with control of immune activation being a key strategy. In cases associated with hepatitis B and C, control of viral replication with specific antiviral therapy is also important and associated with improvement in most of the associated clinical manifestations. PMID:25809574

  17. Gastrointestinal Manifestations of Cystic Fibrosis.

    PubMed

    Kelly, Thomas; Buxbaum, James

    2015-07-01

    Gastrointestinal symptoms of cystic fibrosis are the most important non-pulmonary manifestations of this genetic illness. Pancreatic manifestations include acute and chronic pancreatitis as well as pancreas insufficiency resulting in malnutrition. Complications in the gastrointestinal lumen are diverse and include distal intestinal obstruction syndrome (DIOS), meconium ileus, intussusception, and constipation; biliary tract complications include focal biliary cirrhosis and cholangiectasis. The common pathophysiology is the inspissation of secretions in the hollow structures of the gastrointestinal tract. Improved survival of CF patients mandates that the adult gastroenterologist be aware of the presentation and treatment of pancreatic, luminal, and hepatobiliary CF complications. PMID:25648641

  18. Oral Manifestations of Viral Diseases

    Microsoft Academic Search

    Denis P. Lynch

    other chapters dealing with specific viruses. Second, the clinical oral manifestations of such infections are described, with an emphasis on the differential diagnosis of specific oral viral lesions. Third, the methods used in the diagno- sis of oral viral lesions are presented. Fourth, a summary of current therapeutic management strategies is presented, along with their relation- ship to long-term prognosis.

  19. Skin Manifestations of Internal Malignancy

    PubMed Central

    Donsky, Howard J.

    1978-01-01

    Although some of the skin manifestations of internal malignancy are relatively rare, their identification may often be made at a crucial time, when intervention may save a life. This article classifies such signs and describes those which may be seen in family practice. ImagesFig. 1Fig. 2Fig. 3Fig. 4Fig. 5Fig. 6Fig. 7Fig. 8 PMID:21301498

  20. Laryngeal Manifestations of Rheumatoid Arthritis

    PubMed Central

    Hamdan, A. L.; Sarieddine, D.

    2013-01-01

    Rheumatoid arthritis is a destructive autoimmune disease that affects 3% of the adult population. It is characterized by the formation of both articular and extra-articular lesions with predilection for small joints. There are ubiquitous reports on the head and neck manifestations of RA with emphasis on the larynx. The laryngeal presenting features of this systemic disease may mimic a plethora of medical conditions, inflammatory and neoplastic. The main phonatory and respiratory symptoms are often subtle and misleading. This paper represents a literature review of the laryngeal manifestations of RA with emphasis on the clinical symptoms, laryngeal findings, diagnosis, and treatment. An early diagnosis of laryngeal involvement may prevent drastic complications. PMID:23864939

  1. Thoracic radiologic manifestations of melioidosis.

    PubMed

    Burivong, Wanaporn; Wu, Xiaohua; Saenkote, Wipawadee; Stern, Eric J

    2012-01-01

    Melioidosis (Burkholderia pseudomallei) is a gram-negative bacterial infection that is highly endemic in Southeast Asia and Oceania. Pulmonary disease is the most common form of involvement. The clinical-radiologic thoracic manifestations of melioidosis can be classified as acute, subacute, subclinical, and chronic forms. Radiographic findings include nodular, alveolar, or mixed infiltration/consolidation with or without cavities. Pleural effusion, pneumothorax, and pericardial involvement can be seen. Melioidosis can easily be confused with other infections, especially tuberculosis. Suspicion of this disease in the proper clinical radiologic setting is important for early diagnosis and treatment. In this article, we provide a broad clinical overview of melioidosis, review the radiologic thoracic manifestations of melioidosis with appropriate clinical correlation, as well as compare and contrast the imaging findings of thoracic melioidosis with other similar pulmonary infections. PMID:23009770

  2. Multisystem manifestations of mitochondrial disorders

    Microsoft Academic Search

    Stefano Di Donato

    2009-01-01

    Mitochondria are cytoplasmic organelles in eukaryotic cells that accomplish several distinct vital functions, including oxidative\\u000a phosphorylation, metabolic anaplerotic and degradative pathways, and integration of signaling for apoptosis. Impaired oxidative\\u000a phosphorylation, the common final pathway of mitochondrial metabolism, results in a variety of clinical manifestations, and\\u000a the term mitochondrial disorders is currently ascribed to (mostly) genetic diseases of the respiratory chain

  3. 21 CFR 11.50 - Signature manifestations.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ...2010-04-01 false Signature manifestations. 11...ELECTRONIC RECORDS; ELECTRONIC SIGNATURES Electronic Records § 11.50 Signature manifestations. ...The date and time when the signature was executed; and...

  4. 21 CFR 11.50 - Signature manifestations.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ...2012-04-01 false Signature manifestations. 11...ELECTRONIC RECORDS; ELECTRONIC SIGNATURES Electronic Records § 11.50 Signature manifestations. ...The date and time when the signature was executed; and...

  5. 21 CFR 11.50 - Signature manifestations.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ...2013-04-01 false Signature manifestations. 11...ELECTRONIC RECORDS; ELECTRONIC SIGNATURES Electronic Records § 11.50 Signature manifestations. ...The date and time when the signature was executed; and...

  6. 21 CFR 11.50 - Signature manifestations.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ...2014-04-01 false Signature manifestations. 11...ELECTRONIC RECORDS; ELECTRONIC SIGNATURES Electronic Records § 11.50 Signature manifestations. ...The date and time when the signature was executed; and...

  7. 21 CFR 11.50 - Signature manifestations.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ...2011-04-01 false Signature manifestations. 11...ELECTRONIC RECORDS; ELECTRONIC SIGNATURES Electronic Records § 11.50 Signature manifestations. ...The date and time when the signature was executed; and...

  8. Cardiac manifestations in systemic sclerosis

    PubMed Central

    Lambova, Sevdalina

    2014-01-01

    Primary cardiac involvement, which develops as a direct consequence of systemic sclerosis (SSc), may manifest as myocardial damage, fibrosis of the conduction system, pericardial and, less frequently, as valvular disease. In addition, cardiac complications in SSc may develop as a secondary phenomenon due to pulmonary arterial hypertension and kidney pathology. The prevalence of primary cardiac involvement in SSc is variable and difficult to determine because of the diversity of cardiac manifestations, the presence of subclinical periods, the type of diagnostic tools applied, and the diversity of patient populations. When clinically manifested, cardiac involvement is thought to be an important prognostic factor. Profound microvascular disease is a pathognomonic feature of SSc, as both vasospasm and structural alterations are present. Such alterations are thought to predict macrovascular atherosclerosis over time. There are contradictory reports regarding the prevalence of atherosclerosis in SSc. According to some authors, the prevalence of atherosclerosis of the large epicardial coronary arteries is similar to that of the general population, in contrast with other rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus. However, the level of inflammation in SSc is inferior. Thus, the atherosclerotic process may not be as aggressive and not easily detectable in smaller studies. Echocardiography (especially tissue Doppler imaging), single-photon emission computed tomography, magnetic resonance imaging and cardiac computed tomography are sensitive techniques for earlier detection of both structural and functional scleroderma-related cardiac pathologies. Screening for subclinical cardiac involvement via modern, sensitive tools provides an opportunity for early diagnosis and treatment, which is of crucial importance for a positive outcome. PMID:25276300

  9. Extraintestinal Manifestations of Inflammatory Bowel Disease

    PubMed Central

    Burakoff, Robert

    2011-01-01

    Extraintestinal manifestations of inflammatory bowel disease are prevalent in both ulcerative colitis and Crohn's disease. The most common manifestations involve the musculoskeletal and dermatologic systems. Other manifestations involve the hepatopan-creatobiliary system (eg, primary sclerosing cholangitis) as well as the ocular, renal, and pulmonary systems. A multidisciplinary team approach is often needed for effective management, and emergency situations require prompt evaluation. PMID:21857821

  10. Coronal manifestations of preflare activity

    NASA Technical Reports Server (NTRS)

    Schmahl, E. J.; Webb, D. F.; Woodgate, B.; Waggett, P.; Bentley, R.; Hurford, G.; Schadee, A.; Schrijver, J.; Harrison, R.; Martens, P.

    1986-01-01

    A variety of coronal manifestations of precursors or preheating for flares are discussed. Researchers found that almost everyone with a telescope sees something before flares. Whether an all-encompassing scenario will ever be developed is not at all clear at present. The clearest example of preflare activity appears to be activated filaments and their manifestations, which presumably are signatures of a changing magnetic field. But researchers have seen two similar eruptions, one without any evidence of emerging flux (Kundu et al., 1985) and the other with colliding poles (Simon et al., 1984). While the reconnection of flux is generally agreed to be required to energize a flare, the emergence of flux from below (at least on short timescales and in compact regions) does not appear to be a necessary condition. In some cases the cancelling of magnetic flux (Martin, 1984) by horizontal motions instead may provide the trigger (Priest, 1985) Researchers found similarities and some differences between these and previous observations. The similarities, besides the frequent involvement of filaments, include compact, multiple precursors which can occur both at and near (not at) the flare site, and the association between coronal sources and activity lower in the atmosphere (i.e., transition zone and chromosphere).

  11. Cardiac manifestations in Behcet's disease

    PubMed Central

    Demirelli, Selami; Degirmenci, Husnu; Inci, Sinan; Arisoy, Arif

    2015-01-01

    Summary Behcet's disease (BD) is a chronic inflammatory disorder, with vasculitis underlying the pathophysiology of its multisystemic effects. Venous pathology and thrombotic complications are hallmarks of BD. However, it has been increasingly recognised that cardiac involvement and arterial complications are also important aspects of the course of the disease. Cardiac lesions include pericarditis, endocarditis, intracardiac thrombosis, myocardial infarction, endomyocardial fibrosis, and myocardial aneurysm. Treatment of cardiovascular involvement in BD is largely empirical, and is aimed towards suppressing the vasculitis. The most challenging aspect seems to be the treatment of arterial aneurysms and thromboses due to the associated risk of bleeding. When the prognosis of cardiac involvement in BD is not good, recovery can be achieved through oral anticoagulation, immunosuppressive therapy, and colchicine use. In this review, we summarise the cardiovascular involvement, different manifestations, and treatment of BD. PMID:25984424

  12. Oral manifestations in transplant patients.

    PubMed

    Nappalli, Deepika; Lingappa, Ashok

    2015-01-01

    Organ transplantation is a widely undertaken procedure and has become an important alternative for the treatment of different end-stage organ diseases that previously had a poor prognosis. The field of organ transplant and hematopoietic stem cell transplant is developing rapidly. The increase in the number of transplant recipients also has an impact on oral and dental services. Most of the oral problems develop as a direct consequence of drug-induced immunosuppression or the procedure itself. These patients may present with oral complaints due to infections or mucosal lesions. Such lesions should be identified, diagnosed, and treated. New treatment strategies permit continuous adaptation of oral care regimens to the changing scope of oral complications. The aim of this review is to analyze those oral manifestations and to discuss the related literature. PMID:26005458

  13. Oral manifestations in transplant patients

    PubMed Central

    Nappalli, Deepika; Lingappa, Ashok

    2015-01-01

    Organ transplantation is a widely undertaken procedure and has become an important alternative for the treatment of different end-stage organ diseases that previously had a poor prognosis. The field of organ transplant and hematopoietic stem cell transplant is developing rapidly. The increase in the number of transplant recipients also has an impact on oral and dental services. Most of the oral problems develop as a direct consequence of drug-induced immunosuppression or the procedure itself. These patients may present with oral complaints due to infections or mucosal lesions. Such lesions should be identified, diagnosed, and treated. New treatment strategies permit continuous adaptation of oral care regimens to the changing scope of oral complications. The aim of this review is to analyze those oral manifestations and to discuss the related literature. PMID:26005458

  14. Hematologic manifestations of celiac disease

    PubMed Central

    Halfdanarson, Thorvardur R.; Litzow, Mark R.; Murray, Joseph A.

    2007-01-01

    Celiac disease is a common systemic disorder that can have multiple hematologic manifestations. Patients with celiac disease may present to hematologists for evaluation of various hematologic problems prior to receiving a diagnosis of celiac disease. Anemia secondary to malabsorption of iron, folic acid, and/or vitamin B12 is a common complication of celiac disease and many patients have anemia at the time of diagnosis. Celiac disease may also be associated with thrombocytosis, thrombocytopenia, leukopenia, venous thromboembolism, hyposplenism, and IgA deficiency. Patients with celiac disease are at increased risk of being diagnosed with lymphoma, especially of the T-cell type. The risk is highest for enteropathy-type T-cell lymphoma (ETL) and B-cell lymphoma of the gut, but extraintestinal lymphomas can also be seen. ETL is an aggressive disease with poor prognosis, but strict adherence to a gluten-free diet may prevent its occurrence. PMID:16973955

  15. Cardiac manifestations in Behcet's disease.

    PubMed

    Demirelli, Selami; Degirmenci, Husnu; Inci, Sinan; Arisoy, Arif

    2015-05-01

    Behcet's disease (BD) is a chronic inflammatory disorder, with vasculitis underlying the pathophysiology of its multisystemic effects. Venous pathology and thrombotic complications are hallmarks of BD. However, it has been increasingly recognised that cardiac involvement and arterial complications are also important aspects of the course of the disease. Cardiac lesions include pericarditis, endocarditis, intracardiac thrombosis, myocardial infarction, endomyocardial fibrosis, and myocardial aneurysm. Treatment of cardiovascular involvement in BD is largely empirical, and is aimed towards suppressing the vasculitis. The most challenging aspect seems to be the treatment of arterial aneurysms and thromboses due to the associated risk of bleeding. When the prognosis of cardiac involvement in BD is not good, recovery can be achieved through oral anticoagulation, immunosuppressive therapy, and colchicine use. In this review, we summarise the cardiovascular involvement, different manifestations, and treatment of BD. PMID:25984424

  16. MUCOCUTANEOUS MANIFESTATIONS OF CHIKUNGUNYA FEVER

    PubMed Central

    Bandyopadhyay, Debabrata; Ghosh, Sudip Kumar

    2010-01-01

    Chikungunya fever (CF) is an arboviral acute febrile illness transmitted by the bite of infected Aedes mosquitoes. After a quiescence of more than three decades, CF has recently re-emerged as a major public health problem of global scale. CF is characterized by an acute onset of high fever associated with a severe disabling arthritis often accompanied by prominent mucocutaneous manifestations. The disease is usually self-limiting, but the joint symptoms and some of the cutaneous features may persist after the defervescence. A wide range of mucocutaneous changes has been described to occur in association with CF during the current epidemic. Besides a morbilliform erythema, hyperpigmentation, xerosis, excoriated papules, aphthous-like ulcers, vesiculobullous and lichenoid eruptions, and exacerbation of pre-existing or quiescent dermatoses had been observed frequently. These unusual features may help in the clinical differential diagnosis of acute viral exanthems mimicking CF. PMID:20418982

  17. Hepatic manifestations of metabolic syndrome.

    PubMed

    Medina-Santillán, Roberto; López-Velázquez, Jorge A; Chávez-Tapia, Norberto; Torres-Villalobos, Gonzalo; Uribe, Misael; Méndez-Sánchez, Nahum

    2013-03-01

    The prevalence of metabolic syndrome is growing around the world at an alarming rate. Obesity involves a plethora of molecules that predispose individuals to an inflammatory state and various metabolic complications. Dysregulation of nutrient metabolism is a key step during the progression of chronic liver disease that induces an inflammatory state, cellular damage, and impaired hepatic insulin signaling, which leads to insulin resistance. Insulin resistance arises from multiple defects in the liver, adipose tissues, and muscle signaling, which leads to a failure to suppress hepatic gluconeogenesis and glycogenolysis, thereby enhancing fat accumulation in the hepatocytes via increased lipolysis and increased hepatic synthesis of triglycerides. This metabolic condition also increases the frequency of other comorbidities such as liver and biliary diseases. Nonalcoholic fatty liver disease is the hepatic expression of metabolic syndrome, which comprises a spectrum of clinical and histological events ranging from simple and benign fatty liver to steatohepatitis, which is characterized by the abnormal activation of pathways leading to an aggressive inflammatory condition. This pathological state may progress to more severe damage known as cirrhosis, which endangers the anatomy and function of liver tissue. In addition, a small group of patients with end-stage liver disease may develop hepatocellular carcinoma and finally death. By contrast, cholesterol gallstone disease is a common metabolic disease, and is considered to be the main biliary indicator of metabolic syndrome. This review provides a detailed summary of the hepatic manifestations associated with metabolic syndrome. Copyright © 2013 John Wiley & Sons, Ltd. PMID:23471889

  18. Ocular manifestations of frontonasal dysplasia.

    PubMed

    Roarty, J D; Pron, G E; Siegel-Bartelt, J; Posnick, J C; Buncic, J R

    1994-01-01

    The ophthalmologic findings associated with frontonasal dysplasia have not been defined previously in a large series of untreated children. We reviewed the ophthalmic manifestations of a series of patients with frontonasal dysplasia who were seen as part of their craniofacial evaluation. All had undergone a complete ophthalmologic examination before any manipulation of either the orbits or the soft tissues of the orbital contents. From 1986 to 1991, 23 patients with frontonasal dysplasia were seen; ophthalmologic abnormalities were found in 20 (87 percent). Abnormalities included significant refractive errors, strabismus, nystagmus, and eyelid ptosis. Three patients had amblyopia, a treatable cause of visual loss, from strabismus or anisometropia. Ten eyes in seven patients (30 percent) had severe structural anomalies, such as optic nerve hypoplasia, optic nerve colobomas, microphthalmia, cataract, corneal dermoid, or inflammatory retinopathy, that resulted in an acuity of 20/100 or worse. The high incidence of ocular abnormalities indicates that early assessment by an ophthalmologist should be part of the initial evaluation of patients with frontonasal dysplasia to detect treatable visual or ocular problems. PMID:8278482

  19. Pulmonary manifestations of ankylosing spondylitis.

    PubMed

    Kanathur, Naveen; Lee-Chiong, Teofilo

    2010-09-01

    Ankylosing spondylitis, a chronic multisystem inflammatory disorder, can present with articular and extra-articular features. It can affect the tracheobronchial tree and the lung parenchyma, and respiratory complications include chest wall restriction, apical fibrobullous disease with or without secondary pulmonary superinfection, spontaneous pneumothorax, and obstructive sleep apnea. Ankylosing spondylitis is a common cause of pulmonary apical fibrocystic disease; early involvement may be unilateral or asymmetrical, but most cases eventually consist of bilateral apical fibrobullous lesions, many of which are progressive with coalescence of the nodules, formation of cysts and cavities, fibrosis, and bronchiectasis. Mycobacterial or fungal superinfection of the upper lobe cysts and cavities occurs commonly. Aspergillus fumigatus is the most common pathogen isolated, followed by various species of mycobacteria. Prognosis of patients with fibrobullous apical lesions is mainly determined by the presence, extent, and severity of superinfection. Pulmonary function test results are nonspecific and generally parallel the severity of parenchymal involvement. A restrictive ventilatory impairment can develop in patients with ankylosing spondylitis because of either fusion of the costovertebral joints and ankylosis of the thoracic spine or anterior chest wall involvement. Chest radiographic findings may mirror the severity of clinical involvement. Pulmonary parenchymal disease is typically progressive, and cyst formation, cavitation, and fibrosis are seen in advanced cases. No treatment has been shown to alter the clinical course of apical fibrobullous disease. Although several antiinflammatory agents, such as infliximab, etanercept, and adalimumab, are being used to treat ankylosing spondylitis, their effects on pulmonary manifestations are unclear. PMID:20692546

  20. Cutaneous manifestations of Taxol therapy.

    PubMed

    Link, C J; Sarosy, G A; Kohn, E C; Christian, M C; Davis, P; Adamo, D O; Reed, E

    1995-01-01

    Taxol is a novel chemotherapeutic agent that has produced substantial responses in early clinical studies [1]. Taxol has excellent activity in a number of malignancies based on recently completed clinical trials, including a 30% response rate in platinum-refractory ovarian cancer patients [2-5]. We are currently conducting trials of dose-intense taxol with granulocyte colony stimulating factor (G-CSF) support in relapsed or refractory ovarian cancer patients. Such dose intensification produces a major response rate in 50% of patients with this disease [6]. Taxol was supplied in 5 ml ampules (6 mg/ml) in polyethoxylated castor oil (Cremophor EL) 50% and dehydrated alcohol and the dose was diluted in either 0.9% sodium chloride or 5% dextrose at concentrations of 0.6 to 1.2 mg/ml. We have noted 3 patients with previously unreported cutaneous manifestations which we believe are taxol related and also report our overall complication rate with the administration of taxol by peripheral intravenous lines. PMID:8729957

  1. Tuberculosis: clinical manifestations and outcomes.

    PubMed

    Nissapatorn, V; Kuppusamy, I; Anuar, A Khairul; Quek, K F; Latt, H M

    2003-01-01

    A total of 290 HIV/AIDS patients were recruited into this retrospective study, which was carried out at the National Tuberculosis Center (NTBC), Kuala Lumpur. The age range was 18 to 75 years with a mean age of 36.10 (SD +/- 7.44) years. Males outnumbered females by a ratio of 31:1. In this study, the majority of patients were male (96.9%), Malay (47.2%), single (66.9%), unemployed (81%), and smoked (61.4%). The main risk marker identified was injecting drug use (74.5%). The most common clinical manifestations were cough, fever, sputum, lymphadenopathy, and chest infiltrations. More than half of the patients (85.9%) were diagnosed with localized tuberculosis (pulmonary) and the others (14.1%) had extra-pulmonary or disseminated tuberculosis. At the time of this study, the majority of the patients (16.9%) had CD4 cell counts of less than 200 cell/mm3, with a median of 221 cell/mm3. Clinical outcomes demonstrated that among those who survived, 11.0% and 20.7% of the patients had completed treatment either > or = 6 or > or = 9 months, respectively, whereas 54.8% of patients were lost to follow-up, including 0.7% for MDR-TB. Diagnostic criteria for tuberculosis in this study were mainly clinical symptoms/signs and chest x-ray findings (31.0%). PMID:19238668

  2. Esophageal manifestations of celiac disease.

    PubMed

    Lucendo, A J

    2011-09-01

    Celiac disease (CD) may often be associated with various motor disorders affecting the different segments of the digestive tract, including the esophagus. Although it has not been universally reported, some available evidences indicate that pediatric and adult celiac patients could manifest a higher frequency of esophagitis and gastroesophageal reflux disease-related symptoms compared to nonceliac patients. In addition, several published studies have consistently shown the efficacy of a gluten-free diet in rapidly controlling esophageal symptoms and in preventing their recurrence. Since the participation of gluten in the esophageal symptoms of CD seems clear, its intimate mechanisms have yet to be elucidated, and several hypothesis have been proposed, including the specific immune alterations characterizing CD, the reduction in nutrient absorption determining the arrival of intact gluten to distal gastrointestinal segments, and various dysregulations in the function of gastrointestinal hormones and peptides. Recent studies have suggested the existence of a possible relationship between CD and eosinophilic esophagitis, which should be more deeply investigated. PMID:21438963

  3. [Neurological manifestations indicative of brucellosis].

    PubMed

    Gouider, R; Samet, S; Triki, C; Fredj, M; Gargouri, A; el Bahri, F; Ben Ghorbel, I; Kasraoui, A; Mhiri, C; Mrabet, A

    1999-03-01

    Eight patients presented neurological signs secondary to Brucella infection. The clinical presentation was a meningoencephalitis in three cases, a meningoencephalomyelitis in one case, an epiduritis with spinal cord compression in one case, an acute polyradiculoneuritis in two cases and a chronic polyradiculoneuritis in one case. Acoustic nerve was impaired in seven cases. Cerebrospinal fluid (CSF) analysis revealed a lymphocytic meningitis and a high protein concentration in all cases. The agglutination test titers were elevated in the serum and in the CSF of seven patients (> or = 1/80) and two patients respectively. Brucella melitensis culture was disclosed in the blood of one patient and in the CSF of two patients. Three patients were treated by the association cycline and rifampicin whereas a tritherapy including cycline, rifampicin and TMP-SMZ was used in the other cases. Outcome was favorable in seven cases. This study outlines the polymorphism of neurological manifestations due to brucellosis, even in familial cases and this diagnostic must be especially done in Middle East and South Mediterranean countries. PMID:10339791

  4. Teaching about Manifest Destiny: Clarifying the Concept.

    ERIC Educational Resources Information Center

    Chiodo, John J.

    2000-01-01

    Provides information on Manifest Destiny and the expansionist ideals of the United States, especially during the 19th century. Offers a lesson plan that will help students understand the concept of Manifest Destiny, including an assessment activity, how to expand the lesson, an appendix, and references. (CMK)

  5. Systemic manifestations of chronic obstructive pulmonary disease.

    PubMed

    Warwick, Eleanor; Scourfield, Andrew; Quint, Jennifer

    2015-06-01

    Chronic obstructive pulmonary disease is a complex multisystem disease with comorbidities and systemic manifestations that affect respiratory symptoms, exacerbation frequency and mortality. This article gives an overview of these systemic manifestations and their importance, and offers strategies for managing them. PMID:26053902

  6. Systemic manifestations of traumatic brain injury.

    PubMed

    Gaddam, Samson Sujit Kumar; Buell, Thomas; Robertson, Claudia S

    2015-01-01

    Traumatic brain injury (TBI) affects functioning of various organ systems in the absence of concomitant non-neurologic organ injury or systemic infection. The systemic manifestations of TBI can be mild or severe and can present in the acute phase or during the recovery phase. Non-neurologic organ dysfunction can manifest following mild TBI or severe TBI. The pathophysiology of systemic manifestations following TBI is multifactorial and involves an effect on the autonomic nervous system, involvement of the hypothalamic-pituitary axis, release of inflammatory mediators, and treatment modalities used for TBI. Endocrine dysfunction, electrolyte imbalance, and respiratory manifestations are common following TBI. The influence of TBI on systemic immune response, coagulation cascade, cardiovascular system, gastrointestinal system, and other systems is becoming more evident through animal studies and clinical trials. Systemic manifestations can independently act as risk factors for mortality and morbidity following TBI. Some conditions like neurogenic pulmonary edema and disseminated intravascular coagulation can adversely affect the outcome. Early recognition and treatment of systemic manifestations may improve the clinical outcome following TBI. Further studies are required especially in the field of neuroimmunology to establish the role of various biochemical cascades, not only in the pathophysiology of TBI but also in its systemic manifestations and outcome. PMID:25702219

  7. Paraneoplastic cutaneous manifestations: concepts and updates*

    PubMed Central

    da Silva, Josenilson Antônio; Mesquita, Kleyton de Carvalho; Igreja, Ana Carolina de Souza Machado; Lucas, Isabella Cristina Rodrigues Naves; Freitas, Aline Ferreira; de Oliveira, Sandra Maximiano; Costa, Izelda Maria Carvalho; Campbell, Iphis Tenfuss

    2013-01-01

    The skin often signals systemic changes. Some neoplastic diseases that affect internal organs may trigger several cutaneous manifestations. Although these dermatoses are relatively unusual, the recognition of some typical paraneoplastic dermatoses may lead to the early diagnosis of a neoplasm and determine a better prognosis. In this review article, we discuss the paraneoplastic cutaneous manifestations strongly associated with neoplasms, which include acanthosis nigricans maligna, tripe palms, erythema gyratum repens, Bazex syndrome, acquired hypertrichosis lanuginosa, necrolytic migratory erythema, Leser-Trélat sign and paraneoplastic pemphigus. We also review the clinical manifestations of each condition and include updated knowledge on disease pathogenesis. PMID:23538999

  8. [Dermatologic manifestations associated with HIV/AIDS].

    PubMed

    Navarrete-Dechent, Cristián; Ortega, Rinna; Fich, Félix; Concha, Marcela

    2015-02-01

    The ongoing human immunodeficiency virus (HIV) infection epidemic coupled with more efficacious and available treatments has led to a larger number of patients living with HIV or AIDS. As a result, skin manifestations related to HIV/AIDS or its therapy have become increasingly more common and are reported to occur in as many as 95% of patients. Herein, we review the most common HIV/AIDS related cutaneous pathologies and classify them into inflammatory, HAART-associated, neoplastic, and infectious manifestations. Cutaneous manifestations should be promptly recognized and treated by physicians and health care personnel in order to provide optimal care. PMID:25860154

  9. Wegener Granulomatosis: Otologic Manifestation as First Symptom

    PubMed Central

    Costa, Carla Fabiane da; Polanski, Jose Fernando

    2014-01-01

    Introduction?Wegener granulomatosis is a systemic vasculitis affecting small and medium-sized vessels of the upper and lower respiratory tract and kidneys. Objective?To describe a case of Wegener disease with atypical manifestation. Resumed Report?We describe the case of a 50-year-old woman with chronic otitis media and sensorineural hearing loss as the primary symptoms, without other manifestations. Conclusion?In cases of acute ear manifestations with or without hearing loss and with poor response to usual treatments, Wegener granulomatosis should be included among the possible etiologies. After adequate diagnoses and treatment of this rare disease, there was favorable evolution.

  10. Wegener Granulomatosis: Otologic Manifestation as First Symptom.

    PubMed

    Costa, Carla Fabiane da; Polanski, Jose Fernando

    2015-07-01

    Introduction?Wegener granulomatosis is a systemic vasculitis affecting small and medium-sized vessels of the upper and lower respiratory tract and kidneys. Objective?To describe a case of Wegener disease with atypical manifestation. Resumed Report?We describe the case of a 50-year-old woman with chronic otitis media and sensorineural hearing loss as the primary symptoms, without other manifestations. Conclusion?In cases of acute ear manifestations with or without hearing loss and with poor response to usual treatments, Wegener granulomatosis should be included among the possible etiologies. After adequate diagnoses and treatment of this rare disease, there was favorable evolution. PMID:26157503

  11. Genetic alterations in syndromes with oral manifestations.

    PubMed

    Anuthama, Krishnamurthy; Prasad, Harikrishnan; Ramani, Pratibha; Premkumar, Priya; Natesan, Anuja; Sherlin, Herald J

    2013-11-01

    Ever since Gregor Johan Mendel proposed the law of inheritance, genetics has transcended the field of health and has entered all walks of life in its application. Thus, the gene is the pivoting factor for all happenings revolving around it. Knowledge of gene mapping in various diseases would be a valuable tool in prenatally diagnosing the condition and averting the future disability and stigma for the posterity. This article includes an array of genetically determined conditions in patients seen at our college out-patient department with complete manifestation, partial manifestation and array of manifestations not fitting into a particular syndrome. PMID:24379857

  12. Genetic alterations in syndromes with oral manifestations

    PubMed Central

    Anuthama, Krishnamurthy; Prasad, Harikrishnan; Ramani, Pratibha; Premkumar, Priya; Natesan, Anuja; Sherlin, Herald J.

    2013-01-01

    Ever since Gregor Johan Mendel proposed the law of inheritance, genetics has transcended the field of health and has entered all walks of life in its application. Thus, the gene is the pivoting factor for all happenings revolving around it. Knowledge of gene mapping in various diseases would be a valuable tool in prenatally diagnosing the condition and averting the future disability and stigma for the posterity. This article includes an array of genetically determined conditions in patients seen at our college out-patient department with complete manifestation, partial manifestation and array of manifestations not fitting into a particular syndrome. PMID:24379857

  13. Endocrine disorders and the neurologic manifestations

    PubMed Central

    2014-01-01

    The nervous system and the endocrine system are closely interrelated and both involved intimately in maintaining homeostasis. Endocrine dysfunctions may lead to various neurologic manifestations such as headache, myopathy, and acute encephalopathy including coma. It is important to recognize the neurologic signs and symptoms caused by the endocrine disorders while managing endocrine disorders. This article provides an overview of the neurologic manifestations found in various endocrine disorders that affect pediatric patients. It is valuable to think about 'endocrine disorder' as a cause of the neurologic manifestations. Early diagnosis and treatment of hormonal imbalance can rapidly relieve the neurologic symptoms. Better understanding of the interaction between the endocrine system and the nervous system, combined with the knowledge about the pathophysiology of the neurologic manifestations presented in the endocrine disorders might allow earlier diagnosis and better treatment of the endocrine disorders. PMID:25654063

  14. Important cutaneous manifestations of inflammatory bowel disease

    PubMed Central

    Trost, L; McDonnell, J

    2005-01-01

    Inflammatory bowel disease (IBD) has many extraintestinal manifestations. Cutaneous manifestations are usually related to the activity of the bowel disease but may have an independent course. Anyone presenting with IBD should be examined for cutaneous manifestations. Pyoderma gangrenosum is a severe painful ulcerating disease that requires moist wound management and, in the absence of secondary infection, systemic corticosteroids, cyclosporine, or both. Infliximab may also be used. Erythema nodosum is a common cause of tender red nodules of the shins. Management includes leg elevation, NSAIDs, and potassium iodide. Oral manifestations of IBD include aphthous stomatitis, mucosal nodularity (cobblestoning), and pyostomatitis vegetans. Treatment should be directed both at the cutaneous lesions and at the underlying systemic condition. PMID:16143688

  15. 40 CFR 267.72 - Manifest discrepancies.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ...discrepancies in type are obvious differences which can be discovered by inspection or waste analysis, such as waste solvent substituted for waste acid, or toxic constituents not reported on the manifest or shipping paper. (b) Upon...

  16. 40 CFR 761.215 - Manifest discrepancies.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ...PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES CONTROL ACT ...AND USE PROHIBITIONS PCB Waste Disposal Records and Reports...the quantity or type of PCB waste designated on the manifest...the quantity and type of PCB waste a facility actually...

  17. 40 CFR 267.72 - Manifest discrepancies.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ...discrepancies in type are obvious differences which can be discovered by inspection or waste analysis, such as waste solvent substituted for waste acid, or toxic constituents not reported on the manifest or shipping paper. (b) Upon...

  18. 40 CFR 761.210 - Manifest discrepancies.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ...PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES CONTROL ACT ...AND USE PROHIBITIONS PCB Waste Disposal Records and Reports...the quantity or type of PCB waste designated on the manifest...the quantity or type of PCB waste actually delivered to and...

  19. 40 CFR 264.72 - Manifest discrepancies.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ...differences in type are obvious differences which can be discovered by inspection or waste analysis, such as waste solvent substituted for waste acid, or toxic constituents not reported on the manifest or shipping paper. (c) Upon...

  20. 40 CFR 265.72 - Manifest discrepancies.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ...differences in type are obvious differences which can be discovered by inspection or waste analysis, such as waste solvent substituted for waste acid, or toxic constituents not reported on the manifest or shipping paper. (c) Upon...

  1. 40 CFR 761.210 - Manifest discrepancies.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ...PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES CONTROL ACT ...AND USE PROHIBITIONS PCB Waste Disposal Records and Reports...the quantity or type of PCB waste designated on the manifest...the quantity or type of PCB waste actually delivered to and...

  2. 40 CFR 761.210 - Manifest discrepancies.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ...PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES CONTROL ACT ...AND USE PROHIBITIONS PCB Waste Disposal Records and Reports...the quantity or type of PCB waste designated on the manifest...the quantity or type of PCB waste actually delivered to and...

  3. 40 CFR 761.215 - Manifest discrepancies.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ...PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES CONTROL ACT ...AND USE PROHIBITIONS PCB Waste Disposal Records and Reports...the quantity or type of PCB waste designated on the manifest...the quantity and type of PCB waste a facility actually...

  4. 40 CFR 267.72 - Manifest discrepancies.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ...discrepancies in type are obvious differences which can be discovered by inspection or waste analysis, such as waste solvent substituted for waste acid, or toxic constituents not reported on the manifest or shipping paper. (b) Upon...

  5. 40 CFR 265.72 - Manifest discrepancies.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ...differences in type are obvious differences which can be discovered by inspection or waste analysis, such as waste solvent substituted for waste acid, or toxic constituents not reported on the manifest or shipping paper. (c) Upon...

  6. 40 CFR 264.72 - Manifest discrepancies.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ...differences in type are obvious differences which can be discovered by inspection or waste analysis, such as waste solvent substituted for waste acid, or toxic constituents not reported on the manifest or shipping paper. (c) Upon...

  7. Neurological manifestations of the oculodentodigital dysplasia syndrome

    Microsoft Academic Search

    Tobias Loddenkemper; Kerstin Grote; Stefan Evers; Michael Oelerich; Florian Stögbauer

    2002-01-01

    Oculodentodigital dysplasia (ODDD) (MIM 164200) is a rare autosomal dominant inherited disorder affecting the development\\u000a of the face, eyes, limbs and dentition. Neurological complications are thought to be occasional manifestations of the disorder.\\u000a This report illustrates the neurological manifestations by a pedigree of two ODDD patients with spastic paraparesis, cerebral\\u000a white matter hyperintensity and basal ganglia hypointensity. A systematic review

  8. Genetic Diseases and Associated Urologic Manifestations

    Microsoft Academic Search

    Jeffrey S. Palmer; Katherine C. Hubert

    \\u000a The diagnosis of a genetic disease is usually made through a combination of clinical characteristics and genetic testing.\\u000a It is important that urologists be able to recognize the cardinal features of these disorders and identify the associated\\u000a urologic manifestations. The purpose of this chapter is to provide an overview of the genetic disorders with urologic manifestations.

  9. Pulmonary manifestations of inflammatory bowel disease

    PubMed Central

    Ji, Xiao-Qing; Wang, Li-Xia; Lu, De-Gan

    2014-01-01

    Extraintestinal manifestations of inflammatory bowel disease (IBD) are a systemic illness that may affect up to half of all patients. Among the extraintestinal manifestations of IBD, those involving the lungs are relatively rare and often overlooked. However, there is a wide array of such manifestations, spanning from airway disease to lung parenchymal disease, thromboembolic disease, pleural disease, enteric-pulmonary fistulas, pulmonary function test abnormalities, and adverse drug reactions. The spectrum of IBD manifestations in the chest is broad, and the manifestations may mimic other diseases. Although infrequent, physicians dealing with IBD must be aware of these conditions, which are sometimes life-threatening, to avoid further health impairment of the patients and to alleviate their symptoms by prompt recognition and treatment. Knowledge of these manifestations in conjunction with pertinent clinical data is essential for establishing the correct diagnosis and treatment. The treatment of IBD-related respiratory disorders depends on the specific pattern of involvement, and in most patients, steroids are required in the initial management. Corticosteroids, both systemic and aerosolized, are the mainstay therapeutic approach, while antibiotics must also be administered in the case of infectious and suppurative processes, whose sequelae sometimes require surgical intervention. PMID:25309080

  10. Rheumatic Manifestations in Patients with Chikungunya Infection.

    PubMed

    Arroyo-Ávila, Mariangelí; Vilá, Luis M

    2015-06-01

    Chikungunya virus (CHIKV) infection is a common cause of febrile arthritis. The most common manifestations of acute infection are fever, symmetrical polyarthralgias or polyarthritis, myalgias, and maculopapular rash. Up to 80% of patients may develop musculoskeletal manifestations that persist longer than 3 months, causing impairment in their quality of life. The most common chronic manifestations are persistent or relapsing-remitting polyarthralgias, polyarthritis, and myalgias. Fingers, wrists, knees, ankles, and toes are the most frequently involved, but proximal joints and axial involvement can occur in the chronic stage. Chronic manifestations of CHIKV infection may resemble those of some autoimmune connective tissue diseases. Furthermore, CHIKV infection can cause cryoglobulinemia and may induce rheumatoid arthritis and seronegative spondyloarthropathies in genetically susceptible individuals. The Centers for Disease Control and Prevention recommend acetaminophen and non steroidal anti-inflammatory drugs for the acute rheumatic manifestations of CHIKV infection. However, some studies suggest that low-dose corticosteroids for about 1-2 months (depending on clinical course) are beneficial in relieving acute rheumatic symptoms. Conversely, hydroxychloroquine in combination with corticosteroids or other disease modifying anti-rheumatic drugs (DMARDs) has been successful in treating chronic rheumatic manifestations. Methotrexate and sulfasalazine (alone or in combination) have also been effective for chronic CHIKV arthritis. Patients with CHIKV infection should be closely monitored to identify those with chronic arthritis who would benefit from a rheumatologic evaluation and early treatment with DMARDs. PMID:26061056

  11. 40 CFR 262.25 - Electronic manifest signatures.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ...The Manifest § 262.25 Electronic manifest signatures. Electronic signature methods for the e-Manifest system shall: ...other Federal requirements pertaining to electronic signatures; and (b) Be a method that is...

  12. 40 CFR 263.25 - Electronic manifest signatures.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ...false Electronic manifest signatures. 263.25 Section 263...25 Electronic manifest signatures. (a) Electronic manifest signatures shall meet the criteria described...this chapter. (b) [Reserved] [79 FR 7560,...

  13. 40 CFR 265.71 - Use of manifest system.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ...2014-07-01 false Use of manifest system. 265.71 Section 265.71 Protection...STORAGE, AND DISPOSAL FACILITIES Manifest System, Recordkeeping, and Reporting § 265.71 Use of manifest system. (a)(1) If a facility...

  14. 77 FR 54863 - Polychlorinated Biphenyls (PCBs): Revisions to Manifesting Regulations

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-09-06

    ...Act (RCRA) Uniform Hazardous Waste Manifest, under the Toxic Substances Control Act (TSCA...manifesting requirements for hazardous waste under RCRA, of which the regulatory...implement the Uniform Hazardous Waste Manifest form were...

  15. Mitochondrial Disorders with Significant Ophthalmic Manifestations

    PubMed Central

    Al-Enezi, Mona; Al-Saleh, Hanan; Nasser, Murad

    2008-01-01

    Mitochondrial diseases are a clinically hetyerogenous group of disorders. They can be caused by mutations of nuclear or mitochondrial DNA (mtDNA). Some affect a single organ, but many involve multiple organ systems and often present with prominent neurologic and myopathic features. The eye is frequently affected, along with muscles and brain, but multisystem disease is common. Ophthalmic manifestations include cataract, retinopathy, optic atrophy, cortical visual loss, ptosis and ophthalmoplegia. Kearns-Sayre Syndrome (KSS), Mitochondrial Encephalopathy, Lactic Acidosis Stroke (MELAS), Myoclonic Epilepsy and Ragged Red Fiber myopathy (MERRF) and Lebers Hereditary Optic Neuropathy (LHON) are well known clinical entities that are secondary to mtDNA abnormalities, which has ophthalmic manifestations. Mitochondrial Dysfunction should be considered in the differential diagnosis of progressive multisystem disorder and specifically if there is associated neuro-ophthalmic manifestations, which may be the presenting symptom of these disorders. PMID:21346843

  16. Cutaneous Manifestations of Systemic Lupus Erythematosus

    PubMed Central

    Uva, Luís; Miguel, Diana; Pinheiro, Catarina; Freitas, João Pedro; Marques Gomes, Manuel; Filipe, Paulo

    2012-01-01

    Systemic lupus erythematosus (SLE) is a multiorgan autoimmune disease of unknown etiology with many clinical manifestations. The skin is one of the target organs most variably affected by the disease. The American College of Rheumatology (ACR) established 11 criteria as a classificatory instrument to operationalise the definition of SLE in clinical trials. They were not intended to be used to diagnose individuals and do not do well in that capacity. Cutaneous lesions account for four of these 11 revised criteria of SLE. Skin lesions in patients with lupus may be specific or nonspecific. This paper covers the SLE-specific cutaneous changes: malar rash, discoid rash, photosensitivity, and oral mucosal lesions as well as SLE nonspecific skin manifestations, their pathophysiology, and management. A deeper thorough understanding of the cutaneous manifestations of SLE is essential for diagnosis, prognosis, and efficient management. Thus, dermatologists should cooperate with other specialties to provide optimal care of SLE patient. PMID:22888407

  17. [Dermatologic manifestations of the antiphospholipid syndrome].

    PubMed

    Francès, C; Barete, S; Soria, A

    2012-04-01

    A wide variety of dermatologic manifestations has been described in the antiphospholipid syndrome (APS). The most frequent skin lesion is livedo reticularis, present not only on the limbs but also on the trunk, with a fine irregular pattern. It belongs to the arterial subset of APS. Circumscribed ulcerations, resembling livedoid vasculitis, may be the first manifestation of APS. Ulcerations may also occur as a late complication of recurrent venous thrombosis. Extensive skin necrosis is a classic manifestation of catastrophic APS. Pseudo-vasculitis lesions are misdiagnosed if a skin biopsy is not performed, especially in the context of systemic lupus erythematosus. In systemic lupus erythematosus, primary anetoderma is always associated with antiphospholipid antibodies. PMID:22078802

  18. Oral manifestations of hematologic and nutritional diseases.

    PubMed

    Schlosser, Bethanee J; Pirigyi, Megan; Mirowski, Ginat W

    2011-02-01

    Oral manifestations of hematologic and nutritional deficiencies can affect the mucous membranes, teeth, periodontal tissues, salivary glands, and perioral skin. This article reviews common oral manifestations of hematologic conditions starting with disorders of the white blood cells including cyclic hematopoiesis (cyclic neutropenia), leukemias, lymphomas, plasma cell dyscrasias, and mast cell disorders; this is followed by a discussion of the impact of red blood cell disorders including anemias and less common red blood cell dyscrasias (sickle cell disease, hemochromatosis, and congenital erythropoietic porphyria) as well as thrombocytopenia. Several nutritional deficiencies exhibit oral manifestations. The authors specifically discuss the impact of water-soluble vitamins (B2, B3, B6, B9, B12, and C), fat-soluble vitamins (A, D, and K) and the eating disorders anorexia nervosa and bulimia nervosa on the oral mucosa. PMID:21093629

  19. Caffeine-induced psychiatric manifestations: a review.

    PubMed

    Wang, Hee Ryung; Woo, Young Sup; Bahk, Won-Myong

    2015-07-01

    The association between caffeine consumption and various psychiatric manifestations has long been observed. We present two cases that show the ability of caffeine to induce psychotic and manic symptoms, and we also review the extant literature on caffeine-induced psychiatric manifestations. On the basis of our own and others' findings, we suggest that caffeine may be related to not only de-novo psychotic or mood symptoms but also to aggravation of pre-existing psychotic or mood disorders. We therefore suggest that caffeine consumption among patients with mood or psychotic symptoms should be assessed carefully in clinical practice as part of routine psychiatric evaluations. PMID:25856116

  20. Neurologic Manifestations of Pediatric Chikungunya Infection

    Microsoft Academic Search

    Stéphanie Robin; Duksha Ramful; Florence Le Seach; Marie-Christine Jaffar-Bandjee; Gérald Rigou; Jean-Luc Alessandri

    2008-01-01

    Chikungunya virus, a mosquito-borne arbovirus, was responsible for a massive epidemic in La Réunion Island during 2005 to 2006. The disease is usually benign, but neurologic involvement, with sometimes fatal outcome, has been described. We report a retrospective hospital-based pediatric series of 30 children (23 boys and 7 girls) who presented neurologic manifestations of chikungunya such as encephalitis (n =

  1. Determinants of systemic manifestations of food allergy

    Microsoft Academic Search

    Scott H. Sicherer

    2000-01-01

    The myriad of systemic manifestations induced by food hypersensitivity responses is testament to the ability of localized exposure to foods in the gastrointestinal tract to result in symptoms in distal target organs. Cow’s milk protein, for example, may induce hives (urticaria), atopic dermatitis, isolated gastrointestinal symptoms, or severe generalized anaphylaxis in different individuals or in the same person at different

  2. Behavioral Manifestations of Child Sexual Abuse: Response.

    ERIC Educational Resources Information Center

    Reece, Robert M.

    1998-01-01

    This response to a previous article on behavioral manifestations of child sexual abuse (EC 619 258) stresses the importance of empirical research and the avoidance of assumptions that incidents of sexual abuse are the sentinel events in the life of a child, when the child's dysfunctional milieu may provide far more traumatic events. (DB)

  3. RHEUMATOLOGICAL MANIFESTATIONS IN PATIENTS WITH MELIOIDOSIS

    Microsoft Academic Search

    P Teparrakkul; JJ Tsai; W Chierakul; T Wacharaprechasgu; W Chaowagul

    Melioidosis, an infection caused by the bacterium Burkholderia pseudomallei, has a wide range of clinical manifestations. Here, we describe rheumatological melioidosis (involving one or more of joint, bone or muscle), and compare features and outcome with patients with- out rheumatological involvement. A retrospective study of patients with culture-confirmed melioidosis admitted to Sappasithiprasong Hospital, Ubon Ratchathani during 2002 and 2005 identified

  4. Real-Life Contextual Manifestations of Wisdom

    ERIC Educational Resources Information Center

    Yang, Shih-Ying

    2008-01-01

    Wisdom pertains to managing human affairs, and it arises in highly contextualized situations. The present study aims to investigate manifestations of wisdom in real-life contexts through semi-structured interviews with 66 individuals nominated as wise persons. All nominees were ethnic Chinese from Taiwan, an East Asian country which has…

  5. Proteus syndrome: emphasis on the pulmonary manifestations

    Microsoft Academic Search

    B. Newman; A. H. Urbach; D. Orenstein; P. S. Dickman

    1994-01-01

    Published articles on the radiologic aspects of Proteus syndrome are sparse. This report highlights the features of this disease with specific attention to the serious pulmonary manifestations that may occur at an early age. Two cases of Proteus syndrome and severe lung disease are presented, with complete autopsy in one case and correlative surgical pathologic data in the other. Multiple

  6. [Shwachman's syndrome with neonatal thoracic manifestations].

    PubMed

    Labrune, M; Dommergues, J P; Chaboche, C; Benichou, J J

    1984-10-01

    The authors report a case of Shwachman's syndrome in whom neonatal respiratory distress was the initial manifestation. Metaphyseal dysplasia, which was severe at birth, progressively disappeared. By the age of 10 years, radiologic anomalies persist only in the pelvis. PMID:6508486

  7. Gastrointestinal Manifestations in Systemic Autoimmune Diseases

    PubMed Central

    COJOCARU, M.; COJOCARU, Inimioara Mihaela; SILOSI, Isabela; VRABIE, Camelia Doina

    2011-01-01

    ABSTRACT In an autoimmune disease, the immune system attacks and harms the body's own tissues. The systemic autoimmune diseases include collagen vascular diseases, the systemic vasculitides, Wegener granulomatosis, and Churg-Strauss syndrome. These disorders can involve any part of the gastrointestinal tract, hepatobiliary system and pancreas. They can cause a variety of gastrointestinal manifestations that are influenced by the pathophysiologic characteristics of the underlying disease process. There is a wide variation of gastrointestinal manifestations from these autoimmune disorders including, but not limited to: oral ulcers, dysphagia, gastroesophageal reflux disease, abdominal pain, constipation, diarrhea, fecal incontinence, pseudo-obstruction, perforation and gastrointestinal bleeding. Clinical workup should be initiated by the patient's subjective complaints. In this review, we analyze the effects of autoimmune diseases on the gastrointestinal tract. PMID:21977190

  8. Spinal muscular atrophy: manifestations and management.

    PubMed

    Mesfin, Addisu; Sponseller, Paul D; Leet, Arabella I

    2012-06-01

    Spinal muscular atrophy (SMA) is an autosomal recessive disorder caused by a homozygous deletion in the SMN1 gene and is manifested by loss of the anterior horn cells of the spinal cord. Classifications of the disorder are based on age of onset and the patient's level of function. Scoliosis and hip subluxation or dislocation are two musculoskeletal manifestations associated with SMA. Severity of scoliosis correlates with age at presentation. Bracing has been unsuccessful in halting curve progression and may interfere with respiratory effort. Early onset scoliosis associated with SMA has been successfully treated with growing rod constructs, and posterior spinal fusion can be used in older children. Hip subluxations and dislocations are best treated nonsurgically if the patient reports no pain because a high rate of recurrent dislocation has been reported with surgical intervention. PMID:22661569

  9. Cutaneous human papillomavirus infection: manifestations and diagnosis.

    PubMed

    Tschandl, Philipp; Rosendahl, Cliff; Kittler, Harald

    2014-01-01

    Keratinocytes react to human papillomavirus (HPV) infection by proliferation resulting in different clinical manifestations ranging from flat warts to common warts and cauliflower-like or filiform warts. The clinical presentation may vary according to the HPV type and anatomical site. Although warts are usually a self-limiting disease, some HPV subtypes may induce malignant changes. Bowenoid papulosis is characterized by multiple flat macules or patches in the genital area that may or may not be pigmented. Rarely other forms of superficial squamous cell carcinomas may be induced by HPV infections, for example periungual Bowen's disease. Dermatoscopy is a noninvasive diagnostic technique that facilitates the diagnosis of skin manifestations induced by HPV. PMID:24643180

  10. Peripheral Nervous System Manifestations of Infectious Diseases

    PubMed Central

    Brizzi, Kate T.

    2014-01-01

    Infectious causes of peripheral nervous system (PNS) disease are underrecognized but potentially treatable. Heightened awareness educed by advanced understanding of the presentations and management of these infections can aid diagnosis and facilitate treatment. In this review, we discuss the clinical manifestations, diagnosis, and treatment of common bacterial, viral, and parasitic infections that affect the PNS. We additionally detail PNS side effects of some frequently used antimicrobial agents. PMID:25360209

  11. The cutaneous manifestations of metastatic malignant melanoma.

    PubMed

    Reed, Kurtis B; Cook-Norris, Robert H; Brewer, Jerry D

    2012-03-01

    Cutaneous metastases are common sequelae of primary malignant melanoma. Because patients with melanoma are examined frequently after diagnosis, it is important that dermatologists are aware of the range of findings that may represent metastatic disease. Many case reports and a few retrospective series have been published detailing the wide variety of clinical presentations of cutaneous metastatic melanoma. This article reviews the various manifestations of metastatic melanoma of the skin and oral mucous membranes and summarizes treatment options for metastatic disease. PMID:22348556

  12. Unusual radiologic manifestations of bronchogenic cyst.

    PubMed

    Dahmash, N S; Chen, J T; Ravin, C E; Reed, J C; Pratt, P C

    1984-06-01

    We have presented four cases of bronchogenic cyst, emphasizing the uncommon radiologic manifestations, including air trapping and/or lobulated contour. Two of the cysts were in the lung parenchyma, one in the left hilum adherent to the left lower lobe bronchus, and one in the posterior mediastinum. Recognition of the unusual radiologic features aids in establishing an appropriate differential diagnosis and directing subsequent evaluation. PMID:6729555

  13. Amyloid goiter: first manifestation of systemic amyloidosis.

    PubMed

    Kazdaghli Lagha, E; M'sakni, I; Bougrine, F; Laabidi, B; Ben Ghachem, D; Bouziani, A

    2010-06-01

    Amyloidosis is an abnormal extracellular deposit, which can occur in several tissues. The mechanism is not clearly defined. In systemic amyloidosis, all the organs can be infiltrated, but amyloid goiter as the initial manifestation of systemic amyloidosis is an exceedingly rare condition. We report a rare case of a patient who presented an amyloid goiter as the first manifestation of systemic amyloidosis. This patient had a known Crohn's disease. He developed a goiter without compressive complications. Histologic examination revealed a diffuse amyloid deposition surrounding thyroid follicles. The gland was enlarged with an eosinophilic and amorphic deposit. Confirmation of amyloid was made by the presence of congophilia and apple-green birefringence under polarized-light microscopy. An immunoreactivity was seen with AA protein. Amyloid goiter is a rare manifestation of amyloidosis. About 250 cases of amyloid goiter have been reported in the literature. The goiter enlarges rapidly and progressively, often becoming compressive like thyroid cancer. The prognosis depends on the treatment of the amyloidosis and the underlying chronic disease. PMID:20822765

  14. [Chronic polyarthritis as isolated manifestation of toxocariasis.

    PubMed

    Viola, Gabriela R; Giacomin, Maria Fernanda A; França, Camila M P; Sallum, Adriana M E; Jacob, Cristina M A; Silva, Clovis A

    2014-10-16

    Human toxocariasis is a parasitic zoonosis mainly caused by Toxocara canis or T. cati and is acquired by ingestion of the parasite's embryonated eggs. Arthralgia and/or arthritis were reported in up to 17% of cases, generally with acute duration (less than 6 weeks). However, to our knowledge, chronic polyarthritis, as the isolated presentation of Toxocara infection, was not reported. One of 5,809 patients that was followed up at our service (0.017%) had chronic polyarthritis as the single manifestation of toxocariasis and was described herein. A 3-year-old girl was referred to our service with severe painful chronic polyarthritis for a period longer than 10 weeks and morning stiffness of 30minutes. Dog contact exposure history in the recreational areas of neighborhood was reported. Her exams showed high levels of eosinophils in peripheral blood (29%), bone marrow aspirate revealed marked eosinophilia (32%) and Toxocara enzyme-linked immunosorbent assay (Elisa) was positive (1:1,280). She was treated with paracetamol (40 mg/kg/day) and thiabendazole (25 mg/kg/day) for 10 days, and all manifestations reduced. After eight months of follow-up, she was on clinical and laboratorial remission. In conclusion, we described a case of chronic polyarthritis, as isolated manifestation of toxocariasis, mimicking juvenile idiopathic arthritis and leukemia. Importantly, this zoonosis should be considered in patients with arthritis and eosinophilia. PMID:25435169

  15. Rare Neurological Manifestation of Celiac Disease

    PubMed Central

    Rani, Uzma; Imdad, Aamer; Beg, Mirza

    2015-01-01

    Celiac disease (CD) is an immune-mediated disease characterized by permanent gastrointestinal tract sensitivity to gluten in genetically predisposed individuals. It has varied clinical manifestations, ranging from gastrointestinal to extraintestinal, including neurological, skin, reproductive and psychiatric symptoms, which makes its diagnosis difficult and challenging. Known neurological manifestations of CD include epilepsy with or without occipital calcification, attention deficit hyperactivity disorder and ataxia, headache, neuropathies and behavior disorders. We present the case of a 14-year-old female with headaches and blurred vision for 1 year; she was noted to have papilledema on ophthalmic examination with increased cerebrospinal fluid opening pressure on lumber puncture and was diagnosed as a case of pseudotumor cerebri (PTC). Meanwhile her workup for chronic constipation revealed elevated tissue transglutaminase IgA and antiendomysial IgA antibodies. Upper gastrointestinal endoscopy with duodenal biopsy confirmed the diagnosis of CD. The patient was started on a gluten-free diet, leading to resolution of not only gastrointestinal symptoms but also to almost complete resolution of symptoms of PTC. This report describes the correlation of CD and PTC as its neurological manifestation. PMID:26120302

  16. Rare Neurological Manifestation of Celiac Disease.

    PubMed

    Rani, Uzma; Imdad, Aamer; Beg, Mirza

    2015-01-01

    Celiac disease (CD) is an immune-mediated disease characterized by permanent gastrointestinal tract sensitivity to gluten in genetically predisposed individuals. It has varied clinical manifestations, ranging from gastrointestinal to extraintestinal, including neurological, skin, reproductive and psychiatric symptoms, which makes its diagnosis difficult and challenging. Known neurological manifestations of CD include epilepsy with or without occipital calcification, attention deficit hyperactivity disorder and ataxia, headache, neuropathies and behavior disorders. We present the case of a 14-year-old female with headaches and blurred vision for 1 year; she was noted to have papilledema on ophthalmic examination with increased cerebrospinal fluid opening pressure on lumber puncture and was diagnosed as a case of pseudotumor cerebri (PTC). Meanwhile her workup for chronic constipation revealed elevated tissue transglutaminase IgA and antiendomysial IgA antibodies. Upper gastrointestinal endoscopy with duodenal biopsy confirmed the diagnosis of CD. The patient was started on a gluten-free diet, leading to resolution of not only gastrointestinal symptoms but also to almost complete resolution of symptoms of PTC. This report describes the correlation of CD and PTC as its neurological manifestation. PMID:26120302

  17. 10 CFR 20.2006 - Transfer for disposal and manifests.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ...false Transfer for disposal and manifests. 20.2006 Section 20.2006 Energy NUCLEAR REGULATORY COMMISSION STANDARDS FOR PROTECTION AGAINST RADIATION Waste Disposal § 20.2006 Transfer for disposal and manifests. (a)...

  18. 10 CFR 20.2006 - Transfer for disposal and manifests.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ...false Transfer for disposal and manifests. 20.2006 Section 20.2006 Energy NUCLEAR REGULATORY COMMISSION STANDARDS FOR PROTECTION AGAINST RADIATION Waste Disposal § 20.2006 Transfer for disposal and manifests. (a)...

  19. 10 CFR 20.2006 - Transfer for disposal and manifests.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ...false Transfer for disposal and manifests. 20.2006 Section 20.2006 Energy NUCLEAR REGULATORY COMMISSION STANDARDS FOR PROTECTION AGAINST RADIATION Waste Disposal § 20.2006 Transfer for disposal and manifests. (a)...

  20. 10 CFR 20.2006 - Transfer for disposal and manifests.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ...2010-01-01 2010-01-01 false Transfer for disposal and manifests. 20.2006 Section 20...STANDARDS FOR PROTECTION AGAINST RADIATION Waste Disposal § 20.2006 Transfer for disposal and manifests. (a) The requirements...

  1. Gene Therapy for Neurologic Manifestations of Mucopolysaccharidoses

    PubMed Central

    Wolf, Daniel A.; Banerjee, Sharbani; Hackett, Perry B.; Whitley, Chester B.; McIvor, R. Scott; Low, Walter C.

    2015-01-01

    Introduction Mucopolysaccharidoses are a family of lysosomal disorders caused by mutations in genes that encode enzymes involved in the catabolism of glycoaminoglycans. These mutations affect multiple organ systems and can be particularly deleterious to the nervous system. At the present time, enzyme replacement therapy and hematopoietic stem-cell therapy are used to treat patients with different forms of these disorders. However, to a great extent the nervous system is not adequately responsive to current therapeutic approaches. Areas Covered Recent advances in gene therapy show great promise for treating mucopolysaccharidoses. This article reviews the current state of the art for routes of delivery in developing genetic therapies for treating the neurologic manifestations of mucopolysaccharidoses. Expert Opinion Gene therapy for treating neurological manifestations of mucopolysaccharidoses can be achieved by intraventricular, intrathecal, intranasal, and systemic administration. The intraventricular route of administration appears to provide the most wide-spread distribution of gene therapy vectors to the brain. The intrathecal route of delivery results in predominant distribution to the caudal areas of the brain while the intranasal route of delivery results in good distribution to the rostral areas of brain. The systemic route of delivery via intravenous delivery can also achieve wide spread delivery to the CNS, however, the distribution to the brain is greatly dependent on the vector system. Intravenous delivery using lentiviral vectors appear to be less effective than adeno-associated viral (AAV) vectors. Moreover, some subtypes of AAV vectors are more effective than others in crossing the blood-brain-barrier. In summary, the recent advances in gene vector technology and routes of delivery to the CNS will facilitate the clinical translation of gene therapy for the treatment of the neurological manifestations of mucopolysaccharidoses. PMID:25510418

  2. Neurologic manifestations of Henoch-Schönlein purpura.

    PubMed

    Bérubé, Maxime D; Blais, Normand; Lanthier, Sylvain

    2014-01-01

    Henoch-Shönlein purpura (HSP) is a systemic small vessel vasculitis. Most patients present during childhood. The characteristic association of purpura, arthralgia, abdominal pain, and nephritis reflects the predominant distribution of vasculitis. Headaches and mild behavioral changes suggest CNS involvement in one-third of HSP patients. Salient central nervous system (CNS) manifestations are rarer and mostly reported in adults and patients with a severe disease course. Diagnosis of CNS vasculitis is rarely confirmed by histopathology and generally relies on "suggestive" imaging showing brain hemorrhages, infarcts and edema, predominantly located in the parieto-occipital regions. Vessel wall friability and thrombogenicity of active vasculitis, antiphospholipid antibody synthesis, and other hemostatic disturbances may contribute to hemorrhagic and thrombotic complications of HSP. Posterior reversible encephalopathy syndrome and hypertensive encephalopathy occur in HSP and can be difficult to differentiate from CNS vasculitis. Some 53% of patients with neurologic complications experience seizures. Cerebral venous thrombosis, subdural hematoma, subarachnoidal hemorrhage, neuro-ophthalmologic complications, myelopathy, and diverse neuromuscular manifestations are also reported. In contrast with other systemic small vessel vasculitides, peripheral nervous system involvement is infrequent in HSP. Systemic involvement of HSP and homeostatic disorders such as hypertension, uremia, and electrolyte disturbances, as well as superimposed infections can affect the nervous system secondarily. Identification of nervous system complications of HSP is often challenging due to prominent systemic manifestations. HSP is usually a self-limiting disease that requires only supportive care. Patients with CNS vasculitis are commonly treated with corticosteroids. One-fifth of patients with CNS involvement remain with sequelae. PMID:24365374

  3. Cutaneous manifestations of category A bioweapons.

    PubMed

    Aquino, Lisa L; Wu, Jashin J

    2011-12-01

    The 2001 anthrax attacks on the United States brought bioterrorism to the forefront of the medical community. Because many bioterrorist agents produce cutaneous disease, dermatologists will likely be first responders during a future attack. Despite this, many dermatologists are not adequately prepared to deal with a bioterrorism attack. It is critical that all first responders be able to recognize symptoms of bioterrorism-related disease and prepared to respond to a bioterrorist attack to minimize threats to public health. This article reviews the diagnosis and treatment of diseases caused by potential biowarfare agents that produce cutaneous manifestations, and also provides information regarding reporting and containment of possible bioterrorism-related diseases. PMID:21777993

  4. Manifestation of optical activity in different materials

    NASA Astrophysics Data System (ADS)

    Konstantinova, A. F.; Golovina, T. G.; Konstantinov, K. K.

    2014-07-01

    Various manifestations of optical activity (OA) in crystals and organic materials are considered. Examples of optically active enantiomorphic and nonenantiomorphic crystals of 18 symmetry classes are presented. The OA of enantiomorphic organic materials as components of living nature (amino acids, sugars, and proteins) is analyzed. Questions related to the origin of life on earth are considered. Examples of differences in the enantiomers of drugs are shown. The consequences of replacing conventional left-handed amino acids with additionally right-handed amino acids for living organisms are indicated.

  5. Ocular manifestations of systemic inflammatory diseases.

    PubMed

    Mohsenin, Amir; Huang, John J

    2012-10-01

    Inflammation of the eye is often times seen in association with systemic inflammatory diseases. Understanding the various forms of ocular involvement in these conditions is important as untreated ophthalmic involvement can lead to severe vision loss. In addition to providing a basic framework for diagnosis and treatment, this review will highlight the ocular manifestations of the following systemic inflammatory conditions: rheumatoid arthritis, systemic lupus erythematosus, Wegener's granulomatosis, Sjögren's syndrome, polyarteritisnodosa, primary antiphospholipid syndrome, Behçet's syndrome, Kawasaki disease, Cogan's syndrome and relapsing polychondritis. PMID:23155672

  6. Catatonia: an unusual manifestation of Wilson's disease.

    PubMed

    Basu, Aniruddha; Thanapal, Sivakumar; Sood, Mamta; Khandelwal, Sudhir K

    2015-01-01

    Wilson's disease, characterized by abnormal copper accumulation in the human body, may present with psychiatric manifestations in about one-fifth of patients. The authors report a patient with Wilson's disease who initially presented with acute psychosis and later developed catatonic symptoms. The atypical presentation led to a delay in diagnosis and institution of appropriate treatment. Wilson's disease can be ruled out in all young patients presenting with psychiatric symptoms for the first time by screening for a Kayser-Fleischer ring. PMID:25716489

  7. Pulmonary Manifestations of Systemic Autoimmune Diseases

    PubMed Central

    COJOCARU, Manole; COJOCARU, Inimioara Mihaela; SILOSI, Isabela; VRABIE, Camelia Doina

    2011-01-01

    ABSTRACT Systemic autoimmune diseases (SAD) are a heterogeneous group of immunologically mediated inflammatory disorders including multiorgan involvement. As expected in a multisystem disease, the entire pulmonary system is vulnerable to injury. Any of its compartments may be independently or simultaneously affected. It is difficult to assess the true prevalence of lung disease in cases of SAD. In this article, we will review the pulmonary manifestations caused by systemic lupus erithematosus, rheumatoid arthritis, systemic sclerosis, polymyositis/dermatomyositis, Sjögren's syndrome, mixed connective tissue disease, Wegener's granulomatosis, Churg-Strauss syndrome, Goodpasture's syndrome, and ankylosing spondylitis. PMID:22368703

  8. Eosinophilic esophagitis -- clinical manifestations, diagnosis, and treatment.

    PubMed

    Lucendo Villarín, A J

    2009-01-01

    Eosinophilic esophagitis (EE) is a chronic inflammatory, immunoallergic disease of the esophagus that represents the most common eosinophilic gut disease. Understanding and diagnosis regarding this condition have greatly increased in recent years, particularly in Europe and North America, in parallel with other allergic disorders. It consists of dense esophageal infiltration with eosinophils in the absence of gastro-esophageal reflux (GER). It involves individuals at all ages, and is particularly common in males during childhood and up to the 5th decade of life. It manifests with chronic, intermittent esophageal symptoms that predominantly include dysphagia, food impaction episodes, and GER-attributable complaints that do not respond to antisecretory therapy. Endoscopically, EE is a polymorphous disease that presents with various changes in esophageal caliber, and subtle changes in mucosal appearance, which lead to biopsy collection as a key procedure for diagnosis. Management must be multidisciplinary, including gastroenterologists, pathologists, allergologists, and also nutrition specialists in pediatric cases. Regarding therapy, dietary food restrictions are especially useful in the management of pediatric EE, but effectiveness is lower in the adult, maybe because of a greater involvement of air allergens. Drug use is standard, particularly involving topical steroids, which may revert manifestations and histological lesions, even though recurrence following discontinuation is common. PMID:19335033

  9. Hematologic manifestations of Helicobacter pylori infection.

    PubMed

    Campuzano-Maya, Germán

    2014-09-28

    Helicobacter pylori (H. pylori) is the most common infection in humans, with a marked disparity between developed and developing countries. Although H. pylori infections are asymptomatic in most infected individuals, they are intimately related to malignant gastric conditions such as gastric cancer and gastric mucosa-associated lymphoid tissue (MALT) lymphoma and to benign diseases such as gastritis and duodenal and gastric peptic ulcers. Since it was learned that bacteria could colonize the gastric mucosa, there have been reports in the medical literature of over 50 extragastric manifestations involving a variety medical areas of specialization. These areas include cardiology, dermatology, endocrinology, gynecology and obstetrics, hematology, pneumology, odontology, ophthalmology, otorhinolaryngology and pediatrics, and they encompass conditions with a range of clear evidence between the H. pylori infection and development of the disease. This literature review covers extragastric manifestations of H. pylori infection in the hematology field. It focuses on conditions that are included in international consensus and management guides for H. pylori infection, specifically iron deficiency, vitamin B12 (cobalamin) deficiency, immune thrombocytopenia, and MALT lymphoma. In addition, there is discussion of other conditions that are not included in international consensus and management guides on H. pylori, including auto-immune neutropenia, antiphospholipid syndrome, plasma cell dyscrasias, and other hematologic diseases. PMID:25278680

  10. Ophthalmic manifestations of inherited neurodegenerative disorders.

    PubMed

    Kersten, Hannah M; Roxburgh, Richard H; Danesh-Meyer, Helen V

    2014-06-01

    Ophthalmic findings are common features of neurodegenerative disorders and, in addition to being clinically important, have emerged as potentially useful biomarkers of disease progression in several conditions. Clinically, these visual system abnormalities can be a clue to diagnosis, as well as being a prominent cause of disability in affected patients. In this Review, we describe the various afferent visual system and other ophthalmic features of inherited neurodegenerative disorders, including the muscular dystrophies, Friedreich ataxia, the spinocerebellar ataxias, hereditary spastic paraplegia, Charcot-Marie-Tooth disease, and other conditions. We focus on the expanding role of optical coherence tomography in diagnostic imaging of the retina and optic nerve head, and the possible use of ophthalmic findings as biomarkers of disease severity in hereditary neurodegenerative disorders. In addition, we discuss the ophthalmic manifestations and treatment implications of mitochondrial dysfunction, which is a feature of many inherited neurodegenerative diseases. PMID:24840976

  11. Brucella arteritis: clinical manifestations, treatment, and prognosis

    PubMed Central

    Herrick, Jesica A.; Lederman, Robert J.; Sullivan, Brigit; Powers, John H.; Palmore, Tara

    2015-01-01

    Brucellosis is the most common bacterial zoonosis, and causes a considerable burden of disease in endemic countries. Cardiovascular involvement is the main cause of mortality due to infection with Brucella spp., and most commonly manifests as endocarditis, peripheral and cerebrovascular aneurysms, or arterial and venous thrombosis. We report a case of brucellosis presenting as bacteremia and aortic endarteritis 18 years after the last known exposure to risk factors for brucella infection. The patient was treated with doxycycline, rifampin, and gentamicin, and underwent surgical repair of a penetrating aortic ulcer, with a good clinical recovery. We review the signs and symptoms, diagnostic approach, prognosis, and treatment of brucella arteritis. We draw attention to the absence of consensus about the optimum therapy for vascular brucellosis, and the urgent need for additional studies and renewed scientific interest in this major pathogen. PMID:24480149

  12. Rheumatological manifestations in inflammatory bowel disease

    PubMed Central

    Voulgari, Paraskevi V.

    2011-01-01

    Rheumatological manifestations in inflammatory bowel disease (IBD) are frequent and include peripheral arthritis, axial involvement and peripheral enthesitis. Secondary osteoporosis and hypertrophic osteoarthropathy may also occur. Complications of IBD (e.g. septic arthritis) must be distinguished from sterile inflammation. Adverse effects of corticosteroid treatment, such as osteonecrosis, may also affect joints. Axial involvement ranges from low back pain to true ankylosing spondylitis. Human leukocyte antigen B27 is associated with axial involvement of IBD. Peripheral arthritis has been classified into two types. Type I is a pauciarticular, asymmetric usually non destructive arthritis affecting large joints and is usually associated with active bowel disease. Type II is a polyarthritis affecting small joints and tends to run a course independent of the bowel disease. Treatment of joint symptoms in IBD include sulphasalazine, azathioprine, methotrexate and glucocorticoids. Anti-tumor necrosis factor antibodies are effective in treating resistant or complicated Crohn’s disease as well as peripheral arthritis and axial involvement. PMID:24713717

  13. Diabetic neuropathy: clinical manifestations and current treatments.

    PubMed

    Callaghan, Brian C; Cheng, Hsinlin T; Stables, Catherine L; Smith, Andrea L; Feldman, Eva L

    2012-06-01

    Diabetic peripheral neuropathy is a prevalent, disabling disorder. The most common manifestation is distal symmetrical polyneuropathy (DSP), but many patterns of nerve injury can occur. Currently, the only effective treatments are glucose control and pain management. While glucose control substantially decreases the development of neuropathy in those with type 1 diabetes, the effect is probably much smaller in those with type 2 diabetes. Evidence supports the use of specific anticonvulsants and antidepressants for pain management in patients with diabetic peripheral neuropathy. However, the lack of disease-modifying therapies for diabetic DSP makes the identification of new modifiable risk factors essential. Growing evidence supports an association between components of the metabolic syndrome, including prediabetes, and neuropathy. Studies are needed to further explore this association, which has implications for the development of new treatments for this common disorder. PMID:22608666

  14. Arthritis associated with HIV infection: radiographic manifestations.

    PubMed

    Rosenberg, Z S; Norman, A; Solomon, G

    1989-10-01

    Radiographs of symptomatic joints were retrospectively evaluated in 24 patients with inflammatory arthritis and human immunodeficiency virus (HIV) infection. Clinically, 20 patients had a seronegative arthritis including Reiter syndrome (54%), psoriatic arthritis (17%), and undifferentiated forms of spondyloarthropathy (13%). These patients were indistinguishable radiographically from patients with typical seronegative disorders except for the predominance of lower-extremity abnormalities. Four patients (17%) had a rheumatoidlike arthritis defined as acute symmetric polyarthritis (ASP). With the exception of extensive proliferative periostitis, ASP simulated classic rheumatoid arthritis. HIV-associated arthritis was manifest during various stages of HIV infection. It preceded acquired immunodeficiency syndrome in 64% of patients with stage IV HIV infection. Awareness of the coexistence of HIV infection in patients with the above-mentioned arthritides is important, since immunosuppressive therapy, commonly used in the treatment of arthritis, can have detrimental effects in patients with HIV infection. PMID:2781004

  15. Quantum manifestations of classical resonance zones

    NASA Astrophysics Data System (ADS)

    De Leon, N.; Davis, M. J.; Heller, E. J.

    1984-01-01

    We examine the concept of nodal breakup of wave functions as a criterion for quantum mechanical ergodicity. We find that complex nodal structure of wave functions is not sufficient to determine quantum mechanical ergodicity. The influence of classical resonances [which manifest themselves as classical resonance zones (CRZ)] may also be responsible for the seeming complexity of nodal structure. We quantify this by reexamining one of the two systems studied by Stratt, Handy, and Miller [J. Chem. Phys. 71, 3311 (1974)] from both a quantum mechanical and classical point of view. We conclude that quasiperiodic classical motion can account for highly distorted quantum eigenstates. One should always keep this in mind when addressing questions regarding quantum mechanical ergodicity.

  16. Common dermatologic manifestations of primary immune deficiencies.

    PubMed

    Relan, Manisha; Lehman, Heather K

    2014-12-01

    The skin is the largest organ of our body; it consists of the epidermis, dermis, hair follicles, sweat glands, blood vessels, and connective tissue matrix. Its main function is to act as a barrier to the outside world and protect us from infections. Any component of the skin is subject to insults from the environment and/or from within the body. Primary immune deficiency patients present with recurrent or prolonged infections not frequently seen in healthy individuals. Oftentimes, these infections involve the skin. Primary immune deficiency may also present with noninfectious cutaneous signs, such as eczema; erythroderma; granulomas; dysplasia of the skin, hair, nails, or teeth; pigmentary changes; angioedema; urticaria; vasculitis; or autoimmune skin disease due to immune dysregulation. Prompt recognition of the underlying diagnosis and initiation of treatment decrease morbidity. This review provides the reader with an up-to-date summary of the common dermatologic manifestations of primary immune deficiency diseases. PMID:25269404

  17. [Dermato-mucosal manifestations of Behçet's disease].

    PubMed

    Francès, C

    1999-11-01

    Oral and genital aphthae are the main clinical dermatologic manifestations of Behçet's disease. They look like those that occur in other aphthosis. Cutaneous lesions include pseudofolliculitis, folliculitis, erythema nodosum-like lesions, Sweet's-like lesions and pyoderma gangrenosum-like lesions. Histologically, these lesions are frequently perivascular with proeminent infiltrates of neutrophils and/or lymphocytes. Hypersensibility to needle pricks is explored by the pathergy test which sensibility is highly variable depending on the countries. When there is no systemic lesions requiring oral corticosteroids or immunosuppressive therapy, colchicine, aspirine, or dapsone may be prescribed. Thalidomide is sometimes required if aphthosis is refractory to other treatments despite its neurotixic and teratogenic effects. PMID:10637669

  18. Cerebellar Stroke-manifesting as Mania.

    PubMed

    Jagadesan, Venkatesan; Thiruvengadam, Kannapiran R; Muralidharan, Rengarajalu

    2014-07-01

    Secondary mania resulting from cerebral Cortex are described commonly. But secondary mania produced by cerebellar lesions are relatively uncommon. This case report describes a patient who developed cerebellar stoke and manic features simultaneously. 28 years old male developed giddiness and projectile vomiting. Then he would lie down for about an hour only to find that he could not walk. He became quarrelsome. His Psycho motor activities and speech were increased. He was euphoric and was expressing grandiose ideas. Bender Gestalt Test showed signs of organicity. Score in Young mania relating scale was 32; productivity was low in Rorschach. Neurological examination revealed left cerebellar signs like ataxia and slurring of speech. Computed tomography of brain showed left cerebellar infarct. Relationship between Psychiatric manifestations and cerebellar lesion are discussed. PMID:25035567

  19. Reading Like a Historian: Manifest Destiny

    NSDL National Science Digital Library

    Stanford History Education Group

    2012-10-01

    In this lesson, students analyze maps, art, and primary source documents in an effort to answer the central historical question: How did Americans justify westward expansion? To begin the lesson, students will examine a painting entitled “American Progress.” Students will compare 2 maps of the U.S.: a political map from 1872 and an electoral map from 1816. Next, students examine another 1816 map; the map is unusual in that it depicts the U.S. stretching to the Pacific—decades before this actually happened! Students will read 2 passages by John O’Sullivan, coiner of the phrase “Manifest Destiny,” and answer guiding questions. A final class discussion reviews students’ answers and touches on the subject of American Exceptionalism.

  20. Manifestations of top compositeness at colliders.

    SciTech Connect

    Kumar, K.; Tait, T. M. P.; Vega-Morales, R.; High Energy Physics; Northwestern Univ.

    2009-01-01

    We explore the possibility that the right-handed top quark is composite, identifying possible signatures of compositeness and how they might manifest themselves at the LHC and Tevatron. We perform a complete analysis of the dimension six modifications of the top coupling to gluons and find that cancellations among operators in the t{bar t} rate allow for very low compositeness scales, but this can be drastically improved by looking at kinematic distributions. Turning to the LHC, we examine four top production from a dimension six four-top operator and estimate the LHC with 100 fb{sup -1} collected luminosity to be sensitive to compositeness scales as high as 5 TeV.

  1. Acoustic manifestations of gas hydrate shelled bubbles

    NASA Astrophysics Data System (ADS)

    Maksimov, A. O.; Sosedko, E. V.

    2009-11-01

    The hydrocarbon seeps emitting buoyant bubble plumes from seafloor vents—gas flares have been actively investigated in different regions of the World Ocean, in particular, on the Sakhalin slope in the Sea of Okhotsk. The gas flares can be easily detected by regular echo sounders, because the scattering cross section of a gas bubble is large. Within the gas-hydrate stability zone—for high hydrostatic pressures and low temperatures, methane-hydrate ice skins are formed on rising seep bubbles which are typically methane. The objective of the present study was to develop a suitable model describing rheological characteristics of gas-hydrate shell and to analyze acoustic manifestations of such bubbles for the frequency range used in marine field experiments.

  2. Neuro-ophthalmic manifestations of prematurity.

    PubMed

    Chhablani, Preeti Patil; Kekunnaya, Ramesh

    2014-10-01

    Increasing rates of preterm births coupled with better survival of these infants have resulted in higher prevalence of systemic and ocular complications associated with prematurity. In addition to retinopathy of prematurity, infants who are born preterm may suffer from severe visual impairment as a result of hypoxic ischemic encephalopathy, hypoglycemia, and other metabolic imbalances. The effect of these processes on the anterior visual pathway may result in optic atrophy, optic nerve hypoplasia or optic disc cupping and affection of the posterior visual pathway leads to cortical visual impairment (CVI). Other ocular associations include strabismus, nystagmus, and ocular motor abnormalities such as tonic down gaze and defective saccades and pursuits. Cortical and subcortical involvement also manifests as defects in functional vision and these have not yet been completely understood. Children with CVI may have visual field defects, photophobia, defective visual processing, and deficient color vision. Since most of these children also suffer from additional systemic disabilities, evaluation, and management remains a challenge. However, early diagnosis and initiation of rehabilitation therapy can prove to be of significant benefit in these children. PMID:25449932

  3. Hypersensitivity manifestations to the fruit mango.

    PubMed

    Sareen, Richa; Shah, Ashok

    2011-04-01

    The objectives of this study are 1) To review the published data and document the current knowledge on allergic manifestations to the fruit mango 2) To highlight the two distinct clinical presentations of hypersensitivity reactions caused by mango 3) To discuss the role of cross-reactivity 4) To increase awareness of potentially life threatening complications that can be caused by allergy to mango. An extensive search of the literature was performed in Medline/PubMed with the key terms "mango", "anaphylaxis", "contact dermatitis", "cross-reactivity", "food hypersensitivity", "oral allergy syndrome" and "urticaria". The bibliographies of all papers thus located were searched for further relevant articles. A total of 17 reports describing 22 patients were documented, including ten patients with immediate hypersensitivity reaction and twelve patients with delayed hypersensitivity reaction to mango. Ten of these patients (four with immediate reaction; six with delayed reaction) were from geographical areas cultivating mango, whereas twelve patients (six with immediate reaction; six with delayed reaction) were from the countries where large scale mango cultivation does not occur. The clinical features, pathogenesis and diagnostic modalities of both these presentations are highlighted. The fruit mango can cause immediate and delayed hypersensitivity reactions, as also "oral allergy syndrome". Although rare, it can even result in a life threatening event. Reactions may even occur in individuals without prior exposure to mango, owing to cross reactivity. It is imperative to recognize such a phenomenon early so as to avoid potentially severe clinical reactions in susceptible patients. PMID:22053296

  4. Radiation nephritis. Clinical manifestations and pathophysiologic mechanisms

    SciTech Connect

    Krochak, R.J.; Baker, D.G.

    1986-05-01

    Radiation nephritis is both volume and dose related. Clinical experience would indicate that a minimum of one third of the renal volume needs to be excluded from nephrotoxic doses which appears to have a threshold of 2,000 cGy. The site of damage leading to renal failure appears to be the microvasculature ultimately expressed as glomerulosclerosis. How much direct damage to the tubular system contributes to this process is unclear, but undoubtedly the resultant systemic physiologic effects potentiate the expression of damage in the irradiated kidney. The acute syndrome, with all the potential manifestations of renal failure, rarely presents sooner than six months and appears to have no clear prodrome, although it would seem reasonable that a subclinical syndrome consisting of abnormalities detectable by urinalysis may occur. Treatment of radiation-induced nephritis or hypertension is no different from treatment for nephritis from any other cause and should be aggressive with lifelong follow-up. Carcinogenesis is a rare late expression of radiation-induced kidney damage. 25 references.

  5. On the surface manifestations of ship wakes.

    NASA Astrophysics Data System (ADS)

    Kapustin, Ivan; Ermakov, Stanislav; Lazareva, Tatyana

    2010-05-01

    During the field experiments on the Black Sea and on the Gorky Reservoir for the last 4 years the widening of the turbulent region generated by surface ships and the surface manifestations of the ship wakes has been studied. Measurements of currents in ship wakes have been made using ADCP (Acoustic Doppler Current Profiler) deployed from a motor boat. It was obtained that the time dependence of the wake width could be described approximately by a 0.4-power function, and the depth of wake remained constant at its initial stage, the latter allowed one to consider the wake widening as a one-dimensional process. We have developed a simple one-dimensional model of ship wake evolution using the semi-empirical theory of turbulence, and the initial stage of the wake widening (when neglecting dissipation) was described by the equation of turbulent energy balance with the pulse initial condition. We also observed in experiment mean circulating currents in the wake region resulting in the wind wave intensification on the boundaries of the wake region. It was shown that the later stage of the wake evolution is characterized by the presence of slicks bands on the edges of the wake. The slick bands formation is a result of the surfactants transport due to air bubbles in the turbulent wake and their compression by the mean currents. The work was supported by RFBR (projects 08-05-00634, 08-05-97011), the Program RAN Radiophysics, and the IPY THORPEX Project.

  6. Monogenic Autoinflammatory Diseases: Concept And Clinical Manifestations

    PubMed Central

    De Jesus, Adriana Almeida; Goldbach-Mansky, Raphaela

    2013-01-01

    The objectives of this review are to describe the clinical manifestations of the growing spectrum of monogenic autoinflammatory diseases including recently described syndromes. The autoinflammatory diseases can be grouped based on clinical findings: 1. the three classic hereditary “periodic fever syndromes”, familial Mediterranean Fever (FMF); TNF receptor associated periodic syndrome (TRAPS); and mevalonate kinase deficiency/hyperimmunoglobulinemia D and periodic fever syndrome (HIDS); 2. the cryopyrin associated periodic syndromes (CAPS), comprising familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and neonatal-onset multisystem inflammatory disease (NOMID) or CINCA, and; 3. pediatric granulomatous arthritis (PGA); 4. disorders presenting with skin pustules, including deficiency of interleukin 1 receptor antagonist (DIRA); Majeed syndrome; pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome; deficiency of interleukin 36 receptor antagonist (DITRA); CARD14 mediated psoriasis (CAMPS), and early-onset inflammatory bowel diseases (EO-IBD); 5. inflammatory disorders caused by mutations in proteasome components, the proteasome associated autoinflammatory syndromes (PRAAS) 6. very rare conditions presenting with autoinflammation and immunodeficiency. PMID:23711932

  7. Sickle cell disease: renal manifestations and mechanisms.

    PubMed

    Nath, Karl A; Hebbel, Robert P

    2015-03-01

    Sickle cell disease (SCD) substantially alters renal structure and function, and causes various renal syndromes and diseases. Such diverse renal outcomes reflect the uniquely complex vascular pathobiology of SCD and the propensity of red blood cells to sickle in the renal medulla because of its hypoxic, acidotic, and hyperosmolar conditions. Renal complications and involvement in sickle cell nephropathy (SCN) include altered haemodynamics, hypertrophy, assorted glomerulopathies, chronic kidney disease, acute kidney injury, impaired urinary concentrating ability, distal nephron dysfunction, haematuria, and increased risks of urinary tract infections and renal medullary carcinoma. SCN largely reflects an underlying vasculopathy characterized by cortical hyperperfusion, medullary hypoperfusion, and an increased, stress-induced vasoconstrictive response. Renal involvement is usually more severe in homozygous disease (sickle cell anaemia, HbSS) than in compound heterozygous types of SCD (for example HbSC and HbS?(+)-thalassaemia), and is typically mild, albeit prevalent, in the heterozygous state (sickle cell trait, HbAS). Renal involvement contributes substantially to the diminished life expectancy of patients with SCD, accounting for 16-18% of mortality. As improved clinical care promotes survival into adulthood, SCN imposes a growing burden on both individual health and health system costs. This Review addresses the renal manifestations of SCD and focuses on their underlying mechanisms. PMID:25668001

  8. Gastrointestinal Manifestations of Patients with Chronic Granulomatous Disease

    Microsoft Academic Search

    Masoud Movahedi; Asghar Aghamohammadi; Nima Rezaei; Abolhasan Farhoudi; Mostafa Moin; Mohammad Gharagozlou; Davoud Mansouri; Saba Arshi; Lida Atarod; Bahram MirSaeid Ghazi; Nikrad Shahnavaz; Ali Babaei Jandaghi; Kamran Abolmaali; Maryam Mahmoudi; Nasrin Bazargan; Akefeh Ahmadi Afshar; Mohammad Nabavi

    Chronic Granulomatous Disease (CGD) represents a group of inherited disorders of phagocytic system, manifesting recurrent infections at different sites. The present study was accomplished in order to determine the gastrointestinal manifestations of CGD patients. Fifty-seven patients (38 males and 19 females) with CGD, who had been referred to three immunodeficiency referral centers in Iran, were studied during a 24-year period

  9. Refractory Hypoglycemia Presenting as First Manifestation of Advanced Hepatocellular Carcinoma

    PubMed Central

    Reddy, Duvurru Nageshwar; Kiat, Tan Chee

    2014-01-01

    Hypoglycemia is a well-established paraneoplastic manifestation of hepatocellular carcinoma (HCC). However, hypoglycemia presenting as the primary presentation of HCC is extremely rare. Most cases are resistant to glucose infusion and may lead to severe complications such as hypoglycemic seizures. We present a patient who had hypoglycemia as first manifestation of HCC and was managed conservatively.

  10. 40 CFR 265.71 - Use of manifest system.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ...Section 262.23(c) of this chapter requires the generator to send three copies of the manifest to the facility when haz-ard-ous waste is sent by rail or water (bulk shipment).] (5) Retain at the facility a copy of the manifest and...

  11. 40 CFR 265.71 - Use of manifest system.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ...Section 262.23(c) of this chapter requires the generator to send three copies of the manifest to the facility when haz-ard-ous waste is sent by rail or water (bulk shipment).] (5) Retain at the facility a copy of the manifest and...

  12. A Review of the Latent and Manifest Benefits (LAMB) Scale

    ERIC Educational Resources Information Center

    Muller, Juanita; Waters, Lea

    2012-01-01

    The latent and manifest benefits (LAMB) scale (Muller, Creed, Waters & Machin, 2005) was designed to measure the latent and manifest benefits of employment and provide a single scale to test Jahoda's (1981) and Fryer's (1986) theories of unemployment. Since its publication in 2005 there have been 13 studies that have used the scale with 5692…

  13. 8 CFR 251.1 - Arrival manifests and lists.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ...States port must annotate the manifest presented at the onward port to indicate the time, date, and place of refueling. The surname, given name, and middle initial of each alien crewman listed also shall be shown on the manifest. In addition, the...

  14. 8 CFR 251.1 - Arrival manifests and lists.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ...States port must annotate the manifest presented at the onward port to indicate the time, date, and place of refueling. The surname, given name, and middle initial of each alien crewman listed also shall be shown on the manifest. In addition, the...

  15. 8 CFR 251.1 - Arrival manifests and lists.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ...States port must annotate the manifest presented at the onward port to indicate the time, date, and place of refueling. The surname, given name, and middle initial of each alien crewman listed also shall be shown on the manifest. In addition, the...

  16. 8 CFR 251.1 - Arrival manifests and lists.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ...States port must annotate the manifest presented at the onward port to indicate the time, date, and place of refueling. The surname, given name, and middle initial of each alien crewman listed also shall be shown on the manifest. In addition, the...

  17. 8 CFR 251.1 - Arrival manifests and lists.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ...States port must annotate the manifest presented at the onward port to indicate the time, date, and place of refueling. The surname, given name, and middle initial of each alien crewman listed also shall be shown on the manifest. In addition, the...

  18. Musculoskeletal manifestations of mild osteogenesis imperfecta in the adult

    Microsoft Academic Search

    Fergus E. McKiernan

    2005-01-01

    The musculoskeletal manifestations of mild forms of osteogenesis imperfecta are not well defined in the adult. The aim of this study was to characterize the musculoskeletal manifestations and resulting impairments reported by adults with mild osteogenesis imperfecta. For this task a survey of musculoskeletal symptoms and impairments was hosted on the Osteogenesis Imperfecta Foundation web site for 6 weeks. Survey

  19. HYPEREOSINOPHILIC SYNDROME: CUTANEOUS INVOLVEMENT AS THE SOLE MANIFESTATION

    PubMed Central

    Sundaramurthi, Vidya lakshmi; Prabhavathy, D; Somasundaram, S V; Wahab, Afthab Jameela

    2011-01-01

    Hypereosinophilic syndrome (HES) encompasses a group of leukoproliferative disorders with variable involvement of the internal organs. More than half of all patients have cutaneous involvement. In a minority of the reported cases, skin involvement has been the only manifestation of HES . We report one such rare case of HES, with cutaneous involvement as the sole manifestation. PMID:21572808

  20. 40 CFR 264.71 - Use of manifest system.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ...paper copy to EPA, the owner or operator may transmit to the EPA system an image file of Page 1 of the manifest, or both a data string file and the image file corresponding to Page 1 of the manifest. Any data or image files transmitted to EPA under this...

  1. Dermatologic manifestation of hyperandrogenism: a retrospective chart review.

    PubMed

    Clark, Charlotte M; Rudolph, Jennifer; Gerber, Donald A; Glick, Sharon; Shalita, Alan R; Lowenstein, Eve J

    2014-01-01

    Several studies have described a wide spectrum of hyperandrogenism diseases, many of which are difficult to distinguish from each other. In order to better understand diseases of hyperandrogenism, the authors performed a retrospective study of the cutaneous features and metabolic findings in women with hyperandrogenism. A retrospective chart analysis compiled by three dermatologists in both academic and private settings was performed, including patients presenting with > or = 2 manifestations of hyperandrogenism. Relevant dermatologic and associated manifestations and laboratory and imaging study findings were reviewed. Moderate to severe acne was the most common manifestation. Other common manifestations that patients first presented with include hirsutism, acanthosis nigricans, androgenic alopecia, and skin tags. Oligomenorrhea was the most common systemic presenting sign. Statistical analysis of various clinical markers revealed correlations with hyperandrogenemia. Acanthosis nigricans and hirsutism were found to be useful clinical markers for hyperandrogenism, whereas androgenic alopecia was not. This study provides some insights into the presentation and diverse manifestations seen in hyperandrogenism. PMID:24933845

  2. Diagnosis and treatment of HIV-associated manifestations in otolaryngology

    PubMed Central

    lacovou, Emily; Vlastarakos, Petros V.; Papacharalampous, George; Kampessis, George; Nikolopoulos, Thomas P.

    2012-01-01

    Almost 30 years after its first description, HIV still remains a global pandemic. The present paper aims to review the current knowledge on the ear, nose and throat (ENT) manifestations of HIV infection, and present the available diagnostic and treatment options. A literature review was conducted in Medline and other available database sources. Information from related books was also included in the data analysis. It is well acknowledged that up to 80% of HIV-infected patients eventually develop ENT manifestations; among which, oral disease appears to be the most common. Oro-pharyngeal manifestations include candidiasis, periodontal and gingival disease, HSV and HPV infection, oral hairy leucoplakia, Kaposi's sarcoma, and non-Hodgkin's lymphoma. ENT manifestations in the neck can present as cervical lymphadenopathy or parotid gland enlargement. Respective nasal manifestations include sinusitis (often due to atypical bacteria), and allergic rhinitis. Finally, otological manifestations include otitis (externa, or media), inner ear involvement (sensorineural hearing loss, disequilibrium), and facial nerve palsy (up to 100 times more frequently compared to the general population). Although ENT symptoms are not diagnostic of the disease, they might be suggestive of HIV infection, or related to its progression and the respective treatment failure. ENT doctors should be aware of the ENT manifestations associated with HIV disease, and the respective diagnosis and treatment. A multi-disciplinary approach may be required to provide the appropriate level of care to HIV patients. PMID:24470939

  3. Matrix Methods for Optimal Manifesting of Multinode Space Exploration Systems

    E-print Network

    Grogan, Paul Thomas

    This paper presents matrix-based methods for determining optimal cargo manifests for space exploration. An exploration system is defined as a sequence of in-space and on-surface transports between multiple nodes coupled ...

  4. Management of Musculoskeletal Manifestations in Inflammatory Bowel Disease

    PubMed Central

    Sheth, Tejas; Pitchumoni, C. S.; Das, Kiron M.

    2015-01-01

    Musculoskeletal manifestations are the most common extraintestinal manifestations in inflammatory bowel diseases. Some appendicular manifestations are independent of gut inflammation and are treated with standard anti-inflammatory strategies. On the other hand, axial involvement is linked to gut inflammatory activity; hence, there is a considerable amount of treatment overlap. Biological therapies have revolutionized management of inflammatory bowel diseases as well as of associated articular manifestations. Newer mechanisms driving gut associated arthropathy have surfaced in the past decade and have enhanced our interests in novel treatment targets. Introduction of biosimilar molecules is expected in the US market in the near future and will provide an opportunity for considerable cost savings on healthcare. A multidisciplinary approach involving a gastroenterologist, rheumatologist, and physical therapist is ideal for these patients.

  5. Dust Acoustic Mode Manifestations in Earth's Dusty Ionosphere

    SciTech Connect

    Kopnin, S.I.; Popel, S.I. [Institute for Dynamics of Geospheres RAS, Leninsky pr. 38, bld. 1, 119334 Moscow (Russian Federation)

    2005-10-31

    Dust acoustic mode manifestations in the dusty ionosphere are studied. The reason for an appearance of the low-frequency radio noises associated with such meteor fluxes as Perseids, Orionids, Leonids, and Gemenids is determined.

  6. 40 CFR 761.208 - Use of the manifest.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ...PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES CONTROL ACT ...AND USE PROHIBITIONS PCB Waste Disposal Records and Reports...1) The generator of PCB waste shall: (i) Sign the manifest...accompany the shipment of PCB waste. (2) For bulk...

  7. 40 CFR 761.208 - Use of the manifest.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ...PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES CONTROL ACT ...AND USE PROHIBITIONS PCB Waste Disposal Records and Reports...1) The generator of PCB waste shall: (i) Sign the manifest...accompany the shipment of PCB waste. (2) For bulk...

  8. Unusual manifestation of a multiple myeloma in the hyoid bone.

    PubMed

    Sychra, V; Eßer, D; Kosmehl, H; Herold, M

    2013-01-01

    The most common manifestation of plasma cell neoplasms is multiple myeloma. Solitary and localized tumours in the form of solitary plasmacytoma of the bone or extramedullary plasmacytoma are rare. In the late stages of multiple myeloma, bulky bone tumour infiltrates may be found which may be the primary clinical manifestation of the previously unknown malignancy. We report a case of a hyoid bone tumour with extramedullary plasma cell infiltrates in the oropharynx in multiple myeloma. PMID:22241868

  9. Mucocutaneous manifestations of acquired hypoparathyroidism: An observational study.

    PubMed

    Sarkar, Somenath; Mondal, Modhuchanda; Das, Kapildev; Shrimal, Arpit

    2012-09-01

    Hypoparathyroidism is a disorder of calcium and phosphorus metabolism due to decreased secretion of parathyroid hormone. Hypoparathyroidism can be hereditary and acquired. Acquired hypoparathyroidism usually occurs following neck surgery (thyroid surgery or parathyroid surgery). Along with systemic manifestations, hypoparathyroidism produces some skin manifestations. Lack of study regarding mucocutaneous manifestations of acquired hypoparathyroidism prompted us to undertake this study. To evaluate the mucocutaneous manifestations of acquired hypoparathyroidism. An observational study done in a tertiary care hospital of Kolkata by comprehensive history taking, through clinical examination and relevant laboratory investigations. Twenty-one patients were included in the study. The commonest form of acquired hypoparathyroidism was neck surgery (thyroidectomy and parathyroidectomy operation). Mucocutaneous manifestations were present in 76.19% of patients. The most frequent mucocutaneous manifestation was found in the hairs like the loss of axillary hair (61.9%), loss of pubic hair (52.38%), coarsening of body hair (47.62%), and alopecia areata (9.52%). The nail changes noted were brittle and ridged nail, followed by onycholysis, onychosezia, and onychomedesis. The most common skin features were xerotic skin in 11 patients (52.38%), followed by pellagra-like skin pigmentation, pustular psoriasis and acne form eruption, bullous impetigo, etc. Mucosa was normal in all the cases excepting the one which showed oral candidiasis. PMID:23087872

  10. 19 CFR 103.31 - Information on vessel manifests and summary statistical reports.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ...vessel manifests and summary statistical reports. 103.31 Section...vessel manifests and summary statistical reports. (a) Disclosure...vessel manifests and summary statistical reports of imports and exports...publication information and data subject to the following...

  11. 19 CFR 103.31 - Information on vessel manifests and summary statistical reports.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ...vessel manifests and summary statistical reports. 103.31 Section...vessel manifests and summary statistical reports. (a) Disclosure...vessel manifests and summary statistical reports of imports and exports...publication information and data subject to the following...

  12. 19 CFR 103.31 - Information on vessel manifests and summary statistical reports.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ...vessel manifests and summary statistical reports. 103.31 Section...vessel manifests and summary statistical reports. (a) Disclosure...vessel manifests and summary statistical reports of imports and exports...publication information and data subject to the following...

  13. 19 CFR 103.31 - Information on vessel manifests and summary statistical reports.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ...vessel manifests and summary statistical reports. 103.31 Section...vessel manifests and summary statistical reports. (a) Disclosure...vessel manifests and summary statistical reports of imports and exports...publication information and data subject to the following...

  14. Database basics help manage hazardous waste manifest data

    SciTech Connect

    Nielsen, E.S. (Varian Associates Inc., Palo Alto, CA (United States))

    1993-07-01

    Hazardous waste generators spend considerable time filling out uniform hazardous waste manifests and such related documents as land disposal forms, profile sheets and laboratory test results. For many environmental professionals, the continual demand for updating and maintaining records is a burdensome task, especially when some records may be outdated, illegible or otherwise difficult to manage. One solution to the paperwork maze is using a commercial software program that allows users to print legible copies of manifests. Some programs create summary reports detailing shipping destinations, annual waste generation totals and other information. Features vary among available programs, and additional capabilities may require customized programming, which can be expensive. For those with reservations about committing to buying or using software specifically designed to produce or manage manifests -- or who have limited budgets -- generic, off-the-shelf database programs designed for personal computers (PCs) or Apple Macintosh equipment offer low-cost alternatives.

  15. Large cerebellar mass lesion: A rare intracranial manifestation of blastomycosis

    PubMed Central

    Munich, Stephan A.; Johnson, Andrew K.; Ahuja, Sumeet K.; Venizelos, Alexander; Byrne, Richard W.

    2013-01-01

    Background: Blastomyces dermatitidis is a dimorphic fungus found endemically in the Mississippi and Ohio River basins and in the Midwestern and Canadian provinces that border the Great Lakes. Unlike other fungal infections, it most commonly affects immunocompetent hosts. Blastomycosis typically manifests as pulmonary infection, but may affect nearly any organ, including the skin, bone, and genitourinary system. Central nervous system (CNS) blastomycosis is rare, but potentially fatal manifestation of this disease. When it does occur, it most commonly presents as acute or chronic meningitis. Case Description: We present a case of a patient who suffered intractable nausea and vomiting for several months before discovery of a large cerebellar blastomycoma causing mass effect and obstructive hydrocephalus. The enhancing lesion with unusual peripheral cystic structures is a unique radiographic appearance of CNS blastomycosis. Conclusion: We review this patient's purely intraparenchymal manifestation of CNS blastomycosis and describe the unique imaging characteristics encountered. PMID:24231945

  16. Dermatological manifestations of hepatitis C virus infection in Saudi Arabia.

    PubMed

    Halawani, Mona R

    2014-06-01

    The Saudi Ministry of Health data indicates that almost 32% of viral hepatitis cases were caused by hepatitis C virus (HCV). It has been widely reported that chronic HCV infection is associated with and may trigger or exacerbate many skin manifestations in 20-40% of patients visiting dermatologists. The most commonly encountered dermatological manifestations of HCV infection globally include mixed cryoglobulinemia, porphyria cutanea tarda, cutaneous and/or oral lichen planus, urticaria, pruritus, thrombocytopenic purpura, and psoriasis. The current article indicates that HCV infection is increasing in Saudi Arabia and approximately 12% of the reported dermatological manifestations are caused by HCV infection. We recommend the urgent need for large-scale, case-control studies to understand the impact of HCV infection in patients with skin disease. PMID:24888650

  17. Clinical manifestations of the aPL syndrome.

    PubMed

    Levy, R A

    1996-10-01

    The antiphospholipid syndrome may present itself to virtually all medical specialties. It can be as non-threatening as thrombophlebitis or mild thrombocytopenia or as severe as cerebral arterial infarct or the so called "catastrophic APS". In order to diagnose it properly, it is important to be aware of its clinical manifestations and laboratory peculiarities. The primary form of APS seems to be more common than the SLE related one. Because any artery or vein may be involved, the spectrum of clinical manifestations of APS is so wide that they include virtually all medical specialties. PMID:8902768

  18. Ocular manifestations of the potentially lethal rheumatologic and vasculitic disorders.

    PubMed

    Foster, C Stephen

    2013-06-01

    Vision threatening ocular inflammation may occur in patients with any of the acquired connective tissue disorders and vasculitic diseases. Additionally, the ocular inflammation may be the presenting manifestation of the disease, which leads the patient to seek medical care. Other manifestations of the potentially lethal disease may be subtle or absent, presenting the thoughtful ophthalmologist with the opportunity to make life saving discoveries. Necrotizing scleritis, peripheral ulcerative keratitis, and retinal vasculitis are the ocular findings which should prompt the ophthalmologist to initiate very aggressive measures aimed at discovering any evidence of extra-ocular abnormalities, laboratory or otherwise. Appropriate therapy will be sight saving and may be life saving. PMID:23688612

  19. A case of gallbladder cancer manifesting chylous ascites and chylothorax.

    PubMed

    Shiina, S; Ohshima, M; Fujino, H; Muto, H; Yasuda, H; Kawabe, T; Tagawa, K; Unuma, T; Ibukuro, K; Inoue, Y

    1989-04-01

    A case of gallbladder cancer manifesting both chylous ascites and chylothorax was reported. A 66-year-old man was hospitalized with milky ascites. The patient was diagnosed as having gallbladder cancer based on findings of endoscopic retrograde cholangiopancreatography (ERCP) and celiac angiography. The diagnosis of chylous ascites was confirmed by the presence of microscopically visible free fat and the biochemical analysis of the fluid. The patient also gradually developed chylous thoracic effusion. Autopsy revealed lymphogenous metastasis in multiple retroperitoneal and mediastinal nodes. Chylothorax and chyloperitoneum are relatively rare. Only five cases have been reported in Japan that manifested both conditions. PMID:2744338

  20. Gastrointestinal manifestations of patients with chronic granulomatous disease.

    PubMed

    Movahedi, Masoud; Aghamohammadi, Asghar; Rezaei, Nima; Farhoudi, Abolhasan; Pourpak, Zahra; Moin, Mostafa; Gharagozlou, Mohammad; Mansouri, Davoud; Arshi, Saba; Atarod, Lida; Mirsaeid Ghazi, Bahram; Shahnavaz, Nikrad; Babaei Jandaghi, Ali; Abolmaali, Kamran; Mahmoudi, Maryam; Bazargan, Nasrin; Ahmadi Afshar, Akefeh; Nabavi, Mohammad

    2004-06-01

    Chronic Granulomatous Disease (CGD) represents a group of inherited disorders of phagocytic system, manifesting recurrent infections at different sites. The present study was accomplished in order to determine the gastrointestinal manifestations of CGD patients. Fifty-seven patients (38 males and 19 females) with CGD, who had been referred to three immunodeficiency referral centers in Iran, were studied during a 24-year period (1980-2004). The median age at the time of study was 14.5 years old (1-56 years). The median onset age of symptoms was 5 months (1 month- 13.75 years), and that of diagnostic age was 5 years (2 months- 54.1 years), with a diagnostic delay of 33 months, on average. Seven patients were presented with acute diarrhea, 3 with oral candidiasis, and 2 with liver abscesses as the first chief complaints. Twenty-four cases (42.1%) had been complicated by gastrointestinal manifestations during their course of the disease. Of those, 12 cases (21.1%) had diarrhea, 7 (12.3%) oral candidiasis, 5 (8.8%) hepatitis, 4 (7.0%) hepatic abscess, and 2 cases (3.5%) gastric outlet obstruction. Also, failure to thrive was detected in 6 patients (10.5%). Four patients died (7%). CGD should be excluded in any patient with gastrointestinal manifestations especially chronic diarrhea, hepatic abscess, and gastric outlet obstruction. PMID:17301397

  1. Clinical manifestations of disordered microcirculatory perfusion in severe sepsis

    Microsoft Academic Search

    Stephen Trzeciak; Emanuel P Rivers

    2005-01-01

    Microcirculatory dysfunction plays a pivotal role in the development of the clinical manifestations of severe sepsis. Prior to the advent of new imaging technologies, clinicians had been limited in their ability to assess the microcirculation at the bedside. Clinical evidence of microcirculatory perfusion has historically been limited to physical examination findings or surrogates that could be derived from global parameters

  2. Pulmonary manifestations in adult patients with chronic granulomatous disease.

    PubMed

    Salvator, Hélène; Mahlaoui, Nizar; Catherinot, Emilie; Rivaud, Elisabeth; Pilmis, Benoit; Borie, Raphael; Crestani, Bruno; Tcherakian, Colas; Suarez, Felipe; Dunogue, Bertrand; Gougerot-Pocidalo, Marie-Anne; Hurtado-Nedelec, Margarita; Dreyfus, Jean-François; Durieu, Isabelle; Fouyssac, Fanny; Hermine, Olivier; Lortholary, Olivier; Fischer, Alain; Couderc, Louis-Jean

    2015-06-01

    Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by failure of superoxide production in phagocytic cells. The disease is characterised by recurrent infections and inflammatory events, frequently affecting the lungs. Improvement of life expectancy now allows most patients to reach adulthood. We aimed to describe the pattern of pulmonary manifestations occurring during adulthood in CGD patients. This was a retrospective study of the French national cohort of adult patients (?16?years old) with CGD. Medical data were obtained for 67 adult patients. Pulmonary manifestations affected two-thirds of adult patients. Their incidence was significantly higher than in childhood (mean annual rate 0.22 versus 0.07, p=0.01). Infectious risk persisted despite anti-infectious prophylaxis. Invasive fungal infections were frequent (0.11 per year per patient) and asymptomatic in 37% of the cases. They often required lung biopsy for diagnosis (10 out of 30). Noninfectious respiratory events concerned 28% of adult patients, frequently associated with a concomitant fungal infection (40%). They were more frequent in patients with the X-linked form of CGD. Immune-modulator therapies were required in most cases (70%). Respiratory manifestations are major complications of CGD in adulthood. Noninfectious pulmonary manifestations are as deleterious as infectious pneumonia. A specific respiratory monitoring is necessary. PMID:25614174

  3. The Development and Manifestation of Altruistic Caring: A Qualitative Inquiry

    ERIC Educational Resources Information Center

    Curry, Jennifer R.; Smith, Heather L.; Robinson, Edward H., III

    2009-01-01

    Qualitative, phenomenological research provides rich information about the constructive, life span perspectives of the manifestation and development of altruism. Using an interpretive phenomenological approach, this study investigated "altruism" as described by 34 older persons in a continuing care retirement community. The findings identified 13…

  4. Empirical Manifestations of Power among Adult Learners in Online Discussions

    ERIC Educational Resources Information Center

    Jun, JuSung

    2007-01-01

    This study examined the nature of power manifestations in a specific online learning setting. The two online classes selected for this study were Master's level courses in a professional school at a large state university in the United States. A total of 1340 postings were made in the two classes over the span of the semester. To test the research…

  5. [New therapeutics import in clinical manifestations on alcoholism (author's transl)].

    PubMed

    Pignol, F; Casanova, P

    The nutritional and neurologic manifestations of alcoholism can be corrected by neutralizing the topic effects of the alcoholemia on neurologic centers and the resultant metabolic excesses. Tiapride has obtained its preferencial action on the mesolimbic region, interesting therapeutic results in the different clinical forms of this type of intoxication. PMID:38510

  6. Pyoderma gangrenosum as first clinical manifestation of gastric adenocarcinoma.

    PubMed

    França, A E da Costa; Salvino, L K Braga; Leite, S H Rodrigues; Ferraz, J G Paraíso; Rocha, T D Santos; Cintra, M L; Velho, P E Neves; Souza, E M

    2006-04-01

    Pyoderma gangrenosum (PG) is a neutrophilic dermatosis of unknown etiology characterized by typical skin ulcers. It may be related to systemic disorders but its association with solid tumors is very unusual. In this setting, we describe a patient in whom PG was the first and isolated manifestation of advanced gastric adenocarcinoma. PMID:16643144

  7. Quantum Theory of Gravity. II. The Manifestly Covariant Theory

    Microsoft Academic Search

    Bryce S. Dewitt

    1967-01-01

    Contrary to the situation which holds for the canonical theory described in the first paper of this series, there exists at present no tractable pure operator language on which to base a manifestly covariant quantum theory of gravity. One must construct the theory by analogy with conventional S-matrix theory, using the c-number language of Feynman amplitudes when nothing else is

  8. Social Support in Elderly Nursing Home Populations: Manifestations and Influences

    ERIC Educational Resources Information Center

    Rash, Elizabeth M.

    2007-01-01

    The purpose of this study was to describe the characteristics of social support and the influencing factors on social support in nursing home environments. Observations and staff questionnaires from two central Florida nursing homes were used in this grounded theory study to answer the following questions: (1) How is social support manifested? and…

  9. Manifestions of Berry's topological phase for the photon

    Microsoft Academic Search

    R. Y. Chiao; Y.-S. Wu

    1986-01-01

    Recently, Berry (1984) recognized in quantum mechanics a topological phase factor arising from the adiabatic transport of a system around a closed circuit, which is essentially the Aharonov-Bohm effect in parameter space. Here manifestations of this phase factor are considered for a photon in a state of adiabatically invariant helicity. An interferometer is suggested to see this phase. Also, an

  10. Laryngeal Manifestations of Relapsing Polychondritis And a Novel Treatment Option

    Microsoft Academic Search

    Lesley Childs; Scott Rickert; Oscar Calderon Wengerman; Robert Lebovics; Andrew Blitzer

    ObjectivesLaryngotracheal involvement in relapsing polychondritis (RP) is rare. However, it is one of the most common causes of death in this patient population. We present three patients who primarily presented with laryngeal manifestations of RP and a novel treatment option for bamboo nodules.

  11. Manifestations cutanées du cancer du sein chez l’homme

    Microsoft Academic Search

    Fouzia Hali; Khadija Khadir; Wassima Idhammou; Fatima-Zahra Bensardi; Mohamed-Rachid Lefriyekh; Abdelatif Benider; Soumia Zamiati; Hakima Benchikhi

    2011-01-01

    ObjectiveThe appearance of skin symptoms in male breast is the main reason for consultation in our context. The aim of this study is to describe the various cutaneous manifestations of male breast cancer through a series of cases collected in a dermatology department.

  12. NEUROLOGIC MANIFESTATIONS AS THE PRESENTING SYMPTOMS IN LUNG CANCER

    Microsoft Academic Search

    M. Ghaffarpour; B. Mansoorian

    This case series reports 100 patients with lung cancer and their presenting neurologic symptoms and signs. 78% of patients were male and 22% were female. Mean age was 62± 1.04 years with a peak age between 65 and 75 years. Most patients presented with pulmonary problems (58%) and neurologic deficits as the presenting manifestations were found to be 30% along

  13. Stress Manifestation in High School Students: An Australian Sample.

    ERIC Educational Resources Information Center

    Moulds, John D.

    2003-01-01

    Assesses stress symptoms associated with main daily hassles among grades 7, 9 and 11 students in all-female, all-male, and coeducational Catholic high schools in Western Sydney, Australia. Factor analysis identified the stress manifestation domains of anxiety, anger, and physiological arousal. Differences in these are examined and a conceptual…

  14. Manifesting heterozygosity in sex-linked spastic paraplegia?

    Microsoft Academic Search

    I D Young; I F Pye; J R Moore

    1984-01-01

    An unusual form of hereditary spastic paraplegia is described. Affected females have a late-onset slowly progressive spastic paraparesis. Affected males show oligophrenia with a rapidly progressive spastic quadriplegia. The mode of inheritance is consistent with sex-linkage, with partial manifestation in female carriers.

  15. Pathological yawning as an ictal seizure manifestation in the elderly.

    PubMed

    Nicotra, Alessia; Khalil, Nofal M; Owbridge, P; Hakda, Mina; Beitverda, Younatan

    2012-01-01

    Excessive yawning has been reported in the peri-ictal period preceding or following seizures. We describe an exceptional case of an elderly man with impairment of consciousness and paroxysmal excessive yawning. We hypothesise that this can be regarded as an autonomic seizure originating from diencephalic/brainstem structures, manifesting with yawning as an ictal phenomenon. PMID:23076687

  16. Mucocutaneous Manifestations in Japanese HIV-Positive Hemophiliacs

    Microsoft Academic Search

    Satoko Shimizu; Ko-Ron Chen; Hisamichi Tagami; Hideji Hanabusa

    2000-01-01

    Background: Although various mucocutaneous manifestations have been reported in patients infected with HIV by sexual transmission or intravenous drug use, the prevalence and characteristics of skin disorders in HIV-positive hemophiliacs coinfected with hepatitits C virus (HCV) have rarely been described. Objective: The purpose of this study was to clarify the characteristics of skin disorders in HIV-positive hemophiliacs and to identify

  17. The Dynamic Manifestation of Cognitive Structures in the

    E-print Network

    Bressler, Steven L.

    The Dynamic Manifestation of Cognitive Structures in the Cerebral Cortex Steven L. Bressler Center imposed by the structures of the internal and external environments. This construction is the dynamic be viewed as a dynamic interaction between cognitive and environmental structures. This means, of course

  18. Atypical manifestations of congenital parvovirus B19 infection

    Microsoft Academic Search

    Immacolata Savarese; Maria Pia De Carolis; Simonetta Costa; Gabriella De Rosa; Sara De Carolis; Serafina Lacerenza; Costantino Romagnoli

    2008-01-01

    Parvovirus B19 infection in pregnancy is associated with fetal anemia, hydrops and fetal death. We report two unusual manifestations\\u000a of vertical parvovirus B19 infection. The first patient developed hydrops as consequence of myocarditis with involvement of\\u000a sino-atrial node. The other had pleural effusion reactive to the hepatic localization of the virus.

  19. Testing Manifest Monotonicity Using Order-Constrained Statistical Inference

    ERIC Educational Resources Information Center

    Tijmstra, Jesper; Hessen, David J.; van der Heijden, Peter G. M.; Sijtsma, Klaas

    2013-01-01

    Most dichotomous item response models share the assumption of latent monotonicity, which states that the probability of a positive response to an item is a nondecreasing function of a latent variable intended to be measured. Latent monotonicity cannot be evaluated directly, but it implies manifest monotonicity across a variety of observed scores,…

  20. Disseminated sporotrichosis as a manifestation of immune reconstitution inflammatory syndrome.

    PubMed

    Gutierrez-Galhardo, Maria Clara; do Valle, Antônio Carlos Francesconi; Fraga, Beatriz Lima Barros; Schubach, Armando Oliveira; Hoagland, Brenda Regina de Siqueira; Monteiro, Paulo César Fialho; Barros, Mônica Bastos de Lima

    2010-01-01

    We describe two cases of disseminated sporotrichosis as a manifestation of immune reconstitution inflammatory syndrome. After the initiation of highly active anti-retroviral therapy, one patient presented disseminated lesions, whereas the other patient's preexisting lesions worsened and became more extensive. Simultaneously, their CD4 T cell counts increased and HIV viral loads decreased. PMID:19019165

  1. Spermatic cord metastasis as early manifestation of small bowel adenocarcinoma

    PubMed Central

    Valizadeh, Nasim; Oskuie, Ali Eishi; Tehranchi, Ali

    2013-01-01

    Malignant tumors of the spermatic cord are rare. There are a few case reports on spermatic cord metastasis from colonic, gastric, pancreas, and prostatic cancer. Here, we report a 36-year-old man with brucellosis presenting with spermatic cord metastasis as early manifestation of small bowel adenocarcinoma. PMID:23662014

  2. Management and evaluation of extra-articular manifestations in spondyloarthritis

    PubMed Central

    Nurmohamed, Michael T.

    2012-01-01

    Spondyloarthritis (SpA) is a chronic inflammatory disease with either predominantly axial symptoms of the spine and sacroiliac joints (axial SpA, including ankylosing spondylitis) or predominantly arthritis (peripheral SpA). Next to these spinal and articular symptoms, many patients with SpA also have extra-articular manifestations (EAMs). EAMs associated with SpA include anterior uveitis (25–30%), psoriasis (10–25%) or inflammatory bowel disease (IBD) (5–10%) and cardiovascular manifestations. Peripheral arthritis occurs in approximately 30% of patients, especially in large joints, and shows an asymmetrical, oligoarticular pattern. Other common joint complaints are due to enthesitis, which manifest as extra-articular bony tenderness in areas such as the Achilles tendon. Acute anterior uveitis presents with acute pain, loss of vision and redness in one eye that usually subsides spontaneously after several weeks. Rapid treatment by an ophthalmologist is required to prevent synechiae formation which could ultimately result in glaucoma and blindness. Although less common, organ involvement in SpA can also be located in the heart, lungs or kidneys. The risk of cardiovascular events is increased in SpA. Cardiac manifestations can involve the aortic valve (1–10%) or the atrioventricular node and the risk of atherosclerotic events is increased in this group. Treatment of SpA includes physical exercise and nonsteroidal anti-inflammatory drugs (NSAIDs), and in case of peripheral arthritis, sulphasalazine can be added. When there is insufficient response to NSAIDs, tumor necrosis factor blockers, especially infliximab, etanercept, adalimumab and golimumab, are very effective in treating axial manifestations, arthritis, enthesitis and psoriasis. Anterior uveitis in SpA can be treated adequately by the ophthalmologist and in the case of refractory uveitis, treatment with adalimumab and infliximab seems to be more effective compared with etanercept. When IBD occurs with SpA, the use of NSAIDs should be minimized, except for celecoxib, and infliximab or adalimumab are preferred to etanercept. The incidence of atherosclerotic events or SpA-specific cardiac manifestations may be decreased by cardiovascular risk management or effective antirheumatic treatment. Overall it is important to realize that extra-articular manifestations frequently occur in patients with SpA and should be taken into account in the choice of treatment. PMID:23227118

  3. Optic neuritis as isolated manifestation of leptomeningeal carcinomatosis: a case report and systematic review of ocular manifestations of neoplastic meningitis.

    PubMed

    Lanfranconi, Silvia; Basilico, Paola; Trezzi, Ilaria; Borellini, Linda; Franco, Giulia; Civelli, Vittorio; Pallotti, Francesco; Bresolin, Nereo; Baron, Pierluigi

    2013-01-01

    Introduction. Leptomeningeal carcinomatosis occurs in about 5% of cancer patients. Ocular involvement is a common clinical manifestation and often the presenting clinical feature. Materials and Methods. We report the case of a 52-year old lady with optic neuritis as isolated manifestation of neoplastic meningitis and a review of ocular involvement in neoplastic meningitis. Ocular symptoms were the presenting clinical feature in 34 patients (83%) out of 41 included in our review, the unique manifestation of meningeal carcinomatosis in 3 patients (7%). Visual loss was the presenting clinical manifestation in 17 patients (50%) and was the most common ocular symptom (70%). Other ocular signs were diplopia, ptosis, papilledema, anisocoria, exophthalmos, orbital pain, scotomas, hemianopsia, and nystagmus. Associated clinical symptoms were headache, altered consciousness, meningism, limb weakness, ataxia, dizziness, seizures, and other cranial nerves involvement. All patients except five underwent CSF examination which was normal in 1 patient, pleocytosis was found in 11 patients, increased protein levels were observed in 16 patients, and decreased glucose levels were found in 8 patients. Cytology was positive in 29 patients (76%). Conclusion. Meningeal carcinomatosis should be considered in patients with ocular symptoms even in the absence of other suggestive clinical symptoms. PMID:24223306

  4. Optic Neuritis as Isolated Manifestation of Leptomeningeal Carcinomatosis: A Case Report and Systematic Review of Ocular Manifestations of Neoplastic Meningitis

    PubMed Central

    Basilico, Paola; Trezzi, Ilaria; Borellini, Linda; Franco, Giulia; Civelli, Vittorio; Bresolin, Nereo; Baron, Pierluigi

    2013-01-01

    Introduction. Leptomeningeal carcinomatosis occurs in about 5% of cancer patients. Ocular involvement is a common clinical manifestation and often the presenting clinical feature. Materials and Methods. We report the case of a 52-year old lady with optic neuritis as isolated manifestation of neoplastic meningitis and a review of ocular involvement in neoplastic meningitis. Ocular symptoms were the presenting clinical feature in 34 patients (83%) out of 41 included in our review, the unique manifestation of meningeal carcinomatosis in 3 patients (7%). Visual loss was the presenting clinical manifestation in 17 patients (50%) and was the most common ocular symptom (70%). Other ocular signs were diplopia, ptosis, papilledema, anisocoria, exophthalmos, orbital pain, scotomas, hemianopsia, and nystagmus. Associated clinical symptoms were headache, altered consciousness, meningism, limb weakness, ataxia, dizziness, seizures, and other cranial nerves involvement. All patients except five underwent CSF examination which was normal in 1 patient, pleocytosis was found in 11 patients, increased protein levels were observed in 16 patients, and decreased glucose levels were found in 8 patients. Cytology was positive in 29 patients (76%). Conclusion. Meningeal carcinomatosis should be considered in patients with ocular symptoms even in the absence of other suggestive clinical symptoms. PMID:24223306

  5. Neonatal lupus with atypical cardiac and cutaneous manifestation.

    PubMed

    Morais, Sofia; Santos, Isabel Cristina; Pereira, Dolores Faria; Mimoso, Gabriela

    2013-01-01

    Neonatal lupus erythematosus is a rare, passively acquired autoimmune disease, caused by maternal autoantibodies. The most common manifestations are skin rash and congenital heart block. Cutaneous manifestations may be present at birth, but often develop within a few weeks after delivery. Congenital heart block may present as bradycardia in utero or during physical examination at birth. Approximately 40-60% of mothers are asymptomatic when the infants are diagnosed. We present a case of a child, born with erythematosus lesions in the face, scalp, trunk, limbs and nodules/papules on the palmar and plantar surfaces. He also had hepatosplenomegaly and thrombocytopenia. Echocardiography showed hyperechoic lesions on the anterior papilar muscle of the left ventricle and on the lateral cusp of the tricuspid valve. The mother had unexplained fever and vasculitic lesions in her hands and feet. Antinuclear antibodies, anti-SSa/Ro and anti-SSb/La were positive in the mother and child, making the diagnosis of neonatal lupus. PMID:23839605

  6. Clinical Manifestations and Outcomes of West Nile Virus Infection

    PubMed Central

    Sejvar, James J.

    2014-01-01

    Since the emergence of West Nile virus (WNV) in North America in 1999, understanding of the clinical features, spectrum of illness and eventual functional outcomes of human illness has increased tremendously. Most human infections with WNV remain clinically silent. Among those persons developing symptomatic illness, most develop a self-limited febrile illness. More severe illness with WNV (West Nile neuroinvasive disease, WNND) is manifested as meningitis, encephalitis or an acute anterior (polio) myelitis. These manifestations are generally more prevalent in older persons or those with immunosuppression. In the future, a more thorough understanding of the long-term physical, cognitive and functional outcomes of persons recovering from WNV illness will be important in understanding the overall illness burden. PMID:24509812

  7. [Otomastoiditis as an initial clinical manifestation of Wegener's granulomatosis].

    PubMed

    De All, Jorge; Koutnouyan, Gabriel; Herrando, Sergio; Diez, Eduardo; Olmedo, Gloria; Gnocchi, Cesar

    2011-01-01

    Wegener's granulomatosis (WG) forms part of a group of primary vasculitis of the small and medium-sized blood vessels, associated with antineutrophil cytoplasmic antibodies (ANCA). This disease may affect any body organ, especially the upper and lower airways and the kidneys. Hardly ever is otomastoiditis the first and only clinical manifestation of generalized Wegener's granulomatosis. We present the case of a patient whose disease started with the inflammation of the middle ear, which was unresponsive to the usual treatment. Later he developed nasal, pulmonary and renal compromise. The determination of ANCA and a transbronchial biopsy confirmed the diagnosis of WG. We report this case to express the view that otomastoiditis in adults, which is refractory to the usual treatment, may seldom be the first and only clinical manifestation of WG. PMID:21296720

  8. Extraintestinal manifestations and complications in inflammatory bowel diseases

    PubMed Central

    Rothfuss, Katja S; Stange, Eduard F; Herrlinger, Klaus R

    2006-01-01

    Crohn’s disease (CD) and ulcerative colitis (UC) are chronic inflammatory bowel diseases (IBD) that often involve organs other than those of the gastrointestinal tract. These nonintestinal affections are termed extraintestinal symptoms. Differentiating the true extraintestinal manifestations of inflammatory bowel diseases from secondary extraintestinal complications, caused by malnutrition, chronic inflammation or side effects of therapy, may be difficult. This review concentrates on frequency, clinical presentation and therapeutic implications of extraintestinal symptoms in inflammatory bowel diseases. If possible, extraintestinal manifestations are differentiated from extraintestinal complications. Special attention is given to the more recently described sites of involvement; i.e. thromboembolic events, osteoporosis, pulmonary involvement and affection of the central nervous system. PMID:16937463

  9. Cutaneous manifestations of biological warfare and related threat agents.

    PubMed

    McGovern, T W; Christopher, G W; Eitzen, E M

    1999-03-01

    The specter of biological warfare (BW) looms large in the minds of many Americans. The US government has required that emergency response teams in more than 100 American cities be trained by the year 2001 to recognize and contain a BW attack. The US military is requiring active duty soldiers to receive immunization against anthrax. Dermatologists need not feel helpless in the face of a potential BW attack. Many potential agents have cutaneous manifestations that the trained eye of a dermatologist can recognize. Through early recognition of a BW attack, dermatologists can aid public health authorities in diagnosing the cause so that preventive and containment measures can be instituted to mitigate morbidity and mortality. This article reviews bacterial, viral, and toxin threat agents and emphasizes those that would have cutaneous manifestations following an aerosol attack. We conclude with clues that can help one recognize a biological attack. PMID:10086453

  10. Rheumatic manifestations and an epipharyngeal mass accompanied by myelodysplastic syndrome.

    PubMed

    Inoue, Mariko; Kano, Toshikazu; Ozaki, Takashi; Takahashi, Yuko; Yamashita, Hiroyuki; Kaneko, Hiroshi; Mimori, Akio

    2015-07-01

    We herein report two cases of myelodysplastic syndrome with rheumatic manifestations. (Case 1) A 70-year-old man presented with fever, arthritis and bone pain and developed cranial nerve palsy caused by an epipharyngeal mass. Steroid therapy led to a prolonged remission of the febrile condition and mass lesion. (Case 2) An 82-year-old male was treated for intractable polyarthritis and fever with steroid therapy, and serious side effects resulted in lethal pneumonia. We herein describe the entire course of steroid therapy in these two cases. Various rheumatic manifestations in myelodyaplastic syndrome often require empirical steroid therapy. It was effective for the soft tissue mass in Case 1, in which indolent lymphoma could not be denied, and was only partially effective for Case 2 of the febrile and putatively benign conditions, suggesting heterogeneous nature of rheumatic complications in myelodysplastic syndrome. PMID:24313918

  11. What type of different clinical manifestations can cardiac sarcoidosis present?

    PubMed

    ?entürk, Ay?egül; Mara?, Yüksel; Argüder, Emine; Karalezli, Ay?egül; Hasano?lu, H Canan; Ö?üt, Tuba; Ba?tu?, Serdal; Karabekir, Ercan

    2015-06-01

    Cardiac sarcoidosis is an infiltrative, granulomatous inflammatory disease of the myocardium. Generally, it can be difficult to diagnose cardiac sarcoidosis clinically because of the non-specific nature of its clinical manifestations. This property can be based on the presence of any clinical evidence of sarcoidosis in the other organs. We present two cases of cardiac sarcoidosis so as to demonstrate its different clinical manifestations. The first patient displayed no cardiac symptoms; the electrocardiogram showed an incidental right bundle branch block. Her cardiac magnetic resonance imaging (CMRI) revealed late-phase opaque material enhancement involving the inferior and inferoseptal segment of the left ventricle. The second patient was severely symptomatic in terms of cardiac involvement, and a transthoracic echocardiogram revealed global hypokinesia and septal brightness; his ejection fraction decreased to 45 %. These cases highlighted the challenges encountered in the diagnosis and treatment of cardiac sarcoidosis. CMRI should be considered in all patients who have suspected findings for cardiac involvement. PMID:25429793

  12. Ocular manifestations in the Hutchinson-Gilford progeria syndrome

    PubMed Central

    Chandravanshi, Shivcharan L; Rawat, Ashok Kumar; Dwivedi, Prem Chand; Choudhary, Pankaj

    2011-01-01

    The Hutchinson-Gilford progeria (HGP) syndrome is an extremely rare genetic condition characterized by an appearance of accelerated aging in children. The word progeria is derived from the Greek word progeros meaning ‘prematurely old’. It is caused by de novo dominant mutation in the LMNA gene (gene map locus 1q21.2) and characterized by growth retardation and accelerated degenerative changes of the skin, musculoskeletal and cardiovascular systems. The most common ocular manifestations are prominent eyes, loss of eyebrows and eyelashes, and lagophthalmos. In the present case some additional ocular features such as horizontal narrowing of palpebral fissure, superior sulcus deformity, upper lid retraction, upper lid lag in down gaze, poor pupillary dilatation, were noted. In this case report, a 15-year-old Indian boy with some additional ocular manifestations of the HGP syndrome is described. PMID:22011502

  13. Neonatal lupus with atypical cardiac and cutaneous manifestation

    PubMed Central

    Morais, Sofia; Santos, Isabel Cristina; Pereira, Dolores Faria; Mimoso, Gabriela

    2013-01-01

    Neonatal lupus erythematosus is a rare, passively acquired autoimmune disease, caused by maternal autoantibodies. The most common manifestations are skin rash and congenital heart block. Cutaneous manifestations may be present at birth, but often develop within a few weeks after delivery. Congenital heart block may present as bradycardia in utero or during physical examination at birth. Approximately 40–60% of mothers are asymptomatic when the infants are diagnosed. We present a case of a child, born with erythematosus lesions in the face, scalp, trunk, limbs and nodules/papules on the palmar and plantar surfaces. He also had hepatosplenomegaly and thrombocytopenia. Echocardiography showed hyperechoic lesions on the anterior papilar muscle of the left ventricle and on the lateral cusp of the tricuspid valve. The mother had unexplained fever and vasculitic lesions in her hands and feet. Antinuclear antibodies, anti-SSa/Ro and anti-SSb/La were positive in the mother and child, making the diagnosis of neonatal lupus. PMID:23839605

  14. [Cutaneous manifestations of internal diseases: purpura, livedo, pyoderma gangrenosum].

    PubMed

    Trüeb, R M

    2003-09-01

    Dermatology and internal medicine have in common that many systemic diseases manifest with skin symptoms that are easily accessible for both diagnostic and investigative procedures. Even if modern practice of medicine tends towards organ specific particularity of specialists, dermatology traditionally strives for interdisciplinary communication. Cutaneous manifestations of internal diseases present in various forms. In a syndrome, a number of symptoms present in an identical pattern, and pattern recognition is diagnostic. Vice versa, a distinct skin sign can be a clue to various internal disorders, that have to be considered in the differential diagnosis. Purpura and livedo due to either inflammatory (immune complex mediated or associated with ANCA) or vasoocclusive microthrombotic vascular disease are presented as examples. Finally, subtile variations in key symptoms of the skin, such as of pyoderma gangrenosum, may give a clue to a specific underlying disease, such as rheumatoid arthritis, inflammatory bowel disease, monoclonal gammopathy, or hematologic disease, as exemplified by the variants of pyoderma gangrenosum. PMID:14526631

  15. Staphylococcus aureus infections: epidemiology, pathophysiology, clinical manifestations, and management.

    PubMed

    Tong, Steven Y C; Davis, Joshua S; Eichenberger, Emily; Holland, Thomas L; Fowler, Vance G

    2015-07-01

    Staphylococcus aureus is a major human pathogen that causes a wide range of clinical infections. It is a leading cause of bacteremia and infective endocarditis as well as osteoarticular, skin and soft tissue, pleuropulmonary, and device-related infections. This review comprehensively covers the epidemiology, pathophysiology, clinical manifestations, and management of each of these clinical entities. The past 2 decades have witnessed two clear shifts in the epidemiology of S. aureus infections: first, a growing number of health care-associated infections, particularly seen in infective endocarditis and prosthetic device infections, and second, an epidemic of community-associated skin and soft tissue infections driven by strains with certain virulence factors and resistance to ?-lactam antibiotics. In reviewing the literature to support management strategies for these clinical manifestations, we also highlight the paucity of high-quality evidence for many key clinical questions. PMID:26016486

  16. Progressive outer retinal necrosis: manifestation of human immunodeficiency virus infection.

    PubMed

    Lo, Phey Feng; Lim, Rongxuan; Antonakis, Serafeim N; Almeida, Goncalo C

    2015-01-01

    We present the case of a 54-year-old man who developed progressive outer retinal necrosis (PORN) as an initial manifestation of HIV infection without any significant risk factors for infection with HIV. PORN is usually found as a manifestation of known AIDS late in the disease. Our patient presented with transient visual loss followed by decrease in visual acuity and facial rash. Subsequent investigation revealed anterior chamber tap positive for varicella zoster virus (VZV), as well as HIV positivity, with an initial CD4 count of 48 cells/µL. Systemic and intravitreal antivirals against VZV, and highly active antiretroviral therapy against HIV were started, which halted further progression of retinal necrosis. This case highlights the importance of suspecting PORN where there is a rapidly progressive retinitis, and also testing the patient for HIV, so appropriate treatment can be started. PMID:25948844

  17. Langerhans Histiocytosis in a Child – Diagnosed by Oral Manifestations

    PubMed Central

    Golai, Shruti; Patil, Sandya Devi; Kakanur, Madhu; Paul, Santosh

    2015-01-01

    Langerhans Histiocytosis (LCH) is a rare reactive and proliferative disease of histiocytes with unknown etiology, characterized by excessive proliferation of histiocytes called Langerhans cells. It occurs mainly in children but occurrence in adults has also been reported. It manifests as punched out lesions in the skull, maxilla, mandible, sternum and other flat bones and causes rapid resorption of the alveolar bone leading to floating teeth appearance in the radiographs. This disease manifests initially in the oral cavity in most of the cases and can be diagnosed by careful clinical and radiological examination. Here, we present a case of LCH in a child which was diagnosed by a swelling in the mandibular region. PMID:26023652

  18. CUTANEOUS MANIFESTATIONS IN PATIENTS SUSPECTED OF CHIKUNGUNYA DISEASE

    PubMed Central

    Prashant, Soma; Kumar, A S; Basheeruddin, D D Mohammed; Chowdhary, T N; Madhu, B

    2009-01-01

    Context: An epidemic of chikungunya disease occurred in India during late 2005 through 2006 affecting nearly 1,400,000 people. Aim: To study the cutaneous manifestations in suspected cases of chikungunya disease. Settings and Design: Patients who attended our outpatient departments from January 2006 to September 2006 were prospectively included if they had symptoms of chikungunya disease according to the ‘case definition’ of the National Institute of Communicable Diseases, Directorate General of Health Services, Government of India. The criteria were an acute illness characterized by the sudden onset of fever and several symptoms such as joint pain, headache, backache, photophobia, and eruption during an epidemic of chikungunya fever in the absence of confirmatory serological tests. Materials and Methods: A total of 115 patients (65 men and 50 women) who satisfied the above criteria were enrolled for the study. Results: An erythematous maculopapular rash subsiding without any sequelae in 3-4 days was the most common cutaneous finding in our patients. Genital ulcers distributed predominantly over the scrotum and base of the penile shaft in men and labia majora in women were the second most common manifestation. Other manifestations included tenderness/edema of hands and feet, grouped hyperpigmented macules over the nose and cheeks, fixed drug eruptions, erythema nodosum, erythema multiformae, generalized urticarial eruptions, and flare up of pre-existing psoriasis and lichen planus. Conclusions: To conclude, a plethora of cutaneous manifestations were noted in suspected cases of chikungunya disease. Genital ulcers, to the best of our knowledge, have not been reported during the earlier epidemics but have been reported by others during the present one. PMID:20101307

  19. Manifestations of morphological impairments in Greek aphasia: A case study

    Microsoft Academic Search

    Kyrana Tsapkini; Gonia Jarema; Eva Kehayia

    2001-01-01

    The present study addresses the issue of morphological manifestations specific to non-fluent aphasia in Greek. The off-line performance of a Greek-speaking aphasic patient is investigated using Paradis'(1987) Bilingual Aphasia Test. His difficulties with inflectional morphology are further probed through a series of comprehension, production, repetition and reading tasks. Results obtained show a dissociation in the patient's processing of nouns and

  20. Ocular Manifestations of Late-Onset Behçet Disease

    Microsoft Academic Search

    Mehmet Citirik; Nilufer Berker; Murat Serkan Songur; Seyhan Sonar Ozkan; Orhan Zilelioglu

    2011-01-01

    Purpose: To describe the demographic characteristics and ocular manifestations of patients with late-onset Behçet disease (BD). Patients and Methods: The patients for this retrospective study were selected using the data obtained from the medical records of 16 patients with late-onset BD between January 2000 and May 2008. Results: Twelve (75%) of the cases were male and 4 (25%) were female.

  1. Brainstem infarcts as an early manifestation of streptococcus anginosus meningitis

    Microsoft Academic Search

    Sung B. Lee; Lyell K. Jones; Caterina Giannini

    2005-01-01

    Background and Purpose: Vasculitis and infarcts are well-established sequelae of bacterial meningitis. However, early large-vessel involvement is\\u000a rare, particularly within the brainstem. There has been one previous case report of a young male who presented with pontine\\u000a infarct as an early manifestation of Streptococcus milleri meningitis. We present another case of brainstem infarction associated with meningitis caused by Streptococcus anginosus

  2. MRI Findings and Clinical Manifestations in Rathke's Cleft Cyst

    Microsoft Academic Search

    N. Saeki; K. Sunami; Y. Sugaya; A. Yamaura

    1999-01-01

    Summary  ?We retrospectively analysed patients with histologically proven Rathke's cleft cyst (RCC) in relation to the clinical manifestations\\u000a and MRI findings, in particular, of cyst size and intensity in order to obtain an insight into their growing mechanisms, clinical\\u000a presentations and their management.\\u000a \\u000a ?Eleven patients with RCC were divided into two groups based on T1 weighted images(WI). The A group consisted

  3. Camptocormia as a clinical manifestation of mitochondrial myopathy

    Microsoft Academic Search

    José A. Gómez-Puerta; Pilar Peris; Josep M. Grau; Núria Guañabens

    2007-01-01

    Camptocormia is an unusual condition characterized by a progressive weakness of the extensor muscles of the spine that cause\\u000a an involuntary truncal flexion. Occasionally, camptocormia can be the clinical manifestation of an underlying myopathy, including\\u000a inflammatory or metabolic myopathies. We present a case of a 78-year-old female with camptocormia associated with a mitochondrial\\u000a myopathy. Additionally, we review the clinical characteristics

  4. [Haematological diseases with primary otorhinolaryngological manifestation (author's transl)].

    PubMed

    Tomity, I T; Sütö, M; Nagymajtényi, E; Martinovits, J

    1977-11-01

    There are reports of 10 haematological diseases with primary oto-rhino-laryngological manifestation. The clinical diagnoses were confirmed by laboratory results. The actual diseases were primarily hidden by a tonsillitis, bleeding of the nose after an eye-operation, by an extreme dyspnoe, or by a tumor-like change in the larynx. The authors stress the general necessity of immediate laboratory examinations in dept. of oto-rhinolaryngology. PMID:145533

  5. Premature centromere division: A possible manifestation of chromosome instability

    SciTech Connect

    Mehes, K. [Univ. Medical School, Pecs (Hungary); Buehler, E.M. [Univ. of Children`s Hospital, Basle (Switzerland)

    1995-03-13

    Retrospective analysis of {open_quotes}routine{close_quotes} chromosome preparations from 2 patients with Fanconi anemia and 2 others with ataxia-teleangiectasia showed increased chromosome breakage and a tendency to premature centromere division (PCD) with special reference to early separation of the large acrocentric (13-15) chromosomes. The findings suggest that PCD may be a manifestation of chromosome instability related to potential malignancy. 28 refs., 1 fig., 1 tab.

  6. [Apropos of 1 case of ophthalmologic manifestation of bilharziasis].

    PubMed

    Kabo, A M; Warter, A

    1993-01-01

    We present an ocular manifestation of schistosomiasis. We observed the case of a 10 year old young girl living near a flooding canal of the Niger river. She presented a tumor of the left lid associated with an important ptosis. This tumor resulted from numerous polyps of the conjunctiva. The histological analysis of a biopsy of the conjunctiva made the diagnostic. It was confirmed by the analysis of urine and by the important amelioration of inflammation resulting from a specific therapy. PMID:8219789

  7. Unusual thoracic CT manifestations of osteosarcoma: review of 16 cases

    Microsoft Academic Search

    Ruchi Rastogi; Rachana Garg; Sanjay Thulkar; Sameer Bakhshi; Ajay Gupta

    2008-01-01

    Pulmonary metastases are common in osteosarcoma and the most common appearance is of multiple well-defined nodules in the\\u000a lung parenchyma. However, a variety of atypical locations and presentations of osteosarcoma metastasis can occur in the thorax.\\u000a We present a review of the thoracic CT findings in 16 patients with histopathologically confirmed osteosarcoma with unusual\\u000a thoracic manifestations. The 16 patients were

  8. Chronic disorders with episodic manifestations: focus on epilepsy and migraine

    PubMed Central

    2006-01-01

    Epilepsy and migraine are chronic neurological disorders with episodic manifestations that are commonly treated in neurological practice and frequently occur together. In this review we examine similarities and contrasts between these disorders, with focus on epidemiology and classification, temporal coincidence, triggers, and mechanistically based therapeutic overlap. This investigation draws attention to unique aspects of both epilepsy and migraine, while identifying areas of crossover in which each specialty could benefit from the experience of the other. PMID:16426991

  9. Optic Neuritis as First Clinical Manifestations the Multiple Sclerosis

    PubMed Central

    Halilovic, Emina Alimanovic; Alimanovic, Ilda; Suljic, Enra; Hassan, Nabil Al

    2014-01-01

    Aim: To analyze the clinical signs of multiple sclerosis (MS) and show that optic neuritis is one of the first event, which indicates the development of disease. Patients and methods: The study involved 89 cases in which it confirmed MS at the time of the March 2009–2011. Since ophthalmological parameters were analyzed visual acuity (VA), visual field (VF), and retinal nerve fibre layer (RNFL) thickness of peripapillary rim by optic coherent tomography (OCT). Results: Ten(10) patients had ON as the first clinical manifestation of the disease which was statistically significant (X2 =9,7 p=0,01) compared to the manifestation of other clinical signs of disease. In VF, centrocecal scotomas were predominant in 50% of the subjects; the RNFL thinning of the neuroretinal rim was verified in all patients, most often in the upper quadrant. A month after pulse corticosteroid therapy, visual acuity in all patients with ON ranged from 0.6 to 1.0. Conclusion: ON is one of the first MS clinical manifestation. In VF, the most common disturbances are in the centrocecal area. The RNFL thinning was verified in all patients with OCT. PMID:25395886

  10. Some exact tests for manifest properties of latent trait models

    PubMed Central

    De Gooijer, Jan G.; Yuan, Ao

    2011-01-01

    Item response theory is one of the modern test theories with applications in educational and psychological testing. Recent developments made it possible to characterize some desired properties in terms of a collection of manifest ones, so that hypothesis tests on these traits can, in principle, be performed. But the existing test methodology is based on asymptotic approximation, which is impractical in most applications since the required sample sizes are often unrealistically huge. To overcome this problem, a class of tests is proposed for making exact statistical inference about four manifest properties: covariances given the sum are non-positive (CSN), manifest monotonicity (MM), conditional association (CA), and vanishing conditional dependence (VCD). One major advantage is that these exact tests do not require large sample sizes. As a result, tests for CSN and MM can be routinely performed in empirical studies. For testing CA and VCD, the exact methods are still impractical in most applications, due to the unusually large number of parameters to be tested. However, exact methods are still derived for them as an exploration toward practicality. Some numerical examples with applications of the exact tests for CSN and MM are provided. PMID:21562625

  11. 15 CFR 30.47 - Clearance or departure of carriers under bond on incomplete manifest.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ...provided in paragraph (a)(1), (2), and (3) of this section. (1) For manifests submitted electronically through AES, the condition of the bond shall be that the manifest and all required filing citations, exclusion, and/or exemption...

  12. 19 CFR 162.65 - Penalties for failure to manifest narcotic drugs or marihuana.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... Penalties for failure to manifest narcotic drugs or marihuana. 162.65 Section...AND SEIZURE Controlled Substances, Narcotics, and Marihuana § 162.65 Penalties for failure to manifest narcotic drugs or marihuana. (a)...

  13. 19 CFR 162.65 - Penalties for failure to manifest narcotic drugs or marihuana.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... Penalties for failure to manifest narcotic drugs or marihuana. 162.65 Section...AND SEIZURE Controlled Substances, Narcotics, and Marihuana § 162.65 Penalties for failure to manifest narcotic drugs or marihuana. (a)...

  14. 19 CFR 162.65 - Penalties for failure to manifest narcotic drugs or marihuana.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... Penalties for failure to manifest narcotic drugs or marihuana. 162.65 Section...AND SEIZURE Controlled Substances, Narcotics, and Marihuana § 162.65 Penalties for failure to manifest narcotic drugs or marihuana. (a)...

  15. 19 CFR 162.65 - Penalties for failure to manifest narcotic drugs or marihuana.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... Penalties for failure to manifest narcotic drugs or marihuana. 162.65 Section...AND SEIZURE Controlled Substances, Narcotics, and Marihuana § 162.65 Penalties for failure to manifest narcotic drugs or marihuana. (a)...

  16. 19 CFR 162.65 - Penalties for failure to manifest narcotic drugs or marihuana.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... Penalties for failure to manifest narcotic drugs or marihuana. 162.65 Section...AND SEIZURE Controlled Substances, Narcotics, and Marihuana § 162.65 Penalties for failure to manifest narcotic drugs or marihuana. (a)...

  17. Abstract--Pathological manifestations of epilepsy are generally associated with a set of clinical events that

    E-print Network

    Slatton, Clint

    Abstract--Pathological manifestations of epilepsy are generally associated with a set of clinical It is widely suggested that certain clinical manifestations of epilepsy are directly reflected in the changes

  18. 40 CFR 761.213 - Use of manifest-Commercial storage and disposal facility requirements.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ...PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES CONTROL ACT ...AND USE PROHIBITIONS PCB Waste Disposal Records and Reports...disposal facility receives PCB waste accompanied by a manifest...section to certify that the PCB waste covered by the manifest...

  19. 40 CFR 761.213 - Use of manifest-Commercial storage and disposal facility requirements.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ...PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES CONTROL ACT ...AND USE PROHIBITIONS PCB Waste Disposal Records and Reports...disposal facility receives PCB waste accompanied by a manifest...section to certify that the PCB waste covered by the manifest...

  20. Ionospheric manifestations of earthquakes and tsunamis in a dynamic atmosphere

    NASA Astrophysics Data System (ADS)

    Godin, Oleg A.; Zabotin, Nikolay A.; Zabotina, Liudmila

    2015-04-01

    Observations of the ionosphere provide a new, promising modality for characterizing large-scale physical processes that occur on land and in the ocean. There is a large and rapidly growing body of evidence that a number of natural hazards, including large earthquakes, strong tsunamis, and powerful tornadoes, have pronounced ionospheric manifestations, which are reliably detected by ground-based and satellite-borne instruments. As the focus shifts from detecting the ionospheric features associated with the natural hazards to characterizing the hazards for the purposes of improving early warning systems and contributing to disaster recovery, it becomes imperative to relate quantitatively characteristics of the observed ionospheric disturbances and the underlying natural hazard. The relation between perturbations at the ground level and their ionospheric manifestations is strongly affected by parameters of the intervening atmosphere. In this paper, we employ the ray theory to model propagation of acoustic-gravity waves in three-dimensionally inhomogeneous atmosphere. Huygens' wavefront-tracing and Hamiltonian ray-tracing algorithms are used to simulate propagation of body waves from an earthquake hypocenter through the earth's crust and ocean to the upper atmosphere. We quantify the influence of temperature stratification and winds, including their seasonal variability, and air viscosity and thermal conductivity on the geometry and amplitude of ionospheric disturbances that are generated by seismic surface waves and tsunamis. Modeling results are verified by comparing observations of the velocity fluctuations at altitudes of 150-160 km by a coastal Dynasonde HF radar system with theoretical predictions of ionospheric manifestations of background infragravity waves in the ocean. Dynasonde radar systems are shown to be a promising means for monitoring acoustic-gravity wave activity and observing ionospheric perturbations due to earthquakes and tsunamis. We will discuss the effects of the background ionospheric disturbances and uncertainty in atmospheric parameters on the feasibility and accuracy of retrieval of the open-ocean tsunami heights from observations of the ionosphere.

  1. Pulmonary manifestations in systemic lupus erythematosus: Association with disease activity

    PubMed Central

    Alamoudi, Omer SB; Attar, Suzan M

    2015-01-01

    Background and objective Although systemic lupus erythematosus (SLE) is the most common connective tissue disease affecting the lung, few studies have assessed risk factors that predict pulmonary manifestations. The objectives of the present study were to determine the prevalence of lung manifestations in SLE patients from Western Saudi Arabia by analysing results from high-resolution computed tomography (HRCT) scans and to identify independent risk factors for lung involvement. Methods This was a 10-year retrospective study involving 184 SLE patients. We examined all HRCT lung abnormalities and determined whether findings were associated with the presence of lupus nephritis (LN), SLE disease activity (as defined by SLE Disease Activity Index 2000 item scores ?4 for any and all items) or levels of complement and anti-double-stranded DNA (anti-dsDNA). Results We identified 61 patients (33%) with pulmonary involvement, and 52 (85%) of these subjects showed HRCT abnormalities. The most common HRCT findings were pleural effusion, consolidation and atelectasis (58%, 42% and 42%, respectively). There was a significant association between abnormal HRCT results and hypocomplementemia, high levels of anti-dsDNA and disease activity (P < 0.05), particularly with regard to pleuropericardial effusion and consolidation. Pulmonary abnormalities were significantly higher within the first five years after SLE diagnosis (P < 0.001). However, neither disease duration nor LN was associated with increased risk. Conclusions Lung manifestations were frequent in SLE patients from Saudi Arabia, with pleural effusion, consolidation and atelectasis being the most common. Low complement levels, high anti-dsDNA levels and disease activity were significantly associated with abnormal HRCT findings (all P < 0.001). PMID:25639532

  2. Various Manifestations of Hyperthyroidism in an Ambulatory Clinic: Case Studies

    PubMed Central

    Tripp, Warren; Rao, Vijaya; Creary, Ludlow B.

    1987-01-01

    This study reviews five cases of women with hyperthyroidism, three black women and two Hispanic women. Initially, two patients presented with voice changes, weight loss, and increased appetite. Only two patients presented with classical symptoms of hyperthyroidism. Examination showed all patients had diffusely enlarged thyroids and exaggerated reflexes. Two patients showed Graves' opthalmopathy. These cases document the variety of presentations of hyperthyroidism. Hence, a high index of suspicion must exist for this disease, even in the absence of a number of the classical manifestations of hyperthyroidism. When patients present to primary care centers with a constellation of symptoms, an examination of the thyroid gland is essential. PMID:3694696

  3. Manifestations bucco-dentaires de la sclérodermie systémique

    PubMed Central

    Salem, Bouomrani; Rim, Bel Hadj Ali; Sihem, Ben Khoud; Maher, Béji

    2013-01-01

    Nous rapportons l'observation d'une jeune femme de 26 ans ayant une sclérodermie systémique diffuse présentant une atteinte bucco-dentaire complexe: microstomie, hyperplasie gingivale, parodontite, dépôts tartriques, caries multiples et chevauchement dentaire antéro-inférieur. Nous discuterons à travers cette observation les manifestations bucco-dentaires de cette connectivite qui sont loin d’être rares mais souvent négligées par les cliniciens malgré leur retentissement fonctionnel majeur. Il convient de surveiller régulièrement l’état bucco-dentaire chez tout patient sclérodermique afin de diagnostiquer précocement ces atteintes. Diagnostiquées à un stade tardif les complications bucco-dentaires de la sclérodermie seront très difficiles à traiter. PMID:24778751

  4. Pycnodysostosis: A bone dysplasia with unusual oral manifestation

    PubMed Central

    Balaji, M S Anandi; Sundaram, R Kanmani Shanmuga; Karthik, Poornima; Asokan, Kannan

    2014-01-01

    Pycnodysostosis, a sclerosing bone dysplasia, is a rare autosomal recessive disorder with an estimated prevalence rate of one in one million. Patients with pycnodyostosis usually have normal intelligence, sexual development and life span. This condition is characterized by increased bone density and fragility along with oral manifestations like malposition teeth, hypoplastic maxilla, receded chin and delayed eruption of permanent teeth with discharging sinuses in the jaws because of poor blood supply. This is one such rare case report of a 47-year-old patient presenting with a complaint of fractured jaw and reviewing the clinical and radiographic characteristics of pycnodysostosis. PMID:25565757

  5. Peripheral nervous system manifestations in systemic autoimmune diseases.

    PubMed

    Cojocaru, Inimioara Mihaela; Cojocaru, Manole; Silosi, Isabela; Vrabie, Camelia Doina

    2014-09-01

    The peripheral nervous system refers to parts of the nervous system outside the brain and spinal cord. Systemic autoimmune diseases can affect both the central and peripheral nervous systems in a myriad of ways and through a heterogeneous number of mechanisms leading to many different clinical manifestations. As a result, neurological complications of these disorders can result in significant morbidity and mortality. The most common complication of peripheral nervous system (PNS) involvement is peripheral neuropathy, with symptoms of numbness, sensory paresthesias, weakness, or gait imbalance. The neuropathy may be multifocal and asymmetric or, less frequently, distal and symmetric. PMID:25705295

  6. Peripheral Nervous System Manifestations in Systemic Autoimmune Diseases

    PubMed Central

    COJOCARU, Inimioara Mihaela; COJOCARU, Manole; SILOSI, Isabela; VRABIE, Camelia Doina

    2014-01-01

    The peripheral nervous system refers to parts of the nervous system outside the brain and spinal cord. Systemic autoimmune diseases can affect both the central and peripheral nervous systems in a myriad of ways and through a heterogeneous number of mechanisms leading to many different clinical manifestations. As a result, neurological complications of these disorders can result in significant morbidity and mortality. The most common complication of peripheral nervous system (PNS) involvement is peripheral neuropathy, with symptoms of numbness, sensory paresthesias, weakness, or gait imbalance. The neuropathy may be multifocal and asymmetric or, less frequently, distal and symmetric. PMID:25705295

  7. A Manifestly Local Theory of Vacuum Energy Sequestering

    E-print Network

    Kaloper, Nemanja; Stefanyszyn, David; Zahariade, George

    2015-01-01

    We present a manifestly local, diffeomorphism invariant and locally Poincare invariant formulation of vacuum energy sequestering. In this theory, quantum vacuum energy generated by matter loops is cancelled by auxiliary fields. The auxiliary fields decouple from gravity almost completely. Their only residual effect is an {\\it a priori} arbitrary, finite contribution to the curvature of the background geometry, which is radiatively stable. Its value is to be determined by a measurement, like the finite part of any radiatively stable UV-sensitive quantity in quantum field theory

  8. Osteoskeletal manifestations of scurvy: MRI and ultrasound findings.

    PubMed

    Polat, Ahmet Veysel; Bekci, Tumay; Say, Ferhat; Bolukbas, Emrah; Selcuk, Mustafa Bekir

    2015-08-01

    Scurvy has become very rare in the modern world. The incidence of scurvy in the pediatric population is extremely low. In the pediatric population, musculoskeletal manifestations are more common and multiple subperiosteal hematomas are an important indicator for the diagnosis of scurvy. Although magnetic resonance imaging findings of scurvy are well described in the literature, to our knowledge, ultrasound findings have not yet been described. In this article, we report a case of scurvy with associated magnetic resonance imaging and ultrasound findings. PMID:25597047

  9. Central nervous system manifestations of Angiostrongylus cantonensis infection.

    PubMed

    Martins, Yuri C; Tanowitz, Herbert B; Kazacos, Kevin R

    2015-01-01

    Over 20 species of Angiostrongylus have been described from around the world, but only Angiostrongylus cantonensis has been confirmed to cause central nervous system disease in humans. A neurotropic parasite that matures in the pulmonary arteries of rats, A. cantonensis is the most common cause of eosinophilic meningitis in southern Asia and the Pacific and Caribbean islands. The parasite can also cause encephalitis/encephalomyelitis and rarely ocular angiostrongyliasis. The present paper reviews the life cycle, epidemiology, pathogenesis, clinical features, diagnosis, treatment, prevention and prognosis of A. cantonesis infection. Emphasis is given on the spectrum of central nervous system manifestations and disease pathogenesis. PMID:25312338

  10. Manifestions of Berry's topological phase for the photon

    NASA Astrophysics Data System (ADS)

    Chiao, R. Y.; Wu, Y.-S.

    1986-08-01

    Recently, Berry (1984) recognized in quantum mechanics a topological phase factor arising from the adiabatic transport of a system around a closed circuit, which is essentially the Aharonov-Bohm effect in parameter space. Here manifestations of this phase factor are considered for a photon in a state of adiabatically invariant helicity. An interferometer is suggested to see this phase. Also, an effective optical activity for a helical optical fiber is predicted. These effects emerge on a classical level as topological features of Maxwell's theory.

  11. A Manifestly Local Theory of Vacuum Energy Sequestering

    E-print Network

    Nemanja Kaloper; Antonio Padilla; David Stefanyszyn; George Zahariade

    2015-05-06

    We present a manifestly local, diffeomorphism invariant and locally Poincare invariant formulation of vacuum energy sequestering. In this theory, quantum vacuum energy generated by matter loops is cancelled by auxiliary fields. The auxiliary fields decouple from gravity almost completely. Their only residual effect is an {\\it a priori} arbitrary, finite contribution to the curvature of the background geometry, which is radiatively stable. Its value is to be determined by a measurement, like the finite part of any radiatively stable UV-sensitive quantity in quantum field theory

  12. Manifest causality in quantum field theory with sources and detectors

    E-print Network

    Robert Dickinson; Jeff Forshaw; Peter Millington; Brian Cox

    2014-06-16

    We introduce a way to compute scattering amplitudes in quantum field theory including the effects of particle production and detection. Our amplitudes are manifestly causal, by which we mean that the source and detector are always linked by a connected chain of retarded propagators. We show how these amplitudes can be derived from a path integral, using the Schwinger-Keldysh "in-in" formalism. Focussing on phi-cubed theory, we confirm that our approach agrees with the standard S-matrix approach in the case of positive energy plane-wave scattering.

  13. Automated Detection of Anomalous Shipping Manifests to Identify Illicit Trade

    SciTech Connect

    Sanfilippo, Antonio P.; Chikkagoudar, Satish

    2013-11-12

    We describe an approach to analyzing trade data which uses clustering to detect similarities across shipping manifest records, classification to evaluate clustering results and categorize new unseen shipping data records, and visual analytics to provide to support situation awareness in dynamic decision making to monitor and warn against the movement of radiological threat materials through search, analysis and forecasting capabilities. The evaluation of clustering results through classification and systematic inspection of the clusters show the clusters have strong semantic cohesion and offer novel ways to detect transactions related to nuclear smuggling.

  14. Physostigmine for cardiac and neurologic manifestations of phenothiazine poisoning.

    PubMed

    Weisdorf, D; Kramer, J; Goldbarg, A; Klawans, H L

    1978-12-01

    Psychotropic drugs such as the phenothiazine neuroleptics and tricyclic antidepressants are known to cause electrocardiographic abnormalities as well as a central anticholinergic syndrome. Physostigmine is known to reverse the central muscarinic anticholinergic manifestations by inhibition of the enzyme cholinesterase. An unusual case of trifluoperazine overdose, in which the patient presented with cardiac arrhythmias and a central anticholinergic syndrome, is presented. Treatment with physostigmine reversed the central anticholinergic syndrome as well as the electrocardiographic abnormalities. Effects of phenothiazines on altering cardiac status are also discussed. PMID:30564

  15. Manifestations of food allergy in infants and children.

    PubMed

    Story, Rachel E

    2008-08-01

    Most adverse reactions to foods are the result of intolerances rather than food allergy. It is important to identify children with potential food allergy as life-threatening, and life-ending reactions can occur. A clear understanding of the manifestations of food allergy caused by both IgE- and non-IgE-related mechanisms will help the practitioner to identify children who likely have food allergy so that additional diagnostic evaluation can be performed and appropriate avoidance can be instituted. PMID:18751570

  16. 66 FR 28240 - Hazardous Waste Management System; Modification of the Hazardous Waste Manifest System

    Federal Register 2010, 2011, 2012, 2013, 2014

    2001-05-22

    ...regulations would permit that company to store image files of signed manifests received at its...completed paper manifests and then store the image files of these manifests on optical disks...supported by the Agency's findings that the image files would bear the required...

  17. Risk prediction and risk reduction in patients with manifest arterial disease

    Microsoft Academic Search

    B. M. B. Goessens

    2006-01-01

    Risicovoorspelling en risicoverlaging bij patienten met manifest vaatlijden Engelstalig abstract The number of patients with clinical manifest arterial disease is increasing because of the aging of the population. Patients with manifest arterial disease have an increased risk of a new vascular event in the same or different arterial bed. Medical treatment of vascular risk factors (hypertension, hyperlipidemia, diabetes mellitus) and

  18. Manifest Security for Distributed Information Karl Crary Robert Harper Frank Pfenning

    E-print Network

    Pierce, Benjamin C.

    Manifest Security for Distributed Information Karl Crary Robert Harper Frank Pfenning Carnegie whose type system ensures compliance with security policies through the use of proofs in a formal logic that are manifestly secure. Manifest security means that the trust relationships, access control and information flow

  19. Myopericarditis and severe myocardial dysfunction as the initial manifestation of systemic lupus erythematosus

    PubMed Central

    Peñataro, Joaquín S; De Mingo, Ana; Del Río, Ana; Martínez, José A; Heras, Magda

    2012-01-01

    Pericarditis is the most frequent cardiac manifestation of systemic lupus erythematosus (SLE). However, a large pericardial effusion as the initial manifestation of the disease is infrequent, especially when it is associated with myocardial damage. We describe an unusual case of a young female with pleuropericarditis and severe myocardial dysfunction as the initial manifestation of SLE. PMID:24062915

  20. Neurological manifestations in Baggio-Yoshinari Syndrome (Brazilian Lyme disease-like syndrome)

    Microsoft Academic Search

    Samuel Katsuyuki Shinjo; Giancarla Gauditano; Paulo Euripedes Marchiori; Virgínia Lúcia Nazário Bonoldi; Izaias Pereira da Costa; Elenice Mantovani; Natalino Hajime Yoshinari

    Introduction: Lyme disease (LD) is a tick-borne disease, caused by Borrelia burgdorferi sensu lato spirochetes, trans- mitted by Ixodes ricinus complex ticks, which leads to multiple systemic clinical manifestations. In Brazil, a different syndrome is described that mimics LD symptoms, but that also manifests high frequencies of recurrent episodes and immune-allergic manifestations. It is transmitted by the Amblyomma cajennense tick

  1. Skin and Systemic Manifestations of Jellyfish Stings in Iraqi Fishermen

    PubMed Central

    Al-Rubiay, KK; Al-Musaoi, HA; Alrubaiy, L; Al-Freje, MG

    2009-01-01

    Background: Jellyfish stings are common worldwide with an estimated 150 million cases annually, and their stings cause a wide range of clinical manifestations from skin inflammation to cardiovascular and respiratory collapse. No studies on jellyfish stings have been carried out in Basra, Iraq. Objectives: To describe the immediate and delayed skin reactions to White Jellyfish (Rhizostoma sp.) stings and the types of local treatment used by fishermen. Methods and Materials: 150 fishermen were enrolled at three Marine stations in Basra, Iraq. Demographic data, types of skin reactions, systemic manifestations and kinds of treatments were collected. Results: Overall, 79% of fishermen in all three Marine stations gave a history of having been stung. The common sites of sings were the hands and arms followed by the legs. Most fishermen claimed that stings led to skin reactions within 5 minutes. The presenting complaints were itching, burning sensation, and erythematic wheals. A few days after the sting, new groups of painless and itchy erythematous monomorphic papular rashes developed at the site of the sting in 62% of cases as a delayed type of skin reaction that resolved spontaneously. The local remedies commonly used by the fishermen were seawater, tap water and ice. A few fishermen considered stings as insignificant and did not think there was a need to seek medical help. Conclusions: We conclude that jellyfish causes many stings among fishermen in the Basra region. Their stings lead to immediate and delayed skin reactions. Self-treatment by topical remedies is common. PMID:21483513

  2. Rare Bone Diseases and Their Dental, Oral, and Craniofacial Manifestations

    PubMed Central

    Foster, B.L.; Ramnitz, M.S.; Gafni, R.I.; Burke, A.B.; Boyce, A.M.; Lee, J.S.; Wright, J.T.; Akintoye, S.O.; Somerman, M.J.; Collins, M.T.

    2014-01-01

    Hereditary diseases affecting the skeleton are heterogeneous in etiology and severity. Though many of these conditions are individually rare, the total number of people affected is great. These disorders often include dental-oral-craniofacial (DOC) manifestations, but the combination of the rarity and lack of in-depth reporting often limit our understanding and ability to diagnose and treat affected individuals. In this review, we focus on dental, oral, and craniofacial manifestations of rare bone diseases. Discussed are defects in 4 key physiologic processes in bone/tooth formation that serve as models for the understanding of other diseases in the skeleton and DOC complex: progenitor cell differentiation (fibrous dysplasia), extracellular matrix production (osteogenesis imperfecta), mineralization (familial tumoral calcinosis/hyperostosis hyperphosphatemia syndrome, hypophosphatemic rickets, and hypophosphatasia), and bone resorption (Gorham-Stout disease). For each condition, we highlight causative mutations (when known), etiopathology in the skeleton and DOC complex, and treatments. By understanding how these 4 foci are subverted to cause disease, we aim to improve the identification of genetic, molecular, and/or biologic causes, diagnoses, and treatment of these and other rare bone conditions that may share underlying mechanisms of disease. PMID:24700690

  3. Extra-Intestinal Manifestations of Familial Adenomatous Polyposis

    PubMed Central

    Groen, Emma J.; Roos, Annemieke; Muntinghe, Friso L.; Enting, Roelien H.; de Vries, Jakob; Kleibeuker, Jan H.; Witjes, Max J. H.; Links, Thera P.

    2008-01-01

    Familial adenomatous polyposis (FAP) is an autosomal dominantly inherited disorder, which results from a germ line mutation in the APC (adenomatous polyposis coli) gene. FAP is characterized by the formation of hundreds to thousands of colorectal adenomatous polyps. Although the development of colorectal cancer stands out as the most prevalent complication, FAP is a multisystem disorder of growth. This means, it is comparable to other diseases such as the MEN syndromes, Von Hippel-Lindau disease and neurofibromatosis. However, the incidence of many of its clinical features is much lower. Therefore, a specialized multidisciplinary approach to optimize health care—common for other disorders—is not usually taken for FAP patients. Thus, clinicians that care for and counsel members of high-risk families should have familiarity with all the extra-intestinal manifestations of this syndrome. FAP-related complications, for which medical attention is essential, are not rare and their estimated lifetime risk presumably exceeds 30%. Affected individuals can develop thyroid and pancreatic cancer, hepatoblastomas, CNS tumors (especially medulloblastomas), and various benign tumors such as adrenal adenomas, osteomas, desmoid tumors and dental abnormalities. Due to improved longevity, as a result of better prevention of colorectal cancer, the risk of these clinical problems will further increase. We present a clinical overview of extra-intestinal manifestations, including management and treatment options for the FAP syndrome. Furthermore, we provide recommendations for surveillance of FAP complications based on available literature. PMID:18612695

  4. Requirements for a Manifestly Covariant Quantum Field Theory

    NASA Astrophysics Data System (ADS)

    Walker, Stuart

    2015-04-01

    Quantum field theory has shown much success in defining manifestly covariant fields in Minkowski space-time. The general procedure for construction of QFT in terms of annihilation and creation operators acting through a simplectic form is outlined. It is demonstrated that this methodology fails to define a general covariant vector field in a space-time spanning a Riemannian manifold with Lorentz metric (M,guv) ; therefore, no appropriate outer product can be defined to produce a general 2nd rank tensor (i.e. stress-energy tensor). The QFT defined in flat space-time is expanded to include curvilinear coordinates. The manifestly covariant QFT in curved space-time is used to redefine the equations of motion in terms of the classical field theory. The resulting theory has the benefit of requiring an S-matrix defining unitarily equivalent quantum theories while providing an exact formulation for the quantized equations of motion in a gravitational field. The physical implications of this construction are discussed including the anisomorphic nature of the simplectic vector space in curvilinear coordinates. An example involving plane wave expansion is discussed.

  5. Clinical manifestations of syphilitic chorioretinitis: a retrospective study

    PubMed Central

    Yang, Bo; Xiao, Jun; Li, XiaoMing; Luo, Lifu; Tong, Bainan; Su, Guanfang

    2015-01-01

    Background: Syphilitic chorioretinitis can produce severe vision loss. However, the clinical manifestations of syphilitic chorioretinitis are still unclear, particularly during different stages. Herein, we will present our diagnostic technique for syphilitic chorioretinitis. Methods: This retrospective study recruited 109 cases; we performed a clinical evaluation including case history, serology analysis, fundus photography, fluorescein fundus angiography with or without indocyanine green angiography, auto-fluorescence, and optical coherence tomography. Results: 109 were diagnosed with acute syphilitic posterior placoid chorioretinitis by fundus photograph that revealed filthy, yellowish-white lesions. For autofluorescence, during early-stage syphilitic chorioretinitis, hyperfluorescence could be observed. During the convalescence stage, the fluorescence became hypofluorescence or disappeared. Fluorescein fundus angiography indicated early-stage transmitted fluorescence or hypofluorescence. During the venous stage, the lesion area had fluorescent leakage, mostly accompanied by retinal vasculitis. During the late stage, speckle staining was observed with optic disc fluorescence. Hypofluorescence or undistinguishable fluorescence was seen at an early stage with indocyanine green angiography. At an advanced stage, the lesion had obvious hypofluorescence. Optical coherence tomography indicated various inner segment/outer segment damage, accompanied by retinal pigment epithelium impairment. The inner segment/outer segment alteration could be lessened with treatment. Conclusions: The clinical manifestations of syphilitic chorioretinitis include impaired vision, shadow blocking, or photopsia of one or both eyes. Fundus photography, fluorescein fundus angiography with or without indocyanine green angiography, autofluorescence, and optical coherence tomography could be useful accessory examinations. Autofluorescence and optical coherence tomography could be the main examinations for monitoring disease progression.

  6. Quantum mechanics and the manifestation of the world

    E-print Network

    Ulrich Mohrhoff

    2014-08-23

    Quantum theory's irreducible empirical core is a probability calculus. While it presupposes the events to which (and on the basis of which) it serves to assign probabilities, and therefore cannot account for their occurrence, it has to be consistent with it. It must make it possible to identify a system of observables that have measurement-independent values. What makes this possible is the incompleteness of the spatiotemporal differentiation of the physical world. This is shown by applying a novel interpretive principle to interfering alternatives involving distinctions between regions of space. Applying the same interpretive principle to alternatives involving distinctions between things makes it safe to claim that the macroworld comes into being through a progressive differentiation of a single, intrinsically undifferentiated entity. By entering into reflexive spatial relations, this entity gives rise to (i) what looks like a multiplicity of relata if the reflexive quality of the relations is not taken into account, and (ii) what looks like a substantial expanse if the spatial quality of the relations is reified. The necessary distinction between two domains (classical and quantum, or macro and micro) and their mutual dependence is best understood as a distinction between the manifested world and its manifestation.

  7. Clinical Manifestations and Distribution of Cutaneous Leishmaniasis in Pakistan

    PubMed Central

    Afghan, Abaseen Khan; Kassi, Masoom; Kasi, Pashtoon Murtaza; Ayub, Adil; Kakar, Niamatullah; Marri, Shah Muhammad

    2011-01-01

    Cutaneous leishmaniasis (CL) is a rising epidemic in Pakistan. It is a major public health problem in the country especially alongside regions bordering the neighboring Afghanistan and cities that have had the maximum influx of refugees. The purpose of our paper is to highlight the diverse clinical manifestations of the disease seen along with the geographic areas affected, where the hosts are particularly susceptible. This would also be helpful in presenting the broad spectrum of the disease for training of health care workers and help in surveillance of CL in the region. The increased clinical diversity and the spectrum of phenotypic manifestations noted underscore the fact that the diagnosis of CL should be not only considered when dealing with common skin lesions, but also highly suspected by dermatologists and even primary care physicians even when encountering uncommon pathologies. Hence, we would strongly advocate that since most of these patients present to local health care centers and hospitals, primary care practitioners and even lady health workers (LHWs) should be trained in identification of at least the common presentations of CL. PMID:22174721

  8. Clinical manifestations and distribution of cutaneous leishmaniasis in pakistan.

    PubMed

    Afghan, Abaseen Khan; Kassi, Masoom; Kasi, Pashtoon Murtaza; Ayub, Adil; Kakar, Niamatullah; Marri, Shah Muhammad

    2011-01-01

    Cutaneous leishmaniasis (CL) is a rising epidemic in Pakistan. It is a major public health problem in the country especially alongside regions bordering the neighboring Afghanistan and cities that have had the maximum influx of refugees. The purpose of our paper is to highlight the diverse clinical manifestations of the disease seen along with the geographic areas affected, where the hosts are particularly susceptible. This would also be helpful in presenting the broad spectrum of the disease for training of health care workers and help in surveillance of CL in the region. The increased clinical diversity and the spectrum of phenotypic manifestations noted underscore the fact that the diagnosis of CL should be not only considered when dealing with common skin lesions, but also highly suspected by dermatologists and even primary care physicians even when encountering uncommon pathologies. Hence, we would strongly advocate that since most of these patients present to local health care centers and hospitals, primary care practitioners and even lady health workers (LHWs) should be trained in identification of at least the common presentations of CL. PMID:22174721

  9. Initial Cutaneous Manifestations of Hutchinson-Gilford Progeria Syndrome

    PubMed Central

    Rork, Jillian F.; Huang, Jennifer T.; Gordon, Leslie B.; Kleinman, Monica; Kieran, Mark W.; Liang, Marilyn G.

    2014-01-01

    Hutchinson-Gilford progeria syndrome (HGPS) is a rare, uniformly fatal premature aging disease with distinct dermatologic features. We sought to identify and describe the initial skin and hair findings as potential diagnostic signs of the disease. We performed a chart review of the structured initial intake histories of 39 individuals with HGPS enrolled in clinical trials from 2007 to 2010 at Boston Children’s Hospital, limited to cutaneous history from birth to 24 months. Medical photographs were provided through the clinical trials and The Progeria Research Foundation Medical and Research Database at Brown University Center for Gerontology and Healthcare Research. All 39 patients reported skin and hair abnormalities within the first 24 months of life. Pathologies included sclerodermoid change, prominent superficial veins, dyspigmentation, and alopecia. The mean age of presentation for each finding was less than 12 months. The most frequently reported skin feature was sclerodermoid change, which commonly involved the abdomen and bilateral lower extremities. Prominent superficial vasculature manifested as circumoral cyanosis and pronounced veins on the scalp and body. Hypo- and hyperpigmentation were observed over areas of sclerodermoid change. Scalp alopecia progressed in a distinct pattern, with preservation of the hair over the midscalp and vertex areas for the longest period of time. HGPS has distinct cutaneous manifestations during the first 2 years of life that may be the first signs of disease. Awareness of these findings could expedite diagnosis. PMID:24456199

  10. Environmental influences on skin aging and ethnic-specific manifestations

    PubMed Central

    Vierkötter, Andrea; Krutmann, Jean

    2012-01-01

    Skin aging does not only occur by passing time alone but also by the exposure to different environmental factors. The skin aging process, which is induced by environmental factors, is named premature or extrinsic skin aging process and can be distinguished from the chronologically (intrinsic) skin aging process by characteristic skin aging signs. Well known environmental factors leading to extrinsic skin aging are sun exposure and smoking. Recently, an epidemiological study could further discover an association between air pollution and skin aging. First of all the skin aging inducing effect of sun exposure was discovered and an own term (photoaging) was given to this special field of extrinsic skin aging. Mechanistic studies have further increased our knowledge about the molecular pathways by which environmental factors contribute to extrinsic skin aging. In this regard, profound knowledge how sun exposure leads to extrinsic skin aging were gained in the last years, and additionally there are also indications how smoking and air pollution might contribute to this process. Moreover it was realized that extrinsic skin aging manifests differently between different populations. Thus, in this review we summarize the influence of the different environmental factors: sun exposure, smoking and air pollution on skin aging and further present ethnic-specific manifestations of extrinsic skin aging. PMID:23467702

  11. Social Comparison Manifests in Event-related Potentials.

    PubMed

    Luo, Yi; Feng, Chunliang; Wu, Tingting; Broster, Lucas S; Cai, Huajian; Gu, Ruolei; Luo, Yue-Jia

    2015-01-01

    Social comparison, a widespread phenomenon in human society, has been found to affect outcome evaluation. The need to belong to a social group may result in distinct neural responses to diverse social comparison outcomes. To extend previous studies by examining how social comparison with hierarchical characteristics is temporally processed, electroencephalography responses were recorded in the current study. Participants played a lottery game with two pseudo-players simultaneously and received both their own and the other two players' outcomes. Results of three event-related potential components, including the P2, the feedback-related negativity (FRN), and the late positive component (LPC), indicate that social comparison manifests in three stages. First, outcomes indicating a different performance from others elicited a larger P2 than evenness. Second, the FRN showed hierarchical sensitivity to social comparison outcomes. This effect manifested asymmetrically. Finally, large difference between the participant's outcome and the other two players' evoked a larger LPC than the medium difference and the even condition. We suggest that during social comparison, people detect if there is any difference between self and others, and then evaluate the information of this difference hierarchically, and finally interpret the situations in which oneself deviates from the group as most motivationally salient. PMID:26183734

  12. Social Comparison Manifests in Event-related Potentials

    PubMed Central

    Luo, Yi; Feng, Chunliang; Wu, Tingting; Broster, Lucas S.; Cai, Huajian; Gu, Ruolei; Luo, Yue-jia

    2015-01-01

    Social comparison, a widespread phenomenon in human society, has been found to affect outcome evaluation. The need to belong to a social group may result in distinct neural responses to diverse social comparison outcomes. To extend previous studies by examining how social comparison with hierarchical characteristics is temporally processed, electroencephalography responses were recorded in the current study. Participants played a lottery game with two pseudo-players simultaneously and received both their own and the other two players’ outcomes. Results of three event-related potential components, including the P2, the feedback-related negativity (FRN), and the late positive component (LPC), indicate that social comparison manifests in three stages. First, outcomes indicating a different performance from others elicited a larger P2 than evenness. Second, the FRN showed hierarchical sensitivity to social comparison outcomes. This effect manifested asymmetrically. Finally, large difference between the participant’s outcome and the other two players’ evoked a larger LPC than the medium difference and the even condition. We suggest that during social comparison, people detect if there is any difference between self and others, and then evaluate the information of this difference hierarchically, and finally interpret the situations in which oneself deviates from the group as most motivationally salient. PMID:26183734

  13. Case report: bipolar disorder as the first manifestation of CADASIL

    PubMed Central

    2014-01-01

    Background Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited cerebrovascular disease, clinically characterized by variable manifestations of migraine, recurrent transient ischemic attack or lacunar strokes, cognitive decline, and mood disturbances. However, manic episodes have rarely been documented as an initial symptom of CADASIL and bipolar disorder presenting as the first manifestation in CADASIL has not been reported previously from evaluations by psychiatrists or psychological testing by psychologists. Case presentation A 53 year old woman developed symptoms of mania in her 50s leading to a personality change involving a continuously labile mood and irritability over a number of years. Neuropsychological testing revealed an intact memory, but impairment in attention and executive function. In the Rorschach test, she showed a high level of cognitive rigidity. Magnetic resonance imaging findings were very consistent with a diagnosis of CADASIL, which was confirmed by genetic testing for NOTCH3 mutations. Atypical antipsychotics proved to be helpful in treating her manic symptoms and for behavior control. Conclusion We present a novel case of CADASIL that first presented as bipolar disorder. We contend that when patients show a late onset personality change or chronically irritable mood that deteriorates over many years, an organic cause such as CADASIL must be considered. Further studies are needed to better understand the exact impacts of cerebral tissue lesions and psychiatric symptoms in CADASIL patients. PMID:24929957

  14. Orodental manifestations in ectodermal dysplasia-a review.

    PubMed

    Bergendal, Birgitta

    2014-10-01

    Oral signs and symptoms are present in most ectodermal dysplasias (EDs). The aim of this article is to summarize some of the literature on current knowledge of oral manifestations and orofacial function in EDs. The review will focus on the most common forms where dental manifestations can be crucial for a differential diagnosis of ED among individuals with hypodontia and oligodontia, and preferably where the investigations included persons who had a genetically verified diagnosis. Disturbances in tooth development are common and can appear as tooth agenesis, variations in size and shape of teeth, defects in the mineralized tissues, and problems in tooth eruption. Abnormalities in number, size, and shape of teeth, and reduced salivary secretion, present in isolated oligodontia as well as in hypohidrotic ED and incontinentia pigmenti. In some more rare EDs these symptoms appear in combination with clefts of lip and/or palate in some affected individuals. Leukokeratosis in the oral mucosa presents in 70% of genetically confirmed cases of pachyonychia congenita. Also, orofacial function is often affected in ED, due to malformations, an incomplete dentition, and low salivary secretion which can compromise chewing, swallowing, and speech. In conclusion, there is a clinical overlap in oral signs and symptoms between isolated oligodontia and the most common EDs. Studies with genetically confirmed diagnoses and larger cohorts, as well as multicenter collaboration and the establishing of international registries, would create a basis for refined diagnostics, where oral examinations should be an integrated part of clinical assessment. PMID:24719393

  15. Management of otolaryngological manifestations in mucopolysaccharidoses: our experience.

    PubMed

    Mesolella, M; Cimmino, M; Cantone, E; Marino, A; Cozzolino, M; Della Casa, R; Parenti, G; Iengo, M

    2013-08-01

    Mucopolysaccharidoses (MPSs) are lysosomal storage disorders caused by deficiency of enzymes involved in the degradation of glycosaminoglycans (GAGs). These disorders are associated with the accumulation of GAGs in tissues with organomegaly, mental retardation and short stature. Otologic and upper respiratory tract pathologies are among the earliest clinical manifestations. We analyzed 20 patients (13 male and 7 female, median age at the beginning of the observation 6 years) with MPS (35% type I, 30% type II, 20% type III, 5% type IV, 10% type VI), focusing on their otorhinolaryngologic problems and the impact of surgery on quality of life. We found ear, nose and throat manifestations in all types of MPS; in particular, recurrent otitis media was present in 30% of cases, hearing loss in 75% (mixed in 43.33%, conductive in 43.33%, sensorineural in 13.33%), adenotonsillar hypertrophy in 75%, frequent infections of the upper airway in 75% and obstructive sleep apnoea syndrome in 45% of cases. Fifty percent of patients required surgical therapy (adenotonsillectomy, adenoidectomy with insertion of middle ear ventilation tubes, tonsillectomy, tracheotomy and exeresis of vocal cord polyps). In our experience the ENT surgery reduced the frequency and severity of ear infections and relieved symptoms related to upper airway obstruction, thereby improving the quality of life in affected patients. PMID:24043915

  16. Respiratory manifestations in patients with inherited metabolic diseases.

    PubMed

    Santamaria, Francesca; Montella, Silvia; Mirra, Virginia; De Stefano, Sara; Andria, Generoso; Parenti, Giancarlo

    2013-12-01

    Growing evidence indicates that inherited metabolic diseases are increasingly being recognised. Life expectancy for many patients is progressively improving because new therapeutic strategies are available. Because most inherited metabolic diseases are systemic disorders, virtually all organs may be involved. Respiratory disease complicates the management of several inherited metabolic diseases, either at presentation or as late-onset features. This review will describe the most exemplary respiratory manifestations of inherited metabolic diseases in childhood and adulthood. Since airways disease worsens the morbidity of many inherited metabolic disorders, leading to increased hospitalisations, mortality and overall healthcare costs, respiratory manifestations of inherited metabolic diseases need to be carefully recognised and treated. All patients with inherited metabolic disease and suspected airway disease should undergo a detailed diagnostic work-up. Current treatments for several inherited metabolic diseases (including enzyme replacement therapy, substrate reduction, bone marrow transplantation, or even more innovative strategies such as pharmacological chaperone or gene therapies) may provide significant benefits for associated respiratory disease. The integration of several specialists dedicated to airway disease management in a multidisciplinary team is essential to provide the most appropriate care to children and adults with inherited metabolic disease. PMID:24293461

  17. Initial cutaneous manifestations of Hutchinson-Gilford progeria syndrome.

    PubMed

    Rork, Jillian F; Huang, Jennifer T; Gordon, Leslie B; Kleinman, Monica; Kieran, Mark W; Liang, Marilyn G

    2014-01-01

    Hutchinson-Gilford progeria syndrome (HGPS) is a rare, uniformly fatal, premature aging disease with distinct dermatologic features. We sought to identify and describe the initial skin and hair findings as potential diagnostic signs of the disease. We performed a chart review of the structured initial intake histories of 39 individuals with HGPS enrolled in clinical trials from 2007 to 2010 at Boston Children's Hospital, limited to cutaneous history from birth to 24 months. Medical photographs were provided through the clinical trials and the Progeria Research Foundation Medical and Research Database at Brown University Center for Gerontology and Healthcare Research. All 39 patients reported skin and hair abnormalities within the first 24 months of life. Pathologies included sclerodermoid change, prominent superficial veins, dyspigmentation, and alopecia. The mean age of presentation for each finding was <12 months. The most frequently reported skin feature was sclerodermoid change, which commonly involved the abdomen and bilateral lower extremities. Prominent superficial vasculature manifested as circumoral cyanosis and pronounced veins on the scalp and body. Hypo- and hyperpigmentation were observed over areas of sclerodermoid change. Scalp alopecia progressed in a distinct pattern, with preservation of the hair over the midscalp and vertex areas for the longest period of time. HGPS has distinct cutaneous manifestations during the first 2 years of life that may be the first signs of disease. Awareness of these findings could expedite diagnosis. PMID:24456199

  18. Urban legends series: oral manifestations of HIV infection.

    PubMed

    Patton, L L; Ramirez-Amador, V; Anaya-Saavedra, G; Nittayananta, W; Carrozzo, M; Ranganathan, K

    2013-09-01

    Human immunodeficiency virus-related oral lesions (HIV-OLs), such as oral candidiasis (OC) and oral hairy leukoplakia (OHL), have been recognized as indicators of immune suppression since the beginning of the global HIV epidemic. The diagnosis and management of HIV disease and spectrum of opportunistic infection has changed over the past 30 years as our understanding of the infection has evolved. We investigated the following controversial topics: (i) Are oral manifestations of HIV still relevant after the introduction of highly active antiretroviral therapy (HAART)? (ii) Can we nowadays still diagnose HIV infection through oral lesions? (iii) Is the actual classification of oral manifestations of HIV adequate or does it need to be reviewed and updated? (iv) Is there any novelty in the treatment of oral manifestations of HIV infection? Results from extensive literature review suggested the following: (i) While HAART has resulted in significant reductions in HIV-OLs, many are still seen in patients with HIV infection, with OC remaining the most common lesion. While the relationship between oral warts and the immune reconstitution inflammatory syndrome is less clear, the malignant potential of oral human papillomavirus infection is gaining increasing attention. (ii) Effective antiretroviral therapy has transformed HIV from a fatal illness to a chronic manageable condition and as a result expanded screening policies for HIV are being advocated both in developed and in developing countries. Affordable, reliable, and easy-to-use diagnostic techniques have been recently introduced likely restricting the importance of HIV-OLs in diagnosis. (iii) The 1993 EC-Clearinghouse classification of HIV-OLs is still globally used despite controversy on the relevance of periodontal diseases today. HIV-OL case definitions were updated in 2009 to facilitate the accuracy of HIV-OL diagnoses by non-dental healthcare workers in large-scale epidemiologic studies and clinical trials. (iv) Research over the last 6 years on novel modalities for the treatment of HIV-OLs has been reported for OC and OHL. PMID:23517181

  19. An atlas of the morphological manifestations of hidradenitis suppurativa.

    PubMed

    Scheinfeld, Noah

    2014-01-01

    This article is dermatological atlas of the morphologic presentations of Hidradenitis Suppurativa (HS). It includes: superficial abscesses (boils, furnucles, carbuncles), abscesses that are subcutaneous and suprafascial, pyogenic granulomas, cysts, painful erythematous papules and plaques, folliculitis, open ulcerations, chronic sinuses, fistulas, sinus tracts, scrotal and genital lyphedema, dermal contractures, keloids (some that are still pitted with follicular ostia), scarring, skin tags, fibrosis, anal fissures, fistulas (i.e. circinate, linear, arcuate), scarring folliculitis of the buttocks (from mild to cigarette-like scarring), condyloma like lesions in intertrigous areas, fishmouth scars, acne inversa, honey-comb scarring, cribiform scarring, tombstone comedones, and morphia-like plaques. HS can co-exist with other follicular diseases such as pilonidal cysts, dissecting cellulitis, acne conglobata, pyoderma gangrenosum, and acanthosis nigricans. In sum, the variety of presentations of HS as shown by these images supports the supposition that HS is a reaction pattern.HS is a follicular based diseased and its manifestations involve a multitude of follicular pathologies [1,2]. It is also known as acne inversa (AI) because of one manifestation that involves the formation of open comedones on areas besides the face. It is as yet unclear why HS is so protean in its manifestations. HS severity is assessed using the Hurley Staging System (Table 1). It also remains unclear why hidradentitis may remain limited to Hurley Stage 1, evolve to the more confluent (Hurley Stage 2), or progress even further to the fully confluent (Hurley Stage 3).In addition, HS can be associated with other follicular based diseases such as pilonidal cysts (PCs) of the sacrum and buttocks, dissecting cellulitis (DC), and acne conglobata (AC), which usually involves the face, chest, When HS occurs with PCs, DC, and/or AC it is referred to as the follicular occlusion triad or tetrad [2]. HS can more rarely be associated with pyoderma gagrenosum (PG) or Crohn disease (CD), other inflammatory diseases of the skin that are not follicular. The reason for this is unclear [2]. What AC, DC, HS, CD, and PG share is that they occur in bacterially rich environments. HS probably occurs with acanthosis nigricans because many HS patients are obese [2]. This concurrence seems under reported. PMID:24746309

  20. Star copolymers in porous environments: scaling and its manifestations

    E-print Network

    Viktoria Blavatska; Christian von Ferber; Yurij Holovatch

    2010-10-11

    We consider star polymers, consisting of two different polymer species, in a solvent subject to quenched correlated structural obstacles. We assume that the disorder is correlated with a power-law decay of the pair correlation function g(x)\\sim x^{-a}. Applying the field-theoretical renormalization group approach in d dimensions, we analyze different scenarios of scaling behavior working to first order of a double \\epsilon=4-d, \\delta=4-a expansion. We discuss the influence of the correlated disorder on the resulting scaling laws and possible manifestations such as diffusion controlled reactions in the vicinity of absorbing traps placed on polymers as well as the effective short-distance interaction between star copolymers.

  1. Thrombotic manifestations in SAPHO syndrome. Review of the literature.

    PubMed

    Carranco-Medina, Tatiana Elizabeth; Hidalgo-Calleja, Cristina; Calero-Paniagua, Ismael; Sánchez-González, María Dolores; Quesada-Moreno, Alba; Usategui-Martín, Ricardo; Pérez-Garrido, Laura; Gómez-Castro, Susana; Montilla-Morales, Carlos Alberto; Martínez-González, Olga; Del Pino-Montes, Javier

    2015-01-01

    SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome is a cluster of osteo-cutaneous manifestations that can lead to serious complications such as thrombosis of the subclavian vein or superior vena cava, mainly in patients with significant inflammatory involvement of the anterior-chest-wall. The objective of this study was to review the cases published in the medical literature related with the presence of thrombotic complications in patients diagnosed with SAPHO syndrome and to try to determine their possible pathogenic mechanism and risk factors. We analyzed 11 published reports of isolated clinical cases or case series, a total of 144 patients, which described a total of 15 cases of venous thrombosis. The clinical characteristics of these patients, evaluated to determine whether they meet the ASAS criteria for axial and peripheral spondyloarthritis, is analyzed the need for early diagnosis and treatment is highlighted. PMID:25441492

  2. Clinical manifestations of primary syphilis in homosexual men.

    PubMed

    Bjeki?, Milan; Markovi?, Milica; Sipeti?, Sandra

    2012-01-01

    At the beginning of a new millennium, syphilis incidence has been increasing worldwide, occurring primarily among men who have sex with men (MSM). The clinical features of primary syphilis among MSM is described, a case-note review of the primary syphilis (PS) patients who attended the Institute of Skin and Venereal Diseases. The diagnosis was assessed based upon the clinical features and positive syphilis serology tests. Among 25 patients with early syphilis referred during 2010, PS was diagnosed in a total of 13 cases. In all patients, unprotected oral sex was the only possible route of transmission, and two out of 13 patients had HIV co-infection. Overall, 77% of men presented with atypical penile manifestation. The VDRL test was positive with low titers. The numerous atypical clinical presentations of PS emphasize the importance of continuing education of non-experienced physicians, especially in countries with lower reported incidence of syphilis. PMID:22846130

  3. LUPUS PANNICULITIS AS AN INITIAL MANIFESTATION OF SYSTEMIC LUPUS ERYTHEMATOSUS

    PubMed Central

    Patel, Raksha M; Marfatia, Y S

    2010-01-01

    In May, 2003, a 28-year-old female presented with large non-healing ulcers on face, trunk and limbs covered with black hemorrhagic crust. There were no other systemic manifestations. Diagnosis of lupus panniculitis was considered on clinical and histopathological grounds. The lesions healed completely, with scarring, with systemic corticosteroid, hydroxychloroquine and topical 2% mupirocin. She came again in November, 2005, with malar rash, joint pain, scarring alopecia of the scalp and albuminuria. Her ANA, AntidsDNA came positive and diagnosed as having systemic lupus erythematosus (SLE). She responded well to systemic corticosteroid, antimalarial and topical antibacterial. The evolution of lupus panniculitis is slow and characterized by regression of the inflammatory lesions when treated with antimalarial drugs. The lupus panniculitis generally has a favorable course. PMID:20418989

  4. Leukemic vasculitis: an unusual manifestation of leukemia cutis.

    PubMed

    Seckin, Dilek; Senol, Asli; Gurbuz, Oya; Demirkesen, Cuyan

    2009-09-01

    Leukemia cutis is frequently observed as papules, nodules, and plaques, but unusual clinical manifestations rarely occur. We report a 64-year-old woman with acute myeloid leukemia M1 who presented with erythematous papules and vesiculobullous lesions limited to the arms, hands, and neck in addition to purpuric papules on the legs. Because of the symmetric distal involvement and vesiculobullous nature of the skin lesions, the differential diagnosis included erythema multiforme and vasculitis. However, a skin biopsy specimen revealed infiltration with a striking predominance of blast cells in the dermis. These cells were also observed within the walls of the venules along with fibrin. Leukemia cutis associated with vasculitis was diagnosed. A few blast cells can be observed in many reactive dermatoses in patients with leukemia. However, in this patient a predominance of blast cells in the dermis and infiltration of vessel walls by these cells favored leukemic vasculitis. PMID:19481293

  5. Manifest anxiety and fearfulness in children and adolescents.

    PubMed

    King, N J; Gullone, E; Ollendick, T H

    1992-03-01

    The relationship between self-reported fear and anxiety was examined in a large sample of normal Australian children and adolescents. Participants completed the Fear Survey Schedule for Children--Revised (Ollendick, 1983) and the Revised Children's Manifest Anxiety Scale (Reynolds & Richmond, 1978). Following an examination of the internal consistency of these instruments, correlational analyses were conducted on anxiety and fear scores. Fear scores were shown to be sensitive to anxiety, sex, and age groups. Furthermore, discriminant analysis showed that high-anxiety children indicated a greater fear of items related to failure and criticism than did low-anxiety children. Other issues, including the content overlap between the two scales used in the investigation, are discussed. PMID:1645159

  6. Orbital Metastasis: A Rare Manifestation of Scapular Bone Osteosarcoma

    PubMed Central

    Rajabi, Mohammad Taher; Jafari, Hajar; Hosseini, Seyedeh Simindokht; Tabatabaie, Seyed Ziaeddin; Rajabi, Mohammad Bagher; Amoli, Fahimeh Asadi

    2014-01-01

    Purpose: To report a case of orbital metastasis from scapular bone osteosarcoma. Case Report: A 55-year-old man who was a known case of scapular bone osteosarcoma, was referred to our clinic with ocular symptoms including acute painful decreased vision, proptosis, conjunctival injection, and chemosis. He had undergone surgical excision of the original tumor and received systemic chemotherapy 4 months before. Imaging studies and incisional biopsy were performed for the orbital lesion, the histopathological examination confirmed the diagnosis of metastatic osteosarcoma. The patient was referred to the oncologist for palliative chemotherapy and further intervention; however, he deceased 2 months later due to sepsis in the context of immunosuppression. Conclusion: Metastatic involvement of the orbit due to osteosarcoma is a rare condition manifesting with orbital mass, pain, diplopia and ocular motility disturbance. Although there is no effective treatment, the combination of modalities such as chemotherapy, radiotherapy, and surgery may delay progression of the disease. PMID:25709781

  7. Dilative Arteriopathy and Leucencephalopathy as Manifestations of a Neurometabolic Disease

    PubMed Central

    Finsterer, Josef; Bastovansky, Adam

    2015-01-01

    Background: Dilative arteriopathy plus leucencephalopathy as a manifestation of a mitochondrial disorder (MID) is rare. Case report: In a 70yo Caucasian female, height 160cm, weight 62kg, with mild right-sided hemiparesis due to subacute ischemic stroke in the posterior leg of the left internal capsule, a megadolichobasilar artery and marked leucencephalopathy and gliosis of the pons were detected. In addition, microbleeds in a peripheral distribution at the cortical/subcortical border were noted. After the exclusion of various differentials, which could have explained her abnormalities, a MID was suspected. Conclusions: Dilative arteriopathy of the intra-cerebral arteries, in association with recurrent stroke and supra- and infratentorial leucencephalopathy, but the absence of neurofibromatosis or increased low-density lipoprotein values, is most likely attributable to a non-syndromic MID.

  8. Severe Psychotic Disorder as the Main Manifestation of Adrenal Insufficiency

    PubMed Central

    Farah, Julia de Lima; Lauand, Carolina Villar; Chequi, Lucas; Fortunato, Enrico; Pasqualino, Felipe; Bignotto, Luis Henrique; Batista, Rafael Loch; Aprahamian, Ivan

    2015-01-01

    We describe a case of severe psychotic disorder as the only manifestation of primary adrenal insufficiency. A 63-year-old man presented with psychotic symptoms without any prior psychiatric history. During the clinical and laboratorial investigation, exams revealed a normovolemic hyponatremia. The patient showed no other clinical signs or symptoms compatible with adrenal insufficiency but displayed very high ACTH and low serum cortisol concentrations. Brain magnetic resonance imaging showed no significant changes, including the pituitary gland. The patient was initially treated with intravenous corticosteroids, resulting in rapid remission of the psychotic symptoms. The association between adrenal insufficiency and neuropsychiatric symptoms is rare but these symptoms can often be the first clinical presentation of the disease. PMID:25954562

  9. Cutaneous manifestations of injectable drug use: hidden secrets.

    PubMed

    Bara?ska-Rybak, Wioletta; B?a?ewicz, Izabela; K?kol, Monika; Roter, Miros?aw; Nowicki, Roman

    2014-04-01

    Abscesses related to drug use are the most common cutaneous manifestations among injection drug users, often occurring when the veins become less accessible. In these cases, other techniques may be used to administer drugs, such as skin popping (subcutaneous injection) or muscle popping (intramuscular injection). The main risk factors for abscess formation include skin popping, use of unsterilized needles, and injection of speedball (a mixture of cocaine and heroin). We present a case of recurrent abscesses accompanied by fever, hypersomnia alternating with insomnia, diaphoresis, fatigue, recent weight loss, and agitation following subcutaneous injection of a tramadol, opipramol, and clonazepam mixture. Differential diagnoses included pyoderma gangrenosum on the basis of hepatitis C virus, skin lesions connected with human immunodeficiency virus infection, vasculitis, endocarditis, and serotonin syndrome. The patient was treated with oral antibiotics, surgical incision, and drainage of the abscesses, with consequent improvement. PMID:24818177

  10. [Cutaneous lupus erythematosus and skin manifestations in systemic lupus erythematosus].

    PubMed

    Sticherling, M

    2013-06-01

    Skin manifestations are frequently observed in lupus erythematosus (LE) and are manifold. Nonspecific and specific symptoms can be differentiated with the latter belonging to the dermatologically well-characterized clinical entities of acute cutaneous, subacute cutaneous, chronic cutaneous as well as intermittent cutaneous LE. These forms are differentially related to systemic LE. Patient history and clinical examinations, laboratory and immunoserological findings as well as organ imaging results determine the time point as well as the intensity of therapy. Apart from cessation of smoking and alcohol consumption as well as stringent UV protection, topical therapy with corticosteroids or calcineurin inhibitors may suffice with limited forms of the disease. In many cases, however, systemic treatment with antimalarial drugs as a basic treatment is mandatory. Several immunosuppressive agents can alternatively be used in conjunction with systemic corticosteroids. Early and effective therapy is important to prevent irreversible scarring of the skin and to avoid internal organ damage. PMID:23743985

  11. Clinical Manifestations among Children with Chronic Functional Constipation

    PubMed Central

    Dehghani, Seyed Mohsen; Kulouee, Niloofar; Honar, Naser; Imanieh, Mohammad-Hadi; Haghighat, Mahmood; Javaherizadeh, Hazhir

    2015-01-01

    BACKGROUND Constipation is one of the most frequent cause of patient visits to pediatric gastroenterology clinics. Early diagnosis and treatment is important. There are few studies about clinical manifestations of constipation in children. We aimed to find the relative frequency of gastrointestinal manifestations of constipation among constipated children. METHODS This cross-sectional study was carried out on children aged < 18 years old with chronic functional constipation referred to Imam Reza Clinic of Shiraz University of Medical Sciences. Children with organic causes of chronic constipation were excluded from study. Rome III criteria were used for defining constipation. The duration of study was 1 year starting from September 2010. Abdominal pain, fecal mass, rectal bleeding, anorexia, fecal soiling, retentive posture, withholding behavior, anal fissure, and peri-anal erythema were recorded for each case based on history and physical examination. Data were analyzed using SPSS software, version 13.0 (Chicago, IL, USA). RESULTS Of 222 children with functional constipation, 124(55.9%) were girls and 98 (44.1%) were boys with a mean ± SD age of 5±3.12 years. The mean ± SD duration of constipation was 2.2±1.9 years. Large and hard stool was present in 93.7% of the patients. Painful defecation and withholding behavior were seen in 92.3% and 91.9% of the patients, respectively. Fecal impaction was more frequent among boys compared with girls (p<0.01). Fecal soiling was present in 40.8% of the boys and 28.2% of the girls (p=0.04). CONCLUSION Large and hard stool, painful defecation and withholding behavior were the most frequent signs or symptoms among children with chronic functional constipation. Fresh rectal bleeding and anal fissure were the least frequent signs and symptoms in this group. PMID:25628851

  12. Cryopyrin-Associated Periodic Syndromes: Otolaryngologic and Audiologic Manifestations

    PubMed Central

    Ahmadi, Neda; Brewer, Carmen C.; Zalewski, Christopher; King, Kelly A.; Butman, John A.; Plass, Nicole; Henderson, Cailin; Goldbach-Mansky, Raphaela; Kim, H. Jeffrey

    2012-01-01

    Objective Cryopyrin-associated periodic syndromes (CAPS) represent a spectrum of CIAS1 gene-mediated autoinflammatory diseases characterized by recurrent systemic inflammation. The clinical spectrum of CAPS varies from mild to severe and includes the syndromes historically described as familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease (NOMID). This article presents the largest cohort of patients with CAPS. The objective is to describe the pathogenesis, otolaryngologic, and audiologic manifestations of CAPS. Study Design Prospective (2003–2009). Setting National Institutes of Health. Subjects and Methods Fifty-seven patients with a diagnosis of CAPS were identified (31 NOMID, 11 NOMID/MWS, 9 MWS, and 6 FCAS). Comprehensive data regarding clinical manifestations, audiologic phenotype, and fluid attenuation inversion recovery MRI (FLAIR-MRI) of the brain and inner ear were obtained. Results Complete audiologic data obtained on 70% of ears revealed conductive hearing loss in 4 (11%) NOMID ears and mixed hearing loss in 5 (13%) NOMID and 2 (14%) NOMID/MWS ears. Sensorineural hearing loss (SNHL), worse in higher frequencies, was the most common type of hearing loss and was present in 23 (61%) NOMID, 10 (71%) NOMID/MWS, and 4 (33%) MWS ears. All of the patients with FCAS had normal hearing except 2, who had SNHL from 4 to 8 kHz. On FLAIR-MRI sequence, cochlear enhancement was noted in 26 of 29 (90%) NOMID, 6 of 11 (55%) NOMID/MWS, 3 of 9 (33%) MWS, and 1 of 6 (17%) FCAS patients and was significantly associated with the presence of hearing loss. Maxillary sinus hypoplasia and mucosal thickening were found in 39% and 86% of the cohort, respectively. Conclusion CIAS1 pathway–mediated CAPS is associated with unregulated autoinflammation mediated by interleukin-1 in the cochlea and hearing loss. Timely diagnosis is crucial to initiate early treatment with interleukin-1 receptor antagonists. PMID:21493283

  13. Clinical Manifestations among Children with Chronic Functional Constipation.

    PubMed

    Dehghani, Seyed Mohsen; Kulouee, Niloofar; Honar, Naser; Imanieh, Mohammad-Hadi; Haghighat, Mahmood; Javaherizadeh, Hazhir

    2015-01-01

    BACKGROUND Constipation is one of the most frequent cause of patient visits to pediatric gastroenterology clinics. Early diagnosis and treatment is important. There are few studies about clinical manifestations of constipation in children. We aimed to find the relative frequency of gastrointestinal manifestations of constipation among constipated children. METHODS This cross-sectional study was carried out on children aged < 18 years old with chronic functional constipation referred to Imam Reza Clinic of Shiraz University of Medical Sciences. Children with organic causes of chronic constipation were excluded from study. Rome III criteria were used for defining constipation. The duration of study was 1 year starting from September 2010. Abdominal pain, fecal mass, rectal bleeding, anorexia, fecal soiling, retentive posture, withholding behavior, anal fissure, and peri-anal erythema were recorded for each case based on history and physical examination. Data were analyzed using SPSS software, version 13.0 (Chicago, IL, USA). RESULTS Of 222 children with functional constipation, 124(55.9%) were girls and 98 (44.1%) were boys with a mean ± SD age of 5±3.12 years. The mean ± SD duration of constipation was 2.2±1.9 years. Large and hard stool was present in 93.7% of the patients. Painful defecation and withholding behavior were seen in 92.3% and 91.9% of the patients, respectively. Fecal impaction was more frequent among boys compared with girls (p<0.01). Fecal soiling was present in 40.8% of the boys and 28.2% of the girls (p=0.04). CONCLUSION Large and hard stool, painful defecation and withholding behavior were the most frequent signs or symptoms among children with chronic functional constipation. Fresh rectal bleeding and anal fissure were the least frequent signs and symptoms in this group. PMID:25628851

  14. Ophthalmic manifestations of tuberous sclerosis: a population based study

    PubMed Central

    Rowley, S; O'Callaghan, F; Osborne, J

    2001-01-01

    BACKGROUND/AIMS—Tuberous sclerosis complex (TSC) has retinal and non-retinal ophthalmic manifestations. This study was designed to determine the prevalence of the ophthalmic manifestations and of refractive errors in a population of patients with TSC.?METHODS—179 patients identified were in a prevalence study of TSC in the south of England and 107 of these agreed to full ophthalmic examination which was successful in 100. Ophthalmic examination included examination of the eyelids, cover test, examination of the irides, dilation funduscopy using both direct and indirect ophthalmoscopy, and refraction using retinoscopy. Myopia was defined as a spherical equivalent +0.5D.?RESULTS—Retinal hamartomas were seen in 44 of the 100 patients. The commonest morphological type of hamartoma seen was the flat, translucent lesion in 31 of the 44 patients (70%). The multinodular "mulberry" lesion was seen in 24 of the 44 patients (55%) and the transitional type lesion was seen in four of the 44 patients (9%). Punched out areas of retinal depigmentation were seen in 39 of the 100 patients but only six of 100 controls. 27% of eyes were myopic, 22% were hyperopic, and 27% had astigmatism >0.75D. Of the non-retinal findings, 39 patients had angiofibromas of the eyelids, five had non-paralytic strabismus, and three had colobomas.?CONCLUSION—Apart from the higher prevalence of flat retinal hamartomas, the findings of this study compare closely with previous large clinic based series of TSC patients. Refractive findings were similar to previous studies of a similarly aged non-TSC population. This is the first series to document the statistically significant association of punched out chorioretinal depigmentation with TSC and the authors believe that it should be looked for as an aid to diagnosis.?? PMID:11264130

  15. Clinical profile of cutaneous manifestations with and without hematologic disease: A comparative study

    PubMed Central

    Pande, Sushil Yashwant; Kharkar, Vidya

    2014-01-01

    Aim: The aim was to study the clinical profile of cutaneous manifestations of hematologic disorders and to compare it with that of non-hematologic disorders. Materials and Methods: Cutaneous manifestations of hematologic diseases fall in seven well-defined categories. A total of 153 outpatients with skin manifestations fitting in these categories were enrolled in a comparative study of 1-year duration. Clinical profile of these cutaneous manifestations was studied and any underlying hematologic disorder was ruled out with the help of a hematologist. Difference in the clinical profile of cutaneous manifestations with and without hematologic diseases was studied. Result: Of the 26,174 outpatients during the study period, 153 had cutaneous manifestations fitting in the categories of hematologic disorders. Of these 153 patients, 33 had hematologic disease as the cause of their cutaneous manifestation (21.57%), whereas 78.42% had no hematologic disorder. Disorders of hemostasis formed the largest group (36%) followed by cutaneous deposits/infiltrations (15%), vesiculobullous disorders (6%), and cutaneous vasculitis (9%) were least commonly associated with hematologic disorders. Conclusion: Hematologic diseases are associated with complex array of cutaneous manifestations. The incidence of hematologic disease–associated cutaneous manifestations was 0.13%. Findings of this study will help dermatologists and physicians with the early recognition of cutaneous signs of hematologic disorders. PMID:24860745

  16. Oral manifestations of Parry–Romberg syndrome: A review of literature

    PubMed Central

    Al-Aizari, Nader A.; Azzeghaiby, Saleh Nasser; Al-Shamiri, Hashem Motahir; Darwish, Shourouk; Tarakji, Bassel

    2015-01-01

    Parry–Romberg syndrome (PRS) or progressive facial hemiatrophy is a developmental craniofacial disorder of unknown etiology characterized by a slowly progressive unilateral facial atrophy. It is associated with different systemic manifestations particularly, maxillofacial, neurologic and ophthalmologic abnormalities. Dentists must be aware of PRS to identify this invalidating disorder. In this article, we review the etiology, clinical features (especially craniofacial and dental manifestations) and treatment of PRS. We searched in PubMed line using specific words such as PRS from 2008 to 2014 (August). We identify 14 papers have described oral manifestations of this syndrome. We excluded all the article papers that did not indicate to oral manifestations of PRS. PMID:25878963

  17. Oral manifestations of Parry-Romberg syndrome: A review of literature.

    PubMed

    Al-Aizari, Nader A; Azzeghaiby, Saleh Nasser; Al-Shamiri, Hashem Motahir; Darwish, Shourouk; Tarakji, Bassel

    2015-01-01

    Parry-Romberg syndrome (PRS) or progressive facial hemiatrophy is a developmental craniofacial disorder of unknown etiology characterized by a slowly progressive unilateral facial atrophy. It is associated with different systemic manifestations particularly, maxillofacial, neurologic and ophthalmologic abnormalities. Dentists must be aware of PRS to identify this invalidating disorder. In this article, we review the etiology, clinical features (especially craniofacial and dental manifestations) and treatment of PRS. We searched in PubMed line using specific words such as PRS from 2008 to 2014 (August). We identify 14 papers have described oral manifestations of this syndrome. We excluded all the article papers that did not indicate to oral manifestations of PRS. PMID:25878963

  18. Severe congenital thrombocytopaenia – first clinical manifestation of Noonan syndrome

    PubMed Central

    Nunes, Paula; Aguilar, Sara; Prado, Sara Noéme; Palaré, Maria João; Ferrão, Anabela; Morais, Anabela

    2012-01-01

    This report focuses on a male infant, the first born of non-consanguineous parents diagnosed with polyhydramnios at 26 weeks of gestation. The newborn was admitted during the neonatal period with bleeding diathesis associated with a low platelet count at birth (5×109/l).The authors registered a persistent low platelet count (9000–129 000/l) during the infants 1st year of life. Physical examination revealed a petechial rash, a dysmorphic face and bilateral cryptorchidism, in the absence of organomegaly. Additionally, cardiologic evaluation revealed an aortic valve dysplasia and an atrial septal defect, while bone marrow biopsy and aspiration were found normal. Throughout the investigation, the authors excluded congenital infection, alloimmune and familiar thrombocytopaenia, Fanconi anaemia and thrombocytopaenia absent radius syndrome. The cytogenetic analysis revealed a mutation in the PTPN11 gene associated with Noonan syndrome. Here the author highlights that severe neonatal thrombocytopaenia is a manifestation that should be considered in the diagnosis and clinical management of Noonan’s syndrome. PMID:22605701

  19. Epidemiology and Clinical Manifestations of Enteroaggregative Escherichia coli

    PubMed Central

    Hebbelstrup Jensen, Betina; Olsen, Katharina E. P.; Struve, Carsten; Petersen, Andreas Munk

    2014-01-01

    SUMMARY Enteroaggregative Escherichia coli (EAEC) represents a heterogeneous group of E. coli strains. The pathogenicity and clinical relevance of these bacteria are still controversial. In this review, we describe the clinical significance of EAEC regarding patterns of infection in humans, transmission, reservoirs, and symptoms. Manifestations associated with EAEC infection include watery diarrhea, mucoid diarrhea, low-grade fever, nausea, tenesmus, and borborygmi. In early studies, EAEC was considered to be an opportunistic pathogen associated with diarrhea in HIV patients and in malnourished children in developing countries. In recent studies, associations with traveler's diarrhea, the occurrence of diarrhea cases in industrialized countries, and outbreaks of diarrhea in Europe and Asia have been reported. In the spring of 2011, a large outbreak of hemolytic-uremic syndrome (HUS) and hemorrhagic colitis occurred in Germany due to an EAEC O104:H4 strain, causing 54 deaths and 855 cases of HUS. This strain produces the potent Shiga toxin along with the aggregative fimbriae. An outbreak of urinary tract infection associated with EAEC in Copenhagen, Denmark, occurred in 1991; this involved extensive production of biofilm, an important characteristic of the pathogenicity of EAEC. However, the heterogeneity of EAEC continues to complicate diagnostics and also our understanding of pathogenicity. PMID:24982324

  20. Unusual manifestation of hepatic encephalopathy in two Irish wolfhound siblings.

    PubMed

    Herden, C; Beineke, A; Hetzel, U; Lemmer, W; Baumgärtner, W

    2003-11-29

    In hepatic encephalopathy the brain lesions are usually characterised by polymicrocavitation, preferentially in the white matter, and the occurrence of Alzheimer type II cells. This paper describes an unusual manifestation of hepatic encephalopathy in two Irish wolfhound siblings in which the white matter was not involved predominantly. Both puppies had developed progressive neurological disturbances and signs of blindness. Histologically, there were widespread spongiform changes in the neuropil and fibre bundles interspersed within the grey matter, and there were some neuronal vacuoles. In both animals, the regions of the brain mainly affected were the nucleus caudatus, amygdala, cerebellar nuclei, mesencephalon, thalamus, hypothalamus and medulla oblongata. An astrogliosis characterised by Alzheimer type II-like cells was also observed. Electron microscopy revealed a splitting of the myelin sheath. No infectious agents such as rabies virus, canine distemper virus or prion proteins were detected. The main findings in the portal regions of the liver consisted of a dilatation of the lymphatic vessels and increased numbers of small arteries, indicating that a portosystemic shunt was the probable cause of the spongiform brain lesions. PMID:14682542

  1. Cytomegalovirus pneumonia as the first manifestation of severe combined immunodeficiency

    PubMed Central

    Jo?czyk-Potoczna, Katarzyna; Ossowska, Lidia; Br?borowicz, Anna; Bartkowska-?niatkowska, Alicja; Wachowiak, Jacek

    2014-01-01

    Severe combined immunodeficiency (SCID) is characterized by the absence of functional T lymphocytes and impairment of adaptive immunity. While heterogeneity of the genetic background in SCID leads to the variability of immune phenotypes, most of affected newborns appear healthy but within the first few months they develop life-threatening opportunistic respiratory or gastrointestinal tract infections. The objective of the study was to define the presenting features and etiology of infections in children with SCID. We retrospectively reviewed five children in whom the diagnosis of SCID had been established in our pediatric immunology clinic over the last 10-year period. A viral respiratory tract infection was the first manifestation of SCID in all the children studied. Cytomegalovirus (CMV) pneumonia was recognized in as many as 4 cases and coronavirus pulmonary infection was diagnosed in one case, whereas Pneumocystis jiroveci was identified as a co-pathogen in one CMV-infected patient. Severe combined immunodeficiency is a pediatric emergency condition and given the significant impact of pulmonary CMV infection in SCID children, establishing an accurate etiological diagnosis is of essential importance in instituting the specific treatment and improving the outcome.

  2. Manifestly Covariant Gauge-invariant Cosmological Perturbation Theory

    E-print Network

    P. G. Miedema; W. A. van Leeuwen

    2014-10-01

    It is shown that a first-order cosmological perturbation theory for the open, flat and closed Friedmann-Lema\\^itre-Robertson-Walker universes admits one, and only one, gauge-invariant variable which describes the perturbation to the energy density and which becomes equal to the usual Newtonian energy density in the non-relativistic limit. The same holds true for the perturbation to the particle number density. Using these two new variables, a new manifestly gauge-invariant cosmological perturbation theory has been developed. Density perturbations evolve diabatically. Perturbations in the total energy density are gravitationally coupled to perturbations in the particle number density, irrespective of the nature of the particles. There is, in first-order, no back-reaction of perturbations to the global expansion of the universe. Small-scale perturbations in the radiation-dominated era oscillate with an increasing amplitude, whereas in older, less precise treatments, oscillating perturbations are found with a decreasing amplitude. This is a completely new and, obviously, important result, since it makes it possible to explain and understand the formation of massive stars after decoupling of matter and radiation.

  3. Glomerulonephritis and cryoglobulinemia: first manifestation of visceral leishmaniasis.

    PubMed

    Ortiz, Milagros; Mon, Carmen; Herrero, Juan Carlos; Oliet, Aniana; Rodríguez, Isabel; Ortega, Olimpia; Gallar, Paloma; Hinostroza, Julie; Cobo, Gabriela; Del Alamo, Manuel; Jiménez, Juana; Torres, Rafael; Digiogia, Cristina; San Martin, Juan; Vigil, Ana I; Blanco, Julia

    2015-06-01

    Visceral leishmaniasis due to Leishmania Infantum is an endemic parasitic infection in the Mediterranean area. Since 2009, Europe's largest outbreak of Leishmaniasis has been reported in the region of Madrid (Spain). Renal involvement is an unusual complication. Different forms of renal disease have been described: interstitial, glomerular, and vascular damage. Direct invasion of renal parenchyma by the parasite has been described as a mechanism of kidney damage, especially in the immunocompromised. Immune complex deposition and T cells adhesion molecules activation have demonstrated that a pathogenic role in glomerulonephritis related to visceral leishmaniasis. The association between mixed cryoglobulinemia and visceral leishmaniasis has been previously reported in six patients. Renal involvement is only described in one of them. From July 2009 to October 2012, 4 patients with membranoproliferative glomerulonephritis and mixed cryoglobulinemia with negative serology for hepatitis B and C were diagnosed in our hospital. Serology of Leishmania in serum bank samples was performed; it was positive in 3 patients. Leishmania parasite was confirmed by other tests. We present 3 patients with mixed cryoglobulinemia and membranoproliferative glomerulonephritis as first clinical manifestation of visceral leishmaniasis. PMID:25600859

  4. Allergic manifestations of skin diseases--atopic dermatitis.

    PubMed

    Breuer, Kristine; Werfel, Thomas; Kapp, Alexander

    2006-01-01

    Atopic dermatitis (AD) is a chronic inflammatory skin disease which often becomes manifest in early infancy and is characterized by itchy eczematous lesions with characteristic localization. The cellular infiltrate of allergic eczematous skin diseases (i.e. AD, allergic contact dermatitis) is mainly composed of mononuclear cells. Whereas allergic contact dermatitis is always triggered by allergen-specific T cells, a number of allergic and nonallergic trigger factors appear to be relevant in AD. This article discusses data coming from immunological studies focusing on T-cell responses in AD. The concept of a switch from a T helper type 1 (Th1) to a Th2 cytokine profile in lesional skin of AD is well accepted. Besides CD4+ T lymphocytes, CD8+ cells are likely to play an important role in the pathogenesis of AD. Recent studies point to the induction of apoptosis in keratinocytes by interferon-gamma derived from skin-homing T cells as a further important mechanism for the induction and maintenance of the eczema. Recent clinical studies have confirmed the major role of food allergy and infectious microorganisms as trigger factors of AD. New therapeutic strategies for AD include topical calcineurin inhibitors which were introduced as a new therapeutic principle at the beginning of this decade. PMID:16354950

  5. Renal manifestations of Dent disease and Lowe syndrome.

    PubMed

    Cho, Hee Yeon; Lee, Bum Hee; Choi, Hyun Jin; Ha, Il Soo; Choi, Yong; Cheong, Hae Il

    2008-02-01

    To date, two responsible genes for the development of Dent disease have been identified: CLCN5 and OCRL1. In this study, genotype-phenotype correlations were studied in patients with Dent disease and those with Lowe syndrome. Among the 12 boys with a phenotype typical of Dent disease, nine had a mutation in CLCN5 (Dent disease 1), two had a mutation in OCRL1 (Dent disease 2), and one had no mutations in either gene. All seven boys with a clinical diagnosis of Lowe syndrome had a mutation in OCRL1. Patients with Lowe syndrome showed more frequent hypophosphatemia/rickets and more prominent tubular proteinuria than patients with Dent disease 1, and patients with Dent disease 2 had higher degree of tubular proteinuria and hypercalciuria than patients with Dent disease 1. Additionally, one patient with Dent disease 2 showed a mild degree of developmental delay, elevated serum muscle enzyme levels, and cryptorchidism. In this study, the genetic heterogeneity in Dent disease and the phenotypic heterogeneity in Lowe syndrome were confirmed. In patients with Dent disease, the presence of the above-mentioned extrarenal manifestations indicates that it is more likely that the patient is affected by Dent disease 2 than by Dent disease 1. PMID:18038239

  6. Investigation of a female manifesting Becker muscular dystrophy.

    PubMed Central

    Glass, I A; Nicholson, L V; Watkiss, E; Johnson, M A; Roberts, R G; Abbs, S; Brittain-Jones, S; Boddie, H G

    1992-01-01

    Females manifesting Becker muscular dystrophy (BMD) are even more rarely observed than for the allelic condition Duchenne muscular dystrophy. The male proband has typical BMD with greatly raised CK activity and a myopathic muscle biopsy. His mother experienced walking difficulties from 35 years of age and has a myopathy with marked calf hypertrophy, a raised CK, and a myopathic muscle biopsy. Dystrophin analysis was undertaken on both the proband and his mother. Immunoblotting showed a protein of normal size but of reduced abundance in both. Immunocytochemical analysis in the proband indicated that the majority of the fibres showed weak dystrophin labelling and in his mother both dystrophin positive and dystrophin negative fibres were present. Non-random X inactivation at locus DXS255, was observed in DNA isolated from peripheral lymphocytes of the mother. Neither extended multiplex PCR performed on DNA from the proband nor analysis of lymphocyte derived mRNA showed a structural alteration in the dystrophin gene suggesting that an unusual mutation was responsible for BMD in this family. Images PMID:1518025

  7. Adult mitochondrial DNA depletion syndrome with mild manifestations.

    PubMed

    Finsterer, Josef; G Kovacs, Gabor; Ahting, Uwe

    2013-06-25

    Mitochondrial DNA depletion syndrome (MDS) is usually a severe disorder of infancy or childhood, due to a reduced copy number of mtDNA molecules. MDS with only mild, nonspecific clinical manifestations and onset in adulthood has not been reported. A 47-year-old Caucasian female with short stature and a history of migraine, endometriosis, Crohn's disease, C-cell carcinoma of the thyroid gland, and a family history positive for mitochondrial disorder (2 sisters, aunt, niece), developed day-time sleepiness, exercise intolerance, and myalgias in the lower-limb muscles since age 46y. She slept 9-10 hours during the night and 2 hours after lunch daily. Clinical exam revealed sore neck muscles, bilateral ptosis, and reduced Achilles tendon reflexes exclusively. Blood tests revealed hyperlipidemia exclusively. Nerve conduction studies, needle electromyography, and cerebral and spinal magnetic resonance imaging were noninformative. Muscle biopsy revealed detached lobulated fibers with subsarcolemmal accentuation of the NADH and SDH staining. Realtime polymerase chain reaction revealed depletion of the mtDNA down to 9% of normal. MDS may be associated with a mild phenotype in adults and may not significantly progress during the first year after onset. In an adult with hypersomnia, severe tiredness, exercise intolerance, and a family history positive for mitochondrial disorder, a MDS should be considered. PMID:23888212

  8. Para-dichlorobenzene toxicity – a review of potential neurotoxic manifestations

    PubMed Central

    Dubey, Divyanshu; Sharma, Vibhash D.; Pass, Steven E.; Sawhney, Anshudha

    2014-01-01

    Background: Para-dichlorobenzene (PDCB) is an active ingredient of mothballs, deodorizers and fumigants. Due to the easy availability of this chemical, there is a considerable risk for accidental or intentional toxic exposure. Recently, multiple cases of PDCB toxicity due to mothball ingestion were reported. PDCB toxicity can affect multiple organ systems including liver, kidneys, skin, lung and the central nervous system (CNS). CNS toxicity often results in leukoencephalopathy and heterogeneous neurological manifestations. Objectives: The objective of this study was to illustrate the clinical presentation, imaging findings, diagnosis and management of PDCB toxicity. Methods: We carried out a literature review of the pharmacological and toxicological properties of PDCB. Conclusions: PDCB and other aromatic hydrocarbons are capable of CNS tissue damage and in promoting functional neurological decline. While very little is currently known about prevalence of PDCB addiction, it cannot be ruled out that its illicit use among young people is under-recognized. The number of cases of PDCB toxicity might also rise due to the increasing industrial and domestic use of this chemical. PMID:24790648

  9. Ocular manifestations in children and adolescents with Lyme arthritis

    PubMed Central

    Huppertz, H.; Munchmeier, D.; Lieb, W.

    1999-01-01

    BACKGROUND—Lyme arthritis is the most frequent late manifestation of Lyme borreliosis and has been associated with ocular inflammation.?METHODS—A group of 153 children and adolescents with arthritis, 84 of whom had Lyme arthritis and 69 other causes of arthritis, were followed prospectively for 22-73 (median 44) months in the course of a national study.?RESULTS—Three of 84 patients with Lyme arthritis had ocular inflammation (4%), including keratitis, anterior uveitis, and uveitis intermedia. All three had symptoms of decreased visual acuity. Whereas anterior uveitis disappeared without sequelae, a corneal scar and a permanent loss of visual acuity in the patients with keratitis and intermediate uveitis remained. Systematic examination of all patients revealed no further ocular involvement. Of 69 patients with other causes of arthritis who were followed in parallel as a control group, four of 15 patients with early onset pauciarticular juvenile rheumatoid arthritis had chronic anterior uveitis and two of 12 patients with juvenile spondyloarthropathy had acute anterior uveitis.?CONCLUSIONS—Ocular involvement with keratitis, anterior uveitis, and intermediate uveitis may occur in children and adolescents with Lyme arthritis. Visual loss appears to be symptomatic, making regular ocular screening of such patients unnecessary.?? PMID:10502576

  10. Fraser Syndrome-Oral Manifestations and a Dental Care Protocol

    PubMed Central

    de Oliveira, Talita Lopes; de Sant'Anna, Giselle Rodrigues

    2014-01-01

    Fraser syndrome is a rare genetic malformation with an autosomal recessive pattern of inheritance and an incidence of consanguinity ranging from 15% to 25%. A 5-year-old male patient who is a carrier of Fraser syndrome initiated treatment in the pediatric dentistry sector. The patient was fed parenterally since birth, experienced recurring bouts of chronic lung disease, and was referred to the pediatric dentistry sector by the medical team. Radiographic examination revealed the presence of all permanent teeth. Supragingival dental calculus, halitosis, and gingival inflammation were also observed. Dental calculus was removed by manual scraping, and chemotherapeutic agents were used, chlorhexidine 0,12%, chlorhexidine gel 2%, and PVP-I, to control the bacterial flora. The patient is still being monitored after an 8-year follow-up period, the complications associated with periodontal disease decreased, and since the initiation of treatment, the patient has not needed to be hospitalized because of chronic lung disease. This study reports the case of a patient diagnosed with Fraser syndrome and describes the clinical manifestations (general and oral). PMID:25587460

  11. Ear nose throat manifestations in hypoidrotic ectodermal dysplasia.

    PubMed

    Callea, Michele; Teggi, Roberto; Yavuz, Izzet; Tadini, Gianluca; Priolo, Manuela; Crovella, Sergio; Clarich, Gabriella; Grasso, Domenico Leonardo

    2013-11-01

    The ectodermal dysplasias (EDs) are a large and complex group of inherited disorders. In various combinations, they all share anomalies in ectodermal derived structures: hair, teeth, nails and sweat gland function. Clinical overlap is present among EDs. Few causative genes have been identified, to date. Altered gene expression is not limited to the ectoderm but a concomitant effect on developing mesenchymal structures, with modification of ectodermal-mesenchymal signaling, takes place. The two major categories of ED include the hidrotic and hypohidrotic form, the latter more frequent; they differentiate each other for the presence or absence of sweat glands. We report Ear Nose Throat manifestations of ED, linked to the reduction of mucous glands in the nasal fossae with reduced ciliar function, and decrease salivary glands function. Often patients report an increased rate of infections of the upper respiratory tract and of the ear. Nasal obstruction due to the presence of nasal crusting, hearing loss and throat hoarseness are the most represented symptoms. Environmental measures, including a correct air temperature and humidification, is mandatory above all in subjects affected by hypohidrotic form. PMID:24080322

  12. Evaluation of ophthalmic manifestations in patients with intracranial tumours.

    PubMed

    Wadud, S A; Ahmed, S; Choudhury, N; Chowdhury, D

    2014-04-01

    The present study was done to observe the ophthalmic manifestations in patients with intracranial tumor. This was a prospective, purposive, consecutive, observational study conducted in patients with radiologically proven intracranial tumors in the department of Ophthalmology with collaboration of Department of Neuro-surgery of Bangabandhu Sheikh Mujib Medical University from January 2006 to December 2010. All cases had tissue histopathology confirmation post-operatively. The most common type of intracranial tumor was pituitary adenoma (58.04%), followed by craniopharyngioma (20.53%), posterior fossa tumour (12.50%) [medulloblestoma, ependymoma], meningioma (8.04%) [sphenoidalwing meningioma, petroclavel meningioma, oligodendroglioma] and others (0.89%) [nasopharyngial carcinoma, esthesio - astrocytoma]. Common neuro-ophthalmological findings were visual blur (91.07%), visual field defect (71.42%), optic disc changes (50%), pupillary light reaction defect (48.21%) and colour vision defect (46.42%). The study shows, pituitary adenoma is the most common tumor that impairs the visual pathway structures followed by craniopharyngioma, posterior fossa tumour & meningioma. Furthermore, decreased visual acuity, visual field defect, abnormal optic discs, relative afferent pupillary defect and ophthalmoplegia etc. are the common neuro-ophthalmic features that should be carefully examined for early detection of intracranial tumors. PMID:24858153

  13. The manifestation of oxygen contamination in ErD2.

    SciTech Connect

    Snow, Clark Sheldon; Parish, Chad Michael; Brewer, Luke N.

    2008-10-01

    Erbium dihydride Er(H,D,T){sub 2} is a fluorite structure rare-earth dihydride useful for the storage of hydrogen isotopes in the solid state. However, thermodynamic predictions indicate that erbium oxide formation will proceed readily during processing, which may detrimentally contaminate Er(H,D,T){sub 2} films. In this work, transmission electron microscopy (TEM) techniques including energy-dispersive x-ray spectroscopy, energy-filtered TEM, selected area electron diffraction, and high-resolution TEM are used to examine the manifestation of oxygen contamination in ErD{sub 2} thin films. An oxide layer {approx}30-130 nm thick was found on top of the underlying ErD{sub 2} film, and showed a cube-on-cube epitaxial orientation to the underlying ErD{sub 2}. Electron diffraction confirmed the oxide layer to be Er{sub 2}O{sub 3}. While the majority of the film was observed to have the expected fluorite structure for ErD{sub 2}, secondary diffraction spots suggested the possibility of either nanoscale oxide inclusions or hydrogen ordering. In situ heating experiments combined with electron diffraction ruled out the possibility of hydrogen ordering, so epitaxial oxide nanoinclusions within the ErD{sub 2} matrix are hypothesized. TEM techniques were applied to examine this oxide nanoinclusion hypothesis.

  14. [Intermittent paraparesis as manifestation of a bilateral chronic subdural hematoma].

    PubMed

    Schaller, B; Radziwill, A J; Wasner, M; Gratzl, O; Steck, A J

    1999-07-27

    Chronic subdural haematomas are mainly related to slight or moderate head trauma with consecutive lesion of bridge or cortical veins and bleeding in the subdural space. Further predisposing factors are known impairment of coagulation (coagulopathies, treatment with anticoagulants, alcohol abuse), risk factors for degenerative disease of the arteries (diabetes mellitus, arterial hypertension), and development of pressure gradients (hydrocephalus, epileptic seizures, lumbar puncture, CSF drainage and cerebral atrophy). Chronic subdural haematomas appear bilaterally in 20 to 25% of cases. We report on a 69-year-old male with a 4-day history of intermittent, proximal, painless paraparesis (BMA grade M2-5) without a trigger event. Sensibility was normal in all qualities and vigilance was not disturbed. Computed tomography of the neurocranium revealed a bitemporally located chronic subdural haematoma with extension to parietal on both sides. Trepanation was performed over the tuber parietale and temporoparietally on both sides, with release of 150 ml fluid. The neurologic deficits regressed totally within 12 hours postoperatively. To the best of our knowledge, we are the first to describe the clinical paradox of intermittent, painless paraparesis with preserved sensibility and without disturbances of vigilance, as manifestation of a chronic subdural haematoma possibly leading to impairment of cerebral blood flow in the area of the middle cerebral artery. Small changes in systemic blood pressure lead to changes in cerebral perfusion pressure due to vessel compression by the haematoma, thus explaining the intermittent character of the clinical presentation. PMID:10464909

  15. Structural manifestations of aging in Se-rich glasses

    NASA Astrophysics Data System (ADS)

    Dash, S.; Ravindren, S.; Chen, P.; Boolchand, P.

    2015-03-01

    We examine weakly cross-linked GexSe100-x (0%manifested. Work supported by NSF Grant DMR 08-53957.

  16. Upper Gastrointestinal Bleeding as a Manifestation of Metastasis from Laryngeal Cancer

    PubMed Central

    Nazeer, Hammad; Thakur, Kshitij; Zeitz, Monica

    2015-01-01

    Upper gastrointestinal (GI) bleeding can be a rare manifestation of primary or metastatic tumor in the stomach. Tumors that commonly metastasize to stomach include breast, lung, and malignant melanoma. Laryngeal cancer usually metastasizes to the lung and cervical spine. We report the first case of upper GI bleed as a manifestation of laryngeal cancer in the stomach.

  17. Manifestations of Heterosexism in Icelandic Upper Secondary Schools and the Responses of LGBT Students

    ERIC Educational Resources Information Center

    Kjaran, Jón Ingvar; Jóhannesson, Ingólfur Ásgeir

    2013-01-01

    How does institutionalized heterosexism manifest itself in Icelandic upper secondary schools and how do lesbian, gay, bisexual, and transgender (LGBT) students respond to these manifestations? In addressing these questions, interviews were conducted with six current and former LGBT upper secondary school students, using queer theory and thematic…

  18. [Case report of skin manifestations in infantile Mediterranean kala-azar].

    PubMed

    Ayadi, A; Loukil, M; Lakhoua, R; Boubaker, S; Debbabi, A; Ben Rachid, S B; Jedidi, H

    1992-04-01

    The case of a 19-month-old girl with visceral leishmaniasis and unusual skin manifestations is reported. Emphasis is put on the fact that such manifestations are unusual in Mediterranean infantile leishmaniasis. The clinical features, parasitology, histology, and course of these skin lesions are discussed. PMID:1616241

  19. Right Bundle Branch Block: An Uncommon Cardiotoxic Manifestation of Hair Dye Poisoning-A Case Report

    PubMed Central

    Balasubramanian, Deepak; Subramanian, Saravanan; Thangaraju, Pugazhenthan; Shanmugam, Kani

    2014-01-01

    Hair dye poisoning has been rising in incidence in the recent years. Apart from the commoner manifestations of upper airway edema, rhabdomyolysis and acute renal failure, cardiac toxicity, convulsions and sudden cardiac death are relatively rare complications. We discuss a case of hair dye poisoning manifesting as oropharyngeal edema along with cardiac complication. The patient survived. PMID:24596762

  20. Recurrent episodic acute kidney injury as presenting manifestation of mitochondrial myopathy

    PubMed Central

    Matthai, T. P.; Zachariah, U. G.; Matthai, S. M.

    2014-01-01

    Mitochondrial cytopathies (MC) are a rare heterogenous group of disorders with frequent multisystem involvement including uncommon renal manifestations. Acute kidney injury (AKI) as the primary manifestation of MC is extremely rare. Here, we report a case of recurrent episodic AKI in an adult male who was subsequently diagnosed to have mitochondrial disease. PMID:25484535

  1. 19 CFR 103.31 - Information on vessel manifests and summary statistical reports.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... (e) Availability of manifest data on CD-ROMS —(1) Availability. Manifest...available to interested members of the public on CD-ROMS. This data, compiled daily, will...this section will not be included on the CD-ROMS. These CD-ROMS may be...

  2. [Severe, refractory neonatal inflammatory syndrome as the manifestation of a systemic multivisceral and meningeal syndrome].

    PubMed

    Lachaux, A; Hermier, M; Foasso, M F; Descos, B; Collet, J P; David, L

    1987-02-01

    A neonate presented with an inflammatory syndrome with multisystemic manifestations. There was no remission until age 6 years, despite anti-inflammatory treatments. Neonatal onset and neuromeningeal manifestations identified a peculiar rare inflammatory syndrome, whose relationship with juvenile rheumatoid arthritis is discussed. In the case reported, renal, abdominal, deep lymphatic and cranial involvements were present: these have not been previously reported. PMID:3579468

  3. Rift Valley fever ocular manifestations: observations during the 1977 epidemic in Egypt

    Microsoft Academic Search

    A L Siam; J M Meegan; K F Gharbawi

    1980-01-01

    Ocular manifestations resulting from Rift Valley fever (RVF) virus infection were studied during an extensive RVF epidemic in Egypt during 1977. Colour photography and fluorescein angiography of 7 serologically diagnosed patients showed the commonest manifestations to be macular, paramacular, and\\/or extramacular retinal lesions, often occurring bilaterally. Haemorrhage and oedema were frequently associated with the lesions, and vasculitis, retinitis, and vascular

  4. Cutaneous Manifestations of Relapsing Polychondritis in a Patient Receiving Goserelin for Carcinoma of the Prostate

    Microsoft Academic Search

    M.-P. Labarfhe; P. Bayle-Lebey; J. Bazex

    1997-01-01

    Relapsing polychondritis is a chronic rheumatologic disorder of unknown etiology. Cutaneous manifestations occur in nearly half of the patients and often precede cartilaginous involvement. We present the case of a man with a history of prostatic adenocarcinoma who underwent monthly injections of goserelin (Zoladex®), an LH-RH analogue. Five months after the beginning of the treatment, he presented cutaneous manifestations, which

  5. Low-dose weekly methotrexate for progressive neuropsychiatric manifestations in Behcet's disease

    Microsoft Academic Search

    Shunsei Hirohata; Hiroko Suda; Takashi Hashimoto

    1998-01-01

    The most serious central nervous system (CNS) manifestation in Behcet's disease is a slowly progressive dementia (progressive NB), which may ultimately lead to the deterioration of the personality of patients. An open trial was designed to investigate the efficacy of low dose weekly methotrexate (MTX) therapy for progressive NB. Six patients with Behcet's disease, whose neuropsychiatric manifestations were judged to

  6. Polyarteritis Nodosa and Extrahepatic Manifestations of HBV Infection: The Case Against Autoimmune Intervention in Pathogenesis

    Microsoft Academic Search

    Christian Trepo

    2001-01-01

    Numerous extrahepatic manifestations have been reported in patients with both acute and chronic hepatitis B (arthralgias or arthritis, skin rashes, glomerulonephritis and neuritis), all of which are present in polyarteritis nodosa (PAN) which is the most unique and spectacular extrahepatic manifestation. In the 1970s, the frequency of PAN due to the hepatitis B (HBV) reached 30%. Immunization programs explain the

  7. Les manifestations buccofaciales de la sclérodermie systémique : étude de 30 patients consécutifs

    Microsoft Academic Search

    C. Vincent; C. Agard; S. Barbarot; J.-M. N’Guyen; B. Planchon; C. Durant; M.-A. Pistorius; B. Dreno; T. Ponge; J.-F. Stalder; J.-M. Mercier; M. Hamidou

    2009-01-01

    IntroductionThe face is frequently involved in systemic sclerosis. The main stomatologic manifestations include limited mouth opening, xerostomia, skin atrophy, trigeminal neuralgia. The objective of this study was to describe oral and facial manifestations observed in scleroderma patients from our cohort.

  8. Guillain-Barré syndrome as an extraintestinal manifestation of Crohn's disease.

    PubMed Central

    de la Fuente-Fernández, R.; Rubio-Nazabal, E.; de la Iglesia-Martínez, F.

    1995-01-01

    A variety of extraintestinal manifestations, probably immune-mediated, may appear during relapses of Crohn's disease. We report the clinical observation of a 34-year-old woman who developed a Guillain-Barré syndrome, aphthous stomatitis and oligoarthritis during a relapse of Crohn's ileocolitis. This case suggests that the Guillain-Barré syndrome may be another extraintestinal manifestation of Crohn's disease. PMID:7567741

  9. 20 CFR 702.604 - Determining the amount of compensation for occupational disease claims which become manifest...

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ...amount of compensation for occupational disease claims which become manifest after retirement...ADMINISTRATION AND PROCEDURE Occupational Disease Which Does Not Immediately Result in Death...amount of compensation for occupational disease claims which become manifest after...

  10. 20 CFR 702.603 - Determining the payrate for compensating occupational disease claims which become manifest after...

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ...the payrate for compensating occupational disease claims which become manifest after retirement...ADMINISTRATION AND PROCEDURE Occupational Disease Which Does Not Immediately Result in Death...the payrate for compensating occupational disease claims which become manifest after...

  11. Systemic lupus erythematosus: Clinical manifestations and immunological parameters in 194 patients. Subgroup classification of SLE

    Microsoft Academic Search

    J. Antolin; M. J. Amerigo; A. Cantabrana; A. Roces; P. Jimenez

    1995-01-01

    Summary We analysed the prevalence of clinical manifestations and immunological parameters in 194 patients with SLE and classified them in subgroups according to age at onset, gender and type of antibodies: we detected significant differences in the various subgroups.

  12. Investigating Factorial Invariance of Latent Variables Across Populations When Manifest Variables Are Missing Completely

    PubMed Central

    Widaman, Keith F.; Grimm, Kevin J.; Early, Dawnté R.; Robins, Richard W.; Conger, Rand D.

    2013-01-01

    Difficulties arise in multiple-group evaluations of factorial invariance if particular manifest variables are missing completely in certain groups. Ad hoc analytic alternatives can be used in such situations (e.g., deleting manifest variables), but some common approaches, such as multiple imputation, are not viable. At least 3 solutions to this problem are viable: analyzing differing sets of variables across groups, using pattern mixture approaches, and a new method using random number generation. The latter solution, proposed in this article, is to generate pseudo-random normal deviates for all observations for manifest variables that are missing completely in a given sample and then to specify multiple-group models in a way that respects the random nature of these values. An empirical example is presented in detail comparing the 3 approaches. The proposed solution can enable quantitative comparisons at the latent variable level between groups using programs that require the same number of manifest variables in each group. PMID:24019738

  13. Disseminated cerebral hemorrhages as unusual manifestation of toxoplasmic encephalitis in AIDS.

    PubMed

    Berlit, P; Popescu, O; Weng, Y; Malessa, R

    1996-11-01

    Hemorrhagic lesions are uncommon in central nervous system (CNS) toxoplasmosis. We report here the case of a patient with multiple cerebral hemorrhages as the initial manifestation of HIV infection due to CNS toxoplasmosis. PMID:8981323

  14. Disseminated cerebral hemorrhages as unusual manifestation of toxoplasmic encephalitis in AIDS

    Microsoft Academic Search

    Peter Berlit; Octavian Popescu; Yvonne Weng; Rolf Malessa

    1996-01-01

    Hemorrhagic lesions are uncommon in central nervous system (CNS) toxoplasmosis. We report here the case of a patient with multiple cerebral hemorrhages as the initial manifestation of HIV infection due to CNS toxoplasmosis.

  15. 19 CFR 4.75 - Incomplete manifest; incomplete export declarations; bond.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ...manifest and all required shipper's export declarations have been filed with the port director: Albania Bulgaria Cambodia China, People's Republic of Cuba Czechoslovakia Estonia German Democratic Republic (Soviet Zone of Germany and...

  16. 19 CFR 4.75 - Incomplete manifest; incomplete export declarations; bond.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ...manifest and all required shipper's export declarations have been filed with the port director: Albania Bulgaria Cambodia China, People's Republic of Cuba Czechoslovakia Estonia German Democratic Republic (Soviet Zone of Germany and...

  17. Metaphor and Manifestation-Cross-Reality with Ubiquitous Sensor/Actuator Networks

    E-print Network

    Paradiso, Joseph A.

    Looks at how MIT Media Lab's Responsive Environments Group is exploring ways to bridge networked electronic sensors and human perception through "cross reality" implementations that render and manifest phenomena between ...

  18. 15 CFR 30.47 - Clearance or departure of carriers under bond on incomplete manifest.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ...Customs and Border Protection regulations citations, exclusion, and/or exemption legends...in this part and all required filing citations, exclusion, and/or exemption legends...the manifest and all required filing citations, exclusion, and/or exemption...

  19. 15 CFR 30.47 - Clearance or departure of carriers under bond on incomplete manifest.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ...Customs and Border Protection regulations citations, exclusion, and/or exemption legends...in this part and all required filing citations, exclusion, and/or exemption legends...the manifest and all required filing citations, exclusion, and/or exemption...

  20. 15 CFR 30.47 - Clearance or departure of carriers under bond on incomplete manifest.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ...Customs and Border Protection regulations citations, exclusion, and/or exemption legends...in this part and all required filing citations, exclusion, and/or exemption legends...the manifest and all required filing citations, exclusion, and/or exemption...

  1. 15 CFR 30.47 - Clearance or departure of carriers under bond on incomplete manifest.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ...Customs and Border Protection regulations citations, exclusion, and/or exemption legends...in this part and all required filing citations, exclusion, and/or exemption legends...the manifest and all required filing citations, exclusion, and/or exemption...

  2. The Effects of Manifest Anxiety on the Reading Achievement of Fifth Grade Students

    ERIC Educational Resources Information Center

    Merryman, Edward P.

    1974-01-01

    The purpose of this study was to determine what effects manifest anxiety has on the achievement of selected reading variables (also tasks or skills) of children who, according to their CMAS scores, vary in general drive or anxiety levels. (Author)

  3. Concurrent Validity of What I Think and Feel: The Revised Children's Manifest Anxiety Scale.

    ERIC Educational Resources Information Center

    Reynolds, Cecil R.

    1980-01-01

    In support of the construct validity of the revised scale, a high correlation was observed between the revised Children's Manifest Anxiety Scale (CMAS) and the trait anxiety inventory, whereas there was no significant correlation with the state anxiety inventory. (Author)

  4. 78 FR 76153 - Agency Information Collection Activities: Entry and Manifest of Merchandise Free of Duty, Carrier...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-12-16

    ...DEPARTMENT OF HOMELAND SECURITY U.S. Customs and Border Protection Agency Information...Manifest of Merchandise Free of Duty, Carrier's Certificate and Release AGENCY: U.S. Customs and Border Protection, Department of...

  5. 76 FR 62086 - Agency Information Collection Activities: Entry and Manifest of Merchandise Free of Duty, Carrier...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-10-06

    ...DEPARTMENT OF HOMELAND SECURITY U.S. Customs and Border Protection Agency Information...Manifest of Merchandise Free of Duty, Carrier's Certificate and Release AGENCY: U.S. Customs and Border Protection (CBP),...

  6. 76 FR 76983 - Agency Information Collection Activities: Entry and Manifest of Merchandise Free of Duty, Carrier...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-12-09

    ...DEPARTMENT OF HOMELAND SECURITY U.S. Customs and Border Protection Agency Information...Manifest of Merchandise Free of Duty, Carrier's Certificate and Release AGENCY: U.S. Customs and Border Protection, Department of...

  7. 78 FR 52958 - Announcement of Test Concerning Manifesting and Entry of Residue Found in Instruments of...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-08-27

    ...Test Concerning Manifesting and Entry of Residue Found in Instruments of International...modified ruling, HQ H026715, requires that residue within containers be ``classified...new type of entry designed to capture residue in containers that will be cleaned...

  8. Extrahepatic Manifestations and Autoantibodies in Patients with Hepatitis C Virus Infection

    PubMed Central

    Himoto, Takashi; Masaki, Tsutomu

    2012-01-01

    Patients with chronic hepatitis C virus (HCV) infection frequently have many extrahepatic manifestations, as persistent HCV infection often triggers lymphoproliferative disorders and metabolic abnormalities. These manifestations primarily include autoimmune disorders such as cryoglobulinemia, Sjögren's syndrome, and autoimmune thyroid disorders. It has been well established that chronic HCV infection plays important roles in the production of non-organ-specific autoantibodies, including antinuclear antibodies and smooth muscle antibodies, and organ-specific autoantibodies such as thyroid autoantibodies. However, the clinical significance of autoantibodies associated with the extrahepatic manifestations caused by HCV infection has not been fully recognized. In this paper, we mainly focus on the relationship between extrahepatic manifestations and the emergence of autoantibodies in patients with HCV infection and discuss the clinical relevance of the autoantibodies in the extrahepatic disorders. PMID:22988469

  9. 19 CFR 4.64 - Electronic passenger and crew member departure manifests.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ...approved by CBP. If the transmission is in US EDIFACT format, the passenger manifest...document type (e.g., P = passport; A = alien registration card); (vii) Passport...date, if a passport is required; (x) Alien registration number, where...

  10. 19 CFR 4.7b - Electronic passenger and crew arrival manifests.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ...approved by CBP. If the transmission is in US EDIFACT format, the passenger manifest...document type (e.g., P = passport, A = alien registration); (viii) Passport number...if a passport is required; (xi) Alien registration number, where...

  11. 19 CFR 4.7b - Electronic passenger and crew arrival manifests.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ...approved by CBP. If the transmission is in US EDIFACT format, the passenger manifest...document type (e.g., P = passport, A = alien registration); (viii) Passport number...if a passport is required; (xi) Alien registration number, where...

  12. 19 CFR 4.64 - Electronic passenger and crew member departure manifests.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ...approved by CBP. If the transmission is in US EDIFACT format, the passenger manifest...document type (e.g., P = passport; A = alien registration card); (vii) Passport...date, if a passport is required; (x) Alien registration number, where...

  13. 19 CFR 4.7b - Electronic passenger and crew arrival manifests.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ...approved by CBP. If the transmission is in US EDIFACT format, the passenger manifest...document type (e.g., P = passport, A = alien registration); (viii) Passport number...if a passport is required; (xi) Alien registration number, where...

  14. 19 CFR 4.64 - Electronic passenger and crew member departure manifests.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ...approved by CBP. If the transmission is in US EDIFACT format, the passenger manifest...document type (e.g., P = passport; A = alien registration card); (vii) Passport...date, if a passport is required; (x) Alien registration number, where...

  15. Longitudinally extensive transverse myelitis as presenting manifestation of small cell carcinoma lung

    PubMed Central

    Jain, Rajendra Singh; Gupta, Pankaj Kumar; Agrawal, Rakesh; Tejwani, Shankar; Kumar, Sunil

    2015-01-01

    Longitudinally extensive transverse myelitis (LETM) is an unusual manifestation of systemic malignancy. It has been mainly reported with lung cancers and lymphoproliferative malignancy. LETM in systemic malignancy can be caused by either intramedullary metastases or paraneoplastic syndrome. We report an unusual case of small-cell carcinoma lung, who presented with LETM without having any cardinal manifestations of lung malignancy. This case report highlights the important differentiating features between intramedullary metastasis and paraneoplastic syndrome. PMID:25988082

  16. Management of extraintestinal manifestations and other complications of inflammatory bowel disease

    Microsoft Academic Search

    Edward V. Loftus

    2004-01-01

    The past 18 months have seen many studies of the prevalence, pathogenesis, and treatment of the extraintestinal manifestations\\u000a of inflammatory bowel disease (IBD). Inhibitors of tumor necrosis factor alpha have shown effectiveness in randomized trials\\u000a for the treatment of spondyloarthropathies and ocular manifestations. Open-label studies suggest that these agents may be\\u000a effective for pyoderma gangrenosum as well. The epidemiology of

  17. Manifestations of juvenile polyposis syndrome in SMAD4 mutation carriers of a kindred.

    PubMed

    Schwetz, Verena; Uhrig, Sabine; Spuller, Ekkehard; Deutschmann, Andrea; Högenauer, Christoph

    2012-08-01

    The autosomal dominantly inherited juvenile polyposis syndrome (JPS) leads to the development of multiple hamartomatous polyps in the gastrointestinal tract and is a precancerous condition. In a large family with a newly identified SMAD4 mutation (c.543delC), we describe the clinical manifestations of JPS. Nine affected SMAD4 mutation-positive family members were screened and treated for manifestations of JPS. Two family members were symptomatic at the time of diagnosis; seven were asymptomatic - independent of the severity of the manifestation. Each mutation carrier presented with colonic juvenile polyps, seven out of nine with additional gastric manifestations. One asymptomatic patient had early gastric cancer; another patient had a villous adenoma with high-grade intraepithelial neoplasia in the colon. Three patients had biliary lesions including a bile duct hamartoma in one and gallbladder polyps in two. Three patients had gastrointestinal vascular malformations. All mutation carriers were affected by JPS. Interestingly, the manifestations and their severity differed considerably between the patients, suggesting secondary factors influencing JPS manifestations such as Helicobacter pylori infection. PMID:22617360

  18. Do evolutionary constraints on thermal performance manifest at different organizational scales?

    PubMed

    Phillips, B L; Llewelyn, J; Hatcher, A; Macdonald, S; Moritz, C

    2014-12-01

    The two foremost hypotheses on the evolutionary constraints on an organism's thermal sensitivity—the hotter-is-better expectation, and the specialist-generalist trade-off—have received mixed support from empirical studies testing for their existence. Could these conflicting results reflect confusion regarding the organizational level (i.e. species > population > individual) at which these constraints should manifest? We propose that these evolutionary constraints should manifest at different organizational levels because of differences in their underlying causes and requirements. The hotter-is-better expectation should only manifest across separate evolutionary units (e.g. species, populations), and not within populations. The specialist-generalist trade-off, by contrast, should manifest within as well as between separate evolutionary units. We measured the thermal sensitivity of sprint performance for 440 rainforest sun skinks (Lampropholis coggeri) representing 10 populations, and used the resulting performance curves to test for evidence for the hypothesized constraints at two organizational levels: (i) across populations and (ii) within populations. As predicted, the hotter-is-better expectation was evident only at the across-population level, whereas the specialist-generalist trade-off was evident within, as well as across, populations. Our results suggest that, depending on the processes that drive them, evolutionary constraints can manifest at different organizational levels. Consideration of these underlying processes, and the organizational level at which a constraint should manifest, may help resolve conflicting empirical results. PMID:25403471

  19. Nonopportunistic Neurologic Manifestations of the Human Immunodeficiency Virus: An Indian Study

    PubMed Central

    Deshpande, Alaka K.; Patnaik, Mrinal M.

    2005-01-01

    Context HIV-1 is a neurotropic virus. In a resource-limited country such as India, large populations of affected patients now have access to adequate chemoprophylaxis for opportunistic infections (OIs), allowing them to live longer. Unfortunately the poor availability of highly active antiretroviral therapy (HAART) has allowed viral replication to proceed unchecked. This has resulted in an increase in the debilitating neurologic manifestations directly mediated by the virus. Objective The main objective of this study was to identify and describe in detail the direct neurologic manifestations of HIV-1 in antiretroviral treatment (ART)-naive, HIV-infected patients (excluding the neurologic manifestations produced by opportunistic pathogens). Design Three hundred successive cases of HIV-1 infected, ART-naive patients with neurologic manifestations were studied over a 3-year period. Each case was studied in detail to identify and then exclude manifestations due to opportunistic pathogens. The remaining cases were then analyzed specially in regard to their occurrence and the degree of immune suppression (CD4+ cell counts). Setting and Patients The study was carried out in an apex, tertiary, referral care center for HIV/AIDS in India. All patients were admitted for a detailed analysis. No interventions were carried out, as this was an observational study. Results Of the 300 cases, 67 (22.3%) had neurologic manifestations due to the direct effects of HIV-1. The HIV infection involved the neuroaxis at all levels. The distribution of cases showed that the region most commonly involved was the brain (50.7%). The manifestations included stroke syndromes (29.8%), demyelinating illnesses (5.9%), AIDS dementia complex (5.9%), and venous sinus thrombosis (4.4%). The other manifestations seen were peripheral neuropathies (35.8% of cases), spinal cord pathologies (5.9% of cases), radiculopathies (4.4% of cases), and a single case of myopathy. The onset of occurrence of these diseases and their progression were then correlated with the CD4+ cell counts. Conclusions HIV infection is responsible for a large number of nonopportunistic neurologic manifestations that occur across a large immune spectrum. During the early course of the disease, the polyclonal hypergammaglobulinemia induced by the virus results in demyelinating diseases of the central- and peripheral nervous systems (CNS and PNS). As the HIV infection progresses, the direct toxic effects of the virus unfold, directly damaging the CNS and PNS, resulting in protean clinical manifestations. PMID:16614624

  20. Nonopportunistic Neurologic Manifestations of the Human Immunodeficiency Virus: An Indian Study

    PubMed Central

    Patnaik, Mrinal M

    2005-01-01

    Context HIV-1 is a neurotropic virus. In a resource-limited country such as India, large populations of affected patients now have access to adequate chemoprophylaxis for opportunistic infections (OIs), allowing them to live longer. Unfortunately the poor availability of highly active antiretroviral therapy (HAART) has allowed viral replication to proceed unchecked. This has resulted in an increase in the debilitating neurologic manifestations directly mediated by the virus. Objective The main objective of this study was to identify and describe in detail the direct neurologic manifestations of HIV-1 in antiretroviral treatment (ART)-naive, HIV-infected patients (excluding the neurologic manifestations produced by opportunistic pathogens). Design Three hundred successive cases of HIV-1 infected, ART-naive patients with neurologic manifestations were studied over a 3-year period. Each case was studied in detail to identify and then exclude manifestations due to opportunistic pathogens. The remaining cases were then analyzed specially in regard to their occurrence and the degree of immune suppression (CD4+ cell counts). Setting and Patients The study was carried out in an apex, tertiary, referral care center for HIV/AIDS in India. All patients were admitted for a detailed analysis. No interventions were carried out, as this was an observational study. Results Of the 300 cases, 67 (22.3%) had neurologic manifestations due to the direct effects of HIV-1. The HIV infection involved the neuroaxis at all levels. The distribution of cases showed that the region most commonly involved was the brain (50.7%). The manifestations included stroke syndromes (29.8%), demyelinating illnesses (5.9%), AIDS dementia complex (5.9%), and venous sinus thrombosis (4.4%). The other manifestations seen were peripheral neuropathies (35.8% of cases), spinal cord pathologies (5.9% of cases), radiculopathies (4.4% of cases), and a single case of myopathy. The onset of occurrence of these diseases and their progression were then correlated with the CD4+ cell counts. Conclusion HIV infection is responsible for a large number of nonopportunistic neurologic manifestations that occur across a large immune spectrum. During the early course of the disease, the polyclonal hypergammaglobulinemia induced by the virus results in demyelinating diseases of the central- and peripheral nervous systems (CNS and PNS). As the HIV infection progresses, the direct toxic effects of the virus unfold, directly damaging the CNS and PNS, resulting in protean clinical manifestations. PMID:19825135

  1. Revisiting the Molecular Mechanism of Neurological Manifestations in Antiphospholipid Syndrome: Beyond Vascular Damage

    PubMed Central

    Carecchio, M.; Cantello, R.; Comi, C.

    2014-01-01

    Antiphospholipid syndrome (APS) is a multiorgan disease often affecting the central nervous system (CNS). Typically, neurological manifestations of APS include thrombosis of cerebral vessels leading to stroke and requiring prompt initiation of treatment with antiplatelet drugs or anticoagulant therapy. In these cases, alterations of the coagulation system at various levels caused by multiple effects of antiphospholipid antibodies (aPL) have been postulated to explain the vascular damage to the CNS in APS. However, several nonvascular neurological manifestations of APS have progressively emerged over the past years. Nonthrombotic, immune-mediated mechanisms altering physiological basal ganglia function have been recently suggested to play a central role in the pathogenesis of these manifestations that include, among others, movement disorders such as chorea and behavioral and cognitive alterations. Similar clinical manifestations have been described in other autoimmune CNS diseases such as anti-NMDAR and anti-VGCK encephalitis, suggesting that the spectrum of immune-mediated basal ganglia disorders is expanding, possibly sharing some pathophysiological mechanisms. In this review, we will focus on thrombotic and nonthrombotic neurological manifestations of APS with particular attention to immune-mediated actions of aPL on the vascular system and the basal ganglia. PMID:24741580

  2. [Mitral valve replacement for a manifesting carrier of duchenne muscular dystrophy].

    PubMed

    Ishii, Hirohito; Nakamura, Kunihide; Nagahama, Hiroyuki; Matsuyama, Masakazu; Endo, Jouji; Nishimura, Masanori

    2015-02-01

    Duchenne muscular dystrophy (DMD) is an X-linked disease, but female carriers infrequently have some symptoms, who are called manifesting carriers. Here we report a case of a manifesting carrier of DMD with skeletal muscle weakness and cardiac abnormalities such as deterioration of cardiac function and left ventricular dilatation, who successfully underwent cardiac surgery. A 79-year-old female with acute heart failure for severe mitral regurgitation was admitted to our hospital. Surgical replacement of the mitral valve was performed under general anesthesia with intravenous anesthetics and non-depolarizing muscle relaxant. Cardiac surgery on a manifesting carrier of DMD is rare and requires a careful preoperative assessment of the heart function and anesthetic management. PMID:25743350

  3. Orthopedic manifestations in patients with mucopolysaccharidosis type II (Hunter syndrome) enrolled in the Hunter Outcome Survey

    PubMed Central

    Link, Bianca; de Camargo Pinto, Louise Lapagesse; Giugliani, Roberto; Wraith, James Edmond; Guffon, Nathalie; Eich, Elke; Beck, Michael

    2010-01-01

    Mucopolysaccharidosis type II (MPS II or Hunter syndrome) is a rare, inherited disorder caused by deficiency of the lysosomal enzyme iduronate-2-sulfatase. As a result of this deficiency, glycosaminoglycans accumulate in lysosomes in many tissues, leading to progressive multisystemic disease. The cardiopulmonary and neurological problems associated with MPS II have received considerable attention. Orthopedic manifestations are common but not as well characterized. This study aimed to characterize the prevalence and severity of orthopedic manifestations of MPS II and to determine the relationship of these signs and symptoms with cardiovascular, pulmonary and central nervous system involvement. Orthopedic manifestations of MPS II were studied using cross-sectional data from the Hunter Outcome Survey (HOS). The HOS is a global, physician-led, multicenter observational database that collects information on the natural history of MPS II and the long-term safety and effectiveness of enzyme replacement therapy. As of January 2009, the HOS contained baseline data on joint range of motion in 124 males with MPS II. In total, 79% of patients had skeletal manifestations (median onset, 3.5 years) and 25% had abnormal gait (median onset, 5.4 years). Joint range of motion was restricted for all joints assessed (elbow, shoulder, hip, knee and ankle). Extension was the most severely affected movement: the exception to this was the shoulder. Surgery for orthopedic problems was rare. The presence of orthopedic manifestations was associated with the presence of central nervous system and pulmonary involvement, but not so clearly with cardiovascular involvement. Orthopedic interventions should be considered on an individual-patient basis. Although some orthopedic manifestations associated with MPS II may be managed routinely, a good knowledge of other concurrent organ system involvement is essential. A multidisciplinary approach is required. PMID:21808707

  4. Diagnosis and clinical manifestations of calcium pyrophosphate and basic calcium phosphate crystal deposition diseases.

    PubMed

    Ea, Hang-Korng; Lioté, Frédéric

    2014-05-01

    Basic calcium phosphate and pyrophosphate calcium crystals are the 2 main calcium-containing crystals that can deposit in all skeletal tissues. These calcium crystals give rise to numerous manifestations, including acute inflammatory attacks that can mimic alarming and threatening differential diagnoses, osteoarthritis-like lesions, destructive arthropathies, and calcific tendinitis. Awareness of uncommon localizations and manifestations such as intraspinal deposition (eg, crowned dens syndrome, tendinitis of longus colli muscle, massive cervical myelopathy compression) prevents inappropriate procedures and cares. Coupling plain radiography, ultrasonography, computed tomography, and synovial fluid analysis allow accurate diagnosis by directly or indirectly identifying the GRAAL of microcrystal-related symptoms. PMID:24703344

  5. Visual manifestations of occipital lobe infarction in three patients on a geriatric psychiatry unit.

    PubMed

    La Mancusa, J C; Cole, A R

    1988-01-01

    The authors present three cases of hospitalized patients on a geriatric psychiatry floor who were found to have previously undiagnosed occipital lobe infarctions associated with visual manifestations. The manifestations discussed are visual field defects, visual hallucinations, and color anomia. The incidence of undiagnosed occipital lobe infarctions and the contribution of these infarctions to visual perception changes in this patient population are unknown. The authors suggest that for patients who present with visual perception changes, a high index of suspicion for occipital lobe infarction should be maintained. Careful visual field testing is an essential part of the admitting work-up for hospitalized geriatric patients. PMID:3252891

  6. [Psychotic manifestations in childhood. III. Abnormal reactions to adventures (author's transl)].

    PubMed

    Asperger, H; Groh, C; Rosenmayr, F W

    1975-01-01

    In childhood psychotic manifestations are in most cases due to somatic diseases, but may be psychogenic. Abnormal reactions to adventures especially to very abnormal occurances--for instance in families with psychotic members--lead to psychotic symptoms. The appearence of those psychotic manifestations is influenced by the age of the child, by his hysterical, anancastic or austistic character and the pathological features (paranoic or depressive) of the inducing person. Usually hospitalization for diagnostic and therapeutical reasons is necessary. In case of induced psychoses separation must be long enough and has to be combined with treatment of the primary patient. PMID:1196651

  7. Pachymeningitis as a manifestation of ANCA-associated vasculitis: a care report and literature review

    PubMed Central

    Li, Sheyu; Tang, Honghu; Rong, Xia; Huang, Xiangyang; Li, Qianrui

    2015-01-01

    Pachymeningitis is a rare cause of headache characterized by dura mater thickening with various origins. We present a 67-year-old male with cranial and spinal pachymeningitis and MPO-ANCA-associated vasculitis, which is considered as the cause of pachymeningitis of this patient. After revision of other 32 reported cases, our study suggested cranial and spinal pachymeningitis could be a manifestation of ANCA-associated vasculitis. Thus, it is important to screen other manifestations of ANCA-associated vasulitis as soon as pachymeningitis was suspected.

  8. [Acquired ichthyosis and livedoid palmoplantar keratoderma: two unusual skin manifestations of systemic lupus erythematosus].

    PubMed

    Biver-Dalle, C; Gil, H; Méaux-Ruault, N; Mermet-Ginet, I; Aubin, F; Humbert, P; Magy-Bertrand, N

    2012-03-01

    Acquired ichthyosis and livedoid palmoplantar keratoderma have rarely been described in systemic lupus erythematosus (SLE). In the first case, a 51-year-old man presented with generalized acquired ichthyosis associated with renal glomurelosclerosis and neurolupus. rituximab allowed total resolution of the acquired ichthyosis. A livedoid palmoplantar keratoderma was observed in a 45-year-old woman as the initial clinical manifestation of a systemic lupus erythematosus associated with Raynaud's phenomenon, photosensitivity and inflammatory polyarthralgia. Although the pathophysiology is not well-defined, these unusual cutaneous manifestations should be considered by physicians when diagnosis of SLE is suspected. PMID:21429634

  9. Ocular Manifestations of Systemic Lupus Erythematosus: A Review of the Literature

    PubMed Central

    Palejwala, Neal V.; Walia, Harpreet S.; Yeh, Steven

    2012-01-01

    About one-third of patients suffering from systemic lupus erythematosus have ocular manifestations. The most common manifestation is keratoconjunctivitis sicca. The most vision threatening are retinal vasculitis and optic neuritis/neuropathy. Prompt diagnosis and treatment of eye disease is paramount as they are often associated with high levels of systemic inflammation and end-organ damage. Initial management with high-dose oral or IV corticosteroids is often necessary. Multiple “steroid-sparing” treatment options exist with the most recently studied being biologic agents. PMID:22811887

  10. Progress 44 Manifest (Partial) USOS (U.S. On-Orbit Segment) Cargo

    E-print Network

    Effect on Astronauts (ALTEA-GAP) hardware Anomalous Long Term Effect on Astronauts Shield Proactive monitoring in Columbus laboratory Portable Pulmonary Function System (PPFS) for ESA Portable PFS maintenance items for (preventive) maintenance on lung function experiment equipment #12;Progress 44 Manifest

  11. Clinical and immunological manifestations in 134 Puerto Rican patients with systemic lupus erythematosus.

    PubMed

    Vilá, L M; Mayor, A M; Valentín, A H; García-Soberal, M; Vilá, S

    1999-01-01

    To gain a better understanding of systemic lupus erythematosus (SLE) in Puerto Ricans we studied the clinical and serologic manifestations in a cohort of 134 patients. The female to male ratio was 18:1. Mean age at diagnosis was 32 +/- 12 y. The mean duration of disease and follow-up were 7.4 +/- 6.0 and 5.8 +/- 6.0 years respectively. Mortality was 3%. Photosensitivity (76.9%) and malar rash (71.9%) were the most common clinical manifestations. Arthritis was observed in 67.5% of patients. Anemia was seen in 67.2% of patients, but only 12.7% had autoimmune hemolytic anemia. Leukopenia (41.8%) and lymphopenia (64.9%) were also common. Serositis was observed in only 28%. Severe kidney damage such as nephrotic syndrome (14.2%) or renal failure (4%) was infrequent. Cardiovascular (12.7%) and neurologic (9.0%) manifestations were also uncommon. Antinuclear antibodies (ANA) were detected in 93.3%, anti-dsDNA antibodies in 54.5%, anti-Ro antibodies in 30.1% and anti-La antibodies in 14.2%. Low C3 and low C4 were observed in 38.3% and 35.7% respectively. This study suggests that Puerto Ricans with SLE present a mild form of disease predominantly manifested by cutaneous, musculoskeletal and hematologic involvement, but low prevalence of major organ damage and low mortality. PMID:10413206

  12. Low early-life social class leaves a biological residue manifested by decreased glucocorticoid and increased

    E-print Network

    Meagher, Mary

    subjects with low early-life SES, there was significant up-regulation of genes bearing response elementsLow early-life social class leaves a biological residue manifested by decreased glucocorticoid of life. One mechanistic hypoth- esis for this phenomenon suggests that social adversity in early life

  13. Nonlinear effects in transonic flutter with emphasis on manifestations of limit cycle oscillations

    Microsoft Academic Search

    G. Schewe; H. Mai; G. Dietz

    2003-01-01

    This paper presents flutter and forced oscillation experiments in a transonic wind tunnel. For an aeroelastic supercritical 2-D airfoil configuration we studied typical transonic phenomena in as pure a form as possible. Various manifestations of small-amplitude limit cycle oscillations were observed for different flow conditions as well as coexisting limit cycles. We demonstrated how very small control forces were sufficient

  14. The Manifest Association Structure of the Single-Factor Model: Insights from Partial Correlations

    ERIC Educational Resources Information Center

    Salgueiro, Maria de Fatima; Smith, Peter W. F.; McDonald, John W.

    2008-01-01

    The association structure between manifest variables arising from the single-factor model is investigated using partial correlations. The additional insights to the practitioner provided by partial correlations for detecting a single-factor model are discussed. The parameter space for the partial correlations is presented, as are the patterns of…

  15. Human-computer interaction in information retrieval: nature and manifestations of feedback

    Microsoft Academic Search

    Amanda Spink; Tefko Saracevic

    1998-01-01

    This study develops a theoretical framework for expressing the nature of feedback as a critical process in interactive information retrieval (IR). Feedback concepts from cybernetics and social sciences perspectives are used to develop a concept of informational feedback applicable to IR. Models from human-computer interaction and interactive IR are then adapted as a framework for studying the manifestations of feedback

  16. Neurofibromatosis and fibrous dysplasia manifesting in the same patient: a rare case report.

    PubMed

    Sujatha, Sampangi; Jatti, Deepa

    2015-02-01

    Neurofibromatosis and fibrous dysplasia show the presence of café-au-lait spots, bone lesions, and endocrinopathies. There has been speculation whether neurofibromatosis and fibrous dysplasia are different manifestations of the same disease or if these conditions are in some way related. We provide a case of whether neurofibromatosis and fibrous dysplasia complicated by hyperparathyroidism and osteoporosis. PMID:24000123

  17. The manifestations and natural history of spondylo-epi-metaphyseal dysplasia with joint laxity.

    PubMed

    Beighton, P; Gericke, G; Kozlowski, K; Grobler, L

    1984-10-01

    Spondylo-epi-metaphyseal dysplasia with joint laxity (SEMDJL) is characterized by severe dwarfism, articular hypermobility and progressive spinal malalignment. The clinical manifestations of 18 affected persons in 13 families of the Afrikans-speaking community of South Africa have been analysed and it has become evident that survival into adulthood is unusual. SEMDJL is inherited as an autosomal recessive trait. PMID:6499247

  18. ERP Manifestations of Processing Printed Words at Different Psycholinguistic Levels: Time Course and Scalp Distribution

    Microsoft Academic Search

    S. Bentin; Y. Mouchetant-Rostaing; M. H. Giard; J. F. Echallier; J. Pernier

    1999-01-01

    The aim of the present study was to examine the time course and scalp distribution of electrophysiological manifestations of the visual word recognition mechanism. Event-related potentials (ERPs) elicited by visually presented lists of words were recorded while subjects were involved in a series of oddball tasks. The distinction between the designated target and nontarget stimuli was manipulated to induce a

  19. Rle de Rhizoctonia solani Khn dans la manifestation de certains symptmes de verse parasitaire du mas

    E-print Network

    Paris-Sud XI, Université de

    Rôle de Rhizoctonia solani Kühn dans la manifestation de certains symptômes de verse parasitaire du, on constate la présence très fréquente de Rhizoctonia solani. Des essais d'inoculation au champ à différentes Importance of Rhizoctonia solani Kfhn in some lodging symptoms on maize. In Western France (Vendee area

  20. Attitudes and InactionA Case Study of the Manifest Demographics of Urban Water Conservation

    Microsoft Academic Search

    Miguel De Oliver

    1999-01-01

    The public’s disposition with respect to conservation is predominantly ascertained by surveys. Rarely are these attitudes contrasted directly against manifested behavior from an independent source. This article analyzes the response to a residential urban water conservation program by using municipal water consumption data at the census tract level in San Antonio, Texas. A selection of demographic variables (i.e., income, education,

  1. Brain metastasis as the first manifestation of small cell lung cancer in a female adolescent

    Microsoft Academic Search

    Li Hui; Yao Li Qing; Chen Zi Qian; Lin Yu Ning; Zheng Zhi Yong

    2011-01-01

    Small cell lung cancer is commonly regarded as an old age related disease. Although the brain is a common site of metastasis for this type of lung cancer, the first manifestation is rarely shown from the metastasis site. In this paper, we report a rare case of 19-year-old female patient with small cell lung cancer whose initial presentation was a

  2. Managerial Power Bases and Subordinates' Manifest Needs as Influences on Psychological Climate

    Microsoft Academic Search

    Edward A. Ward

    1998-01-01

    This study investigated the-relative and interactive effects of manifest needs and managerial power bases as explanatory variables of psychological climate. Previously validated measures of the constructs were completed by 420 full-time employees in the United States of America. Using the dimensions of psychological climate as the dependent variables denoting managerial effectiveness, stepwise multiple regression analyses with the managerial power bases

  3. Quantum manifestations of classical stochasticity in the wave functions of quadrupole vibrations of nuclei

    SciTech Connect

    Bolotin, Yu.L.; Gonchar, V.Yu.; Tarasov, V.N. [National Research Center Khar`kov Physicotechnical Institute (Ukraine)

    1995-09-01

    Quantum manifestations of classical stochasticity in the structure of the stationary wave functions of highly excited states of the Hamiltonian of quadrupole vibrations are studied. Correlations between the structure of the wave functions and the type of classical motion are found in the regularity-chaos-regularity transition. 28 refs., 8 figs., 3 tabs.

  4. Cobalamin deficiency as an extra intestinal manifestation of Helicobacter pylori infection

    PubMed Central

    Andrès, Emmanuel

    2015-01-01

    We read with great interest the excellent review by Wong et al on extra intestinal manifestations of Helicobacter pylori (H. pylori) infection published in the journal. This is a well-documented and structured review. However, I believe that Wong et al failed to report the relationship between H. pylori infection and cobalamin. PMID:25805959

  5. Manifest: A computer program for 2-D flow modeling in Stirling machines

    NASA Technical Reports Server (NTRS)

    Gedeon, David

    1989-01-01

    A computer program named Manifest is discussed. Manifest is a program one might want to use to model the fluid dynamics in the manifolds commonly found between the heat exchangers and regenerators of Stirling machines; but not just in the manifolds - in the regenerators as well. And in all sorts of other places too, such as: in heaters or coolers, or perhaps even in cylinder spaces. There are probably nonStirling uses for Manifest also. In broad strokes, Manifest will: (1) model oscillating internal compressible laminar fluid flow in a wide range of two-dimensional regions, either filled with porous materials or empty; (2) present a graphics-based user-friendly interface, allowing easy selection and modification of region shape and boundary condition specification; (3) run on a personal computer, or optionally (in the case of its number-crunching module) on a supercomputer; and (4) allow interactive examination of the solution output so the user can view vector plots of flow velocity, contour plots of pressure and temperature at various locations and tabulate energy-related integrals of interest.

  6. Exercise intolerance and systemic manifestations of pulmonary emphysema in a mouse model

    Microsoft Academic Search

    Lars Lüthje; Tobias Raupach; Hellmuth Michels; Bernhard Unsöld; Gerd Hasenfuss; Harald Kögler; Stefan Andreas

    2009-01-01

    BACKGROUND: Systemic effects of chronic obstructive pulmonary disease (COPD) significantly contribute to severity and mortality of the disease. We aimed to develop a COPD\\/emphysema model exhibiting systemic manifestations of the disease. METHODS: Female NMRI mice were treated 5 times intratracheally with porcine pancreatic elastase (emphysema) or phosphate-buffered saline (control). Emphysema severity was quantified histologically by mean linear intercept, exercise tolerance

  7. Therapy Insight: the recognition and treatment of retinal manifestations of systemic vasculitis

    Microsoft Academic Search

    Petros Aristodemou; Miles Stanford

    2006-01-01

    A variety of retinal signs can occur in patients who have systemic vasculitides, or who experience complications of these diseases or their treatment. Although treatment of these retinal manifestations is usually the treatment of the systemic disease, specific treatment is occasionally indicated to preserve vision. The more prevalent of the systemic vasculitides are giant cell arteritis, polyarteritis nodosa, Wegener's granulomatosis,

  8. Clinical manifestations of Relapsing Polychondritis: case report Manifestaciones clínicas en Policondritis Recurrente: relato de un caso

    Microsoft Academic Search

    Maria Alves; João Batista FerreiraV

    The Relapsing Polychondritis - RP is a rare systemic affection, of immunological nature, characterize by an inflammatory process that affects cartilaginous structures, upper airway and peripheral articulation. The diagnosis is basically clinical and the otorhinolaryngological complications can be the initial manifestations of the illness. Having in view the rarity of the disease, it was objectified to describe a PR case

  9. Change in the Manifestation of Overt Aggression during Early Adolescence: Gender and Ethnicity

    ERIC Educational Resources Information Center

    Kim, Sangwon; Kamphaus, Randy W.; Orpinas, Pamela; Kelder, Steve H.

    2010-01-01

    This study examined how the manifestation of overt aggression changes during early adolescence using Hierarchical Linear Modeling. The distinct courses of physical and verbal aggression identified in this study provide support for developmental transformations in overt aggression, which would have been obscured unless aggression had been defined…

  10. A Longitudinal Study of Reactive Curiosity, Manifest Anxiety, and Achievement in Elementary School.

    ERIC Educational Resources Information Center

    Yost, Michael

    The study attempted to determine: (1) whether achievement in the early school years is related to anxiety and curiosity in later school years; and (2) whether anxiety and curiosity in early school years are related to achievement in later school years. The Stafford Achievement Test (SAT), the Children's Manifest Anxiety Scale (CMAS), and the…

  11. Manifest Destiny and Competing Voices on the Eve of the Cherokee Removal

    ERIC Educational Resources Information Center

    Chandler, Prentice T.

    2011-01-01

    Manifest Destiny, the idea that Providence guided the conquest and settlement of North America, is one of the most contested ideas in American culture and history. One's opinion about this central aspect of American mythology depends heavily on one's point of view. Exploring westward expansion and the Cherokee Trail of Tears with primary sources…

  12. Oral manifestations in HIV infection: fungal and bacterial infections, Kaposi's sarcoma

    Microsoft Academic Search

    Peter A. Reichart

    2003-01-01

    Oral candidiasis (OC) is a frequent oral manifestation of HIV infection, is a marker disease and occurs as a pseudomembranous, erythematous or rarely hyperplastic variant; angular cheilitis is also seen. Candida albicans is frequently isolated but other species such as C. krusei and C. dublienensis are emerging. Resistance against fluconazole is common. Bacterial oral infections are comparatively rare and are

  13. Chronic pain is a manifestation of the Ehlers-Danlos syndrome

    Microsoft Academic Search

    Anubha Sacheti; Judy Szemere; Bruce Bernstein; Triantafyllos Tafas; Neil Schechter; Petros Tsipouras

    1997-01-01

    The Ehlers-Danlos syndrome (EDS) is a group of heritable systemic disorders of connective tissue manifesting joint hypermobility, skin extensibility, and tissue fragility. Although the presence of pain has been documented in the various types of the EDS, its natural history, distribution, and management have not been defined. We conducted a structured interview in 51 individuals affected with different types of

  14. Treatment of intractable gastrointestinal manifestations of chronic granulomatous disease with cyclosporine

    Microsoft Academic Search

    Joel R. Rosh; Hope B. Tang; Lloyd Mayer; Gabriel Groisman; Sheeja K. Abraham; Alice Prince

    1995-01-01

    Gastrointestinal manifestations of chronic granulomatous disease of childhood include granulomatous inflammatory bowel disease. Severe colitis and perirectal disease developed in a 12-year-old boy with chronic granulomatous disease while he was receiving interferon gamma therapy. The boy had a deficiency of the 22 kd light chain of the cytochrome b heterodimer. After conventional medical therapy proved to be ineffective, a rapid

  15. Microscopic manifestation of the spin phase transition at filling factor 2/3

    E-print Network

    Loss, Daniel

    . 1a, and the system will undergo a first-order quantum phase transition to a fully polarized stateLETTERS Microscopic manifestation of the spin phase transition at filling factor 2/3 BASILE VERDENE polarizations1­4 . Recent photoluminescence measurements confirmed directly that indeed many of the fractions

  16. Superimposed Segmental Manifestation of Both Rare and Common Cutaneous Disorders: A New Paradigm

    Microsoft Academic Search

    R. Happle

    2009-01-01

    In autosomal dominant skin disorders, a superimposed mosaic involvement arranged in a linear or otherwise segmental pattern is sometimes noted. Molecular proof of such “type 2 segmental manifestation” has so far been provided in Hailey-Hailey disease and Cowden syndrome. A similar superimposed segmental involvement can be found in numerous common disorders with a polygenic background, such a psoriasis, lichen planus,

  17. Skin manifestations induced by TNF-alpha inhibitors in juvenile idiopathic arthritis.

    PubMed

    Pontikaki, Irene; Shahi, Edit; Frasin, Lucretia Adina; Gianotti, Raffaele; Gelmetti, Carlo; Gerloni, Valeria; Meroni, Pier Luigi

    2012-04-01

    The tumor necrosis factor alpha (TNF?) inhibitors have been used with good clinical results in the treatment of juvenile idiopathic arthritis (JIA). Anti TNF? therapy is generally well tolerated. Besides the site injection reactions, other various cutaneous manifestations have been encountered as adverse events. Here, we report four young patients receiving treatment with anti-TNF? (infliximab, adalimumab, and etanercept) for JIA developing different skin manifestations more than 1 year after the initiation of therapy. They underwent a dermatological exam. All four patients were ACR-Ped 30 responders to anti-TNF drugs. The first patient developed cutaneous vasculitis, the second one had lichen planus manifestations, while the third and the fourth developed psoriatic palmoplantar pustulosis accompanied by plaque-type psoriasis localized to the scalp. None of the patients had a personal or family history of dermatological diseases. In the first two patients, skin lesions healed with topical treatment after the discontinuation of anti-TNF agent, while psoriatic lesions did not resolve despite discontinuation of the drug and dermatological treatment. TNF inhibition can be both anti-inflammatory and pro-inflammatory. Cutaneous manifestations could be considered as a paradoxical adverse event of the anti-TNF-alpha treatment not only in rheumatoid arthritis but also in juvenile idiopathic arthritis. PMID:21403999

  18. Polymorphisms in STK17A gene are associated with systemic lupus erythematosus and its clinical manifestations.

    PubMed

    da Silva Fonseca, Andréia Maria; de Azevedo Silva, Jaqueline; Pancotto, João Alexandre Trés; Donadi, Eduardo Antônio; Segat, Ludovica; Crovella, Sergio; Sandrin-Garcia, Paula

    2013-09-25

    Systemic lupus erythematosus (SLE) is an autoimmune disorder with several clinical manifestations. SLE etiology has a strong genetic component, which plays a key role in disease's predisposition, as well as participation of environmental factors, such and UV light exposure. In this regard, we investigated whether polymorphisms in STK17A, a DNA repair related gene, encoding for serine/threonine-protein kinase 17A, are associated with SLE susceptibility. A total of 143 SLE patients and 177 healthy controls from Southern Brazil were genotyped for five STK17A TagSNPs. Our results indicated association of rs7805969 SNP (A and G/A genotype, OR=1.40 and OR=1.73, respectively) with SLE predisposition and the following clinical manifestations: arthritis, cutaneous and immunological alterations. When analyzing haplotypes distribution, we found association between TGGTC, TAGTC and AAGAT haplotypes and risk to develop SLE. When considering clinical manifestations, the haplotypes TGGTT and TAGTC were associated with protection against cutaneous alterations and the haplotype TAGTC to hematological alterations. We also observed association between SLE clinical manifestations and ethnicity, with the European-derived patients being more susceptible to cutaneous and hematological alterations. PMID:23860322

  19. Overview of common, rare and atypical manifestations of cutaneous lupus erythematosus and histopathological correlates.

    PubMed

    Obermoser, G; Sontheimer, R D; Zelger, B

    2010-08-01

    The skin is the second most frequently affected organ system in lupus erythematosus. Although only very rarely life threatening--an example is lupus erythematosus-associated toxic epidermal necrolysis--skin disease contributes disproportionally to disease burden in terms of personal and psychosocial wellbeing, vocational disability, and hence in medical and social costs. Since several manifestations are closely associated with the presence and activity of systemic lupus erythematosus, prompt and accurate diagnosis of cutaneous lupus erythematosus is essential. This review aims to cover common, rare, and atypical manifestations of lupus erythematosus-associated skin disease with a detailed discussion of histopathological correlates. Cutaneous lupus erythematosus covers a wide morphological spectrum well beyond acute, subacute and chronic cutaneous lupus erythematosus, which are commonly classified as lupus-specific skin disease. Other uncommon or less well-known manifestations include lupus erythematosus tumidus, lupus profundus, chilblain lupus, mucosal lupus erythematosus, and bullous lupus erythematosus. Vascular manifestations include leukocytoclastic and urticarial vasculitis, livedoid vasculopathy and livedo reticularis/ racemosa. Finally, we discuss rare presentations such as lupus erythematosus-related erythema exsudativum multiforme (Rowell syndrome), Kikuchi-Fujimoto disease, extravascular necrotizing palisaded granulomatous dermatitis (Winkelmann granuloma), and neutrophilic urticarial dermatosis. PMID:20693199

  20. Splenomegaly as a Primary Manifestation of Gaucher Disease in a Young Adult Woman

    Microsoft Academic Search

    Giuseppe Merra; Antonio Dal Lago; Roberta Ricci; Daniela Antuzzi; Giovanni Gasbarrini; Antonio Gasbarrini; Giovanni Ghirlanda

    2008-01-01

    Gaucher disease is the most common lysosomal storage disease. It is caused by the defective activity of acid ?-glucosidase, which results in the accumulation of lipid glucocerebroside in macrophages throughout the body. In this case report we describe the case of a young adult woman with splenomegaly as the primary manifestation of this pathology. This is a case of type

  1. Age Dependence of Clinical and Pathological Manifestations of Autoimmune Demyelination Implications for Multiple Sclerosis

    Microsoft Academic Search

    Mary E. Smith; Nancy L. Eller; Henry F. McFarland; Michael K. Racke; Cedric S. Raine

    A prominent feature of the clinical spectrum of mul- tiple sclerosis (MS) is its high incidence of onset in the third decade of life and the relative rarity of clinical manifestations during childhood and adolescence, features suggestive of age-related restriction of clini- cal expression. Experimental allergic encephalomy- elitis (EAE), a model of central nervous system (CNS) autoimmune demyelination with many

  2. Prevalence of oral and systemic manifestations in pediatric HIV cohorts with and without drug therapy.

    PubMed

    Jose, Renju; Chandra, Sharath; Puttabuddi, Jaishankar H; Vellappally, Sajith; Al Khuraif, Abul-Aziz A; Halawany, Hassan S; Abraham, Nimmi B; Jacob, Vimal; Hashim, Mohamed

    2013-09-01

    The prevalence of orofacial and systemic manifestations and their association with drug therapy in pediatric HIV patients is scarce in the literature. The aim of the study was to determine the prevalence of oro-facial and systemic manifestations in HIV sero-positive children with and without highly active antiretroviral therapy (HAART). The study population consisted of 100 pediatric HIV patients (n=47 on HAART and n=53 not on HAART). The majority of the children (n=56) had at least one or more oro-facial manifestation associated with HIV. Oral candidiasis was the most common oral finding present in the HAART (14/33) and non-HAART groups (19/33). Recurrent aphthous ulcers was the only significant oral finding, present more in the HAART group. The percentage of children with upper respiratory tract infection was also more in the HAART group. The other lesions which were found to be significant were seborrheic dermatitis, pulmonary tuberculosis and otitis media. There was no significant difference in the participants' oral findings based on CD4 counts in the HAART and non- HAART groups. The prevalence of oral and systemic manifestations is a persistent feature associated with pediatric HIV, though of moderate intensity in those using HAART and may vary according to individual immune status. PMID:24329176

  3. Thoracolumbar fracture with listhesis--an uncommon manifestation of child abuse

    Microsoft Academic Search

    Terry L. Levin; Walter E. Berdon; Ian Cassell; Netta M. Blitman

    2003-01-01

    Background. Thoracolumbar fracture with listhesis (FL) is an uncommon manifestation of child abuse (increasingly known as nonaccidental trauma), with only six prior reports in the literature. Objective. This article seeks to call attention to FL of the thoracolumbar spine in abused children and infants. Materials and methods. We reviewed plain films, CT and MR images in seven new cases of

  4. Identifying the Clusters within Nonmotor Manifestations in Early Parkinson's Disease by Using Unsupervised Cluster Analysis

    PubMed Central

    Yang, Hui-Jun; Kim, Young Eun; Yun, Ji Young; Kim, Han-Joon; Jeon, Beom Seok

    2014-01-01

    Background Classical and data-driven classifications of Parkinson's disease (PD) are based primarily on motor symptoms, with little attention being paid to the clustering of nonmotor manifestations. Methods Clinical data on demographic, motor and nonmotor features, including the Korean version of the sniffin' stick (KVSS) test results, and responses to the screening questionnaire of the nonmotor features were collected from 56 PD patients with disease onset within 3 years. Nonmotor subgroups were classified using unsupervised hierarchical cluster analysis (HCA). In addition to unsupervised HCA, we performed a cross-sectional analysis comparing the performance on the KVSS olfactory test with other nonmotor manifestations of the patients. Results Forty-nine patients (87.5%) had hyposmia based on the KVSS test. HCA suggested three nonmotor clusters for all PD patients and two nonmotor clusters in de novo PD patients, without a priori assumptions about the relatedness. In the cross-sectional analysis, dream-enactment behavior was more prevalent in patients with lower olfactory scores, implying impaired olfactory function (P?=?0.029 for all PD patients; P?=?0.046 for de novo PD patients). Conclusion We propose the existence of different clusters of nonmotor manifestations in early PD by using unsupervised hierarchical clustering. To our knowledge, this study is the first to report the identification of nonmotor subgroups based on unsupervised HCA of multiple nonmotor manifestations in the early stage of the disease. PMID:24643014

  5. Manifesting Destiny: A Land Education Analysis of Settler Colonialism in Jamestown, Virginia, USA

    ERIC Educational Resources Information Center

    McCoy, Kate

    2014-01-01

    Globally, colonization has been and continues to be enacted in the take-over of Indigenous land and the subsequent conversion of agriculture from diverse food and useful crops to large-scale monoculture and cash crops. This article uses a land education analysis to map the rise of the ideology and practices of Manifest Destiny in Virginia.…

  6. 19 CFR 123.9 - Explanation of a discrepancy in a manifest.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ...DEPARTMENT OF THE TREASURY CBP RELATIONS WITH CANADA AND MEXICO General Provisions § 123.9 Explanation of a discrepancy...vessel or vehicle arriving in the U.S. or the U.S. Virgin Islands who fails to present a manifest to Customs is liable...

  7. 19 CFR 123.9 - Explanation of a discrepancy in a manifest.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ...DEPARTMENT OF THE TREASURY CUSTOMS RELATIONS WITH CANADA AND MEXICO General Provisions § 123.9 Explanation of a discrepancy...vessel or vehicle arriving in the U.S. or the U.S. Virgin Islands who fails to present a manifest to Customs is liable...

  8. 19 CFR 123.9 - Explanation of a discrepancy in a manifest.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ...DEPARTMENT OF THE TREASURY CBP RELATIONS WITH CANADA AND MEXICO General Provisions § 123.9 Explanation of a discrepancy...vessel or vehicle arriving in the U.S. or the U.S. Virgin Islands who fails to present a manifest to Customs is liable...

  9. 19 CFR 123.9 - Explanation of a discrepancy in a manifest.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ...DEPARTMENT OF THE TREASURY CBP RELATIONS WITH CANADA AND MEXICO General Provisions § 123.9 Explanation of a discrepancy...vessel or vehicle arriving in the U.S. or the U.S. Virgin Islands who fails to present a manifest to Customs is liable...

  10. 19 CFR 123.9 - Explanation of a discrepancy in a manifest.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ...DEPARTMENT OF THE TREASURY CBP RELATIONS WITH CANADA AND MEXICO General Provisions § 123.9 Explanation of a discrepancy...vessel or vehicle arriving in the U.S. or the U.S. Virgin Islands who fails to present a manifest to Customs is liable...

  11. Dengue fever with hemorrhagic manifestations in travellers returning to Quebec from Asia

    PubMed Central

    Duperval, Raymond; Frost, Eric H; Artsob, Harvey

    1993-01-01

    Two cases of dengue fever with hemorrhagic manifestations were observed in 1990 and 1992 among travellers returning from Asia, while a third presented with classical dengue fever after the patient’s first trip to an endemic region. All experienced rash, thrombocytopenia and coagulation disorders and had flavivirus serology consistent with exposure to dengue virus. PMID:22346452

  12. The Tragedy of the Virtual Commons as Manifested in the Death of Blogs

    E-print Network

    Boyer, Edmond

    The Tragedy of the Virtual Commons as Manifested in the Death of Blogs John Lenarcic and Pradipta." It is advanced that the unfettered buildup of inactive blogs is leading to a surfeit of information which in a blog is postulated as being sufficient feedback from readers that leads to the emergence of an ongoing

  13. Group A Escherichia coli-Related Purpura Fulminans: an Unusual Manifestation Due to an Unusual Strain?

    PubMed Central

    Amara, Marlène; Bonacorsi, Stéphane; Bedel, Jérôme; Mira, Jean-Paul; Laurent, Virginie; Socha, Koryna; Bruneel, Fabrice; Pangon, Béatrice; Bédos, Jean-Pierre

    2014-01-01

    We describe an exceptional case of life-threatening group A Escherichia coli-induced purpura fulminans. Genotyping of common polymorphisms in genes involved in innate immunity or coagulation did not reveal known susceptibility to such a manifestation. Genetic analysis of the strain revealed an unusual conserved virulence plasmidic region, pointing out its potential virulence. PMID:25232165

  14. Manifestations of the local gastric immune response in gnotobiotic piglets infected with Helicobacter pylori

    Microsoft Academic Search

    Steven Krakowka; Susan S. Ringler; Kathryn A. Eaton; Wendy B. Green; Robert Leunk

    1996-01-01

    Helicobacter pylori, a human gastric bacterial pathogen, was inoculated into gnotobiotic piglets and manifestations of the resultant gastric inflammation was analyzed by in situ immunochemistry and flow cytometric analysis of isolated lamina propria leukocytes (LPL) and peripheral blood leukocytes (PBL) recovered from infected and control piglets. Gastric mucosa tissue sections from uninfected control piglets were essentially negative for cluster differentiation-

  15. Seborrheic Area Erythema as a Common Skin Manifestation in Japanese Patients with Dermatomyositis

    Microsoft Academic Search

    N. Okiyama; H. Kohsaka; N. Ueda; T. Satoh; I. Katayama; K. Nishioka; H. Yokozeki

    2008-01-01

    Background: Although dermatomyositis (DM)-associated facial erythema was noted in the nasolabial folds of Japanese patients, DM-associated facial erythema other than heliotrope rash has drawn little attention in previous studies. Objectives: To characterize phenotypical features and frequencies of erythema, especially those in the seborrheic area of the head, in DM patients. Methods: A retrospective study on skin manifestations in 33 DM

  16. Solar Wind: Manifestations of Solar Activity E N CYC LO PE D IA O F AS T R O N O MY AN D AS T R O PHYS I C S Solar Wind: Manifestations of Solar

    E-print Network

    Webb, David F.

    Solar Wind: Manifestations of Solar Activity E N CYC LO PE D IA O F AS T R O N O MY AN D AS T R O PHYS I C S Solar Wind: Manifestations of Solar Activity The Sun's outer atmosphere, the corona, is continually heated and expands to create the solar wind. Solar activity waxes and wanes with the 11 yr cycle

  17. The Laryngeal Manifestations of Laryngeal-pharynx Reflux and its Correlation with the Population of Manaus City

    Microsoft Academic Search

    Alexandre Borges Barbosa; Luciana da Silva Barberena; Keila Lucas; Pansini Barbosa; Daniel Santana Ribeiro

    The present work is justified for the lack of local data regarding the problem and it aims: to detect the manifestations of laryngeal-pharynx reflux; to determine eating habits of the manauenses (people from Manaus) which damage laryngeal-pharynx structure; to correlate the laryngeal manifestations of laryngeal- pharynx reflux with the eating habits of the manauense population; to detect inadequate and harmful

  18. Manifestly gauge invariant, continuum calculation of the SU(N) Yang-Mills two-loop {beta} function

    SciTech Connect

    Morris, Tim R.; Rosten, Oliver J. [School of Physics and Astronomy, University of Southampton, Highfield, Southampton SO17 1BJ (United Kingdom)

    2006-03-15

    The manifestly gauge invariant exact renormalization group provides a framework for performing continuum computations in SU(N) Yang-Mills theory, without fixing the gauge. We use this formalism to compute the two-loop {beta} function in a manifestly gauge invariant way, and without specifying the details of the regularization scheme.

  19. [Features of the spatial organization of EEG manifestations in different variants of anxiety symptoms in patients with depression].

    PubMed

    Kutsenko, D O; Ivonin, A A; Shuvaev, V T; Lisianskaia, N G; Nozdrachev, A D

    2015-01-01

    Investigations were carried out on 36 patients with reactive depressive states and repeating depressive episodes with severe manifestation of anxiety syndrome. The spatial organization of brain activity by cross-correlation analysis was studied. Shown, that clinical depression and anxiety syndrome is reflected in the structure of the spatial organization of EEG depending on options manifestation of anxiety disorder. PMID:25857176

  20. 19 CFR 4.76 - Procedures and responsibilities of carriers filing outbound vessel manifest information via the AES.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ...outbound vessel manifest information via the AES. 4.76 Section 4.76 Customs Duties...outbound vessel manifest information via the AES. (a) The sea carrier's module...component of the Automated Export System (AES) (see, part 192, subpart B,...

  1. 19 CFR 4.76 - Procedures and responsibilities of carriers filing outbound vessel manifest information via the AES.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ...outbound vessel manifest information via the AES. 4.76 Section 4.76 Customs Duties...outbound vessel manifest information via the AES. (a) The sea carrier's module...component of the Automated Export System (AES) (see, part 192, subpart B,...

  2. 19 CFR 4.76 - Procedures and responsibilities of carriers filing outbound vessel manifest information via the AES.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ...outbound vessel manifest information via the AES. 4.76 Section 4.76 Customs Duties...outbound vessel manifest information via the AES. (a) The sea carrier's module...component of the Automated Export System (AES) (see, part 192, subpart B,...

  3. 19 CFR 4.76 - Procedures and responsibilities of carriers filing outbound vessel manifest information via the AES.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ...outbound vessel manifest information via the AES. 4.76 Section 4.76 Customs Duties...outbound vessel manifest information via the AES. (a) The sea carrier's module...component of the Automated Export System (AES) (see, part 192, subpart B,...

  4. A Learning and Memory Area in the Octopus Brain Manifests a Vertebrate-Like Long-Term Potentiation

    E-print Network

    Hochner, Binyamin

    of Octopus vulgaris from the Bay of Naples, Italy, were held in individual aquaria in synthetic seawaterA Learning and Memory Area in the Octopus Brain Manifests a Vertebrate- Like Long-Term Potentiation in the octopus brain manifests a vertebrate-like long-term potentiation. J Neurophysiol 90: 3547­3554, 2003

  5. Electroencephalographic and seizure manifestations in two patients with folate receptor autoimmune antibody-mediated primary cerebral folate deficiency.

    PubMed

    Steele, Sonya U; Cheah, Sue Mei; Veerapandiyan, Aravindhan; Gallentine, William; Smith, Edward C; Mikati, Mohamad A

    2012-08-01

    Seizure semiology and electroencephalographic (EEG) manifestations of autoimmune-mediated cerebral folate deficiency (CFD) before and after therapy have yet to be fully characterized. Here, we report these findings in two such patients. Our first patient presented with the novel manifestation of infantile spasms at the age of 3months, while the second developed the previously reported initial onset of tonic seizures with static developmental delay, but subsequently manifested the novel finding of electrical status epilepticus in sleep at the age of 15years. Awareness of these new manifestations, together with the previously reported manifestations of developmental delay, seizure onset during the first 2years of life, occurrence of tonic, myoclonic-astatic, absence, and generalized tonic-clonic seizures, with an EEG of generalized spike-slow waves and multifocal spikes, is important to increase the index of suspicion of this treatable disorder. PMID:22749608

  6. Manifestly gauge invariant perturbations of scalar–tensor theories of gravity

    NASA Astrophysics Data System (ADS)

    Han, Yu; Giesel, Kristina; Ma, Yongge

    2015-07-01

    The general relativistic perturbations of scalar–tensor theories (STT) of gravity are studied in a manifestly gauge invariant Hamiltonian formalism. After the derivation of the Hamiltonian equations of motion in this framework, the gauge invariant formalism is used to compute the evolution equations of linear perturbations around a general relativistic spacetime background in the Jordan frame. These equations are then specialized to the case of a flat FRW cosmological background. Furthermore, the equivalence between the Jordan frame and the Einstein frame of STT in the manifestly gauge invariant Hamiltonian formalism is analyzed, and it is shown that also in this framework they can be related by a conformal transformation. Finally, the obtained evolution equations for the linear perturbations in our formalism are compared with those in the standard cosmological perturbation theory. It turns out that the perturbation equations in the two different formalisms coincide with each other in a suitable limit.

  7. Oral manifestations of plummer-vinson syndrome: a classic report with literature review.

    PubMed

    Samad, Abdul; Mohan, N; Balaji, R V Suresh; Augustine, Dominic; Patil, Shankar Gouda

    2015-03-01

    Plummer-Vinson syndrome (PVS) is a triad of microcytic hypochromic anemia, atrophic glossitis, and esophageal webs or strictures. It is one of the syndromes associated with iron deficiency anemia. Symptoms resulting from anemia predominates the clinical picture, apart from the additional features such as glossitis, angular cheilitis, and dysphagia. Dysphagia is main clinical feature of PVS. PVS carries an increased risk of development of squamous cell carcinoma of esophagus and pharynx. A classic case report of PVS with clinical features, oral manifestations, malignant potential, differential diagnosis, investigation, dental implication, and treatment is discussed here with the literature review from the dentist's point of view. The article carries a message that dental surgeons have to be familiar with the oral manifestations of anemia and be able to suspect PVS to aid in early diagnosis and prompt treatment. PMID:25878483

  8. Celiac Disease and Dermatologic Manifestations: Many Skin Clue to Unfold Gluten-Sensitive Enteropathy

    PubMed Central

    Caproni, Marzia; Bonciolini, Veronica; D'Errico, Antonietta; Antiga, Emiliano; Fabbri, Paolo

    2012-01-01

    Cutaneous manifestations of intestinal diseases are increasingly reported both in the adult and in the children, and this association cannot longer be considered a simple random. Besides the well-known association between celiac disease (CD) and dermatitis herpetiformis (DH), considered as the cutaneous manifestation of gluten-dependent enteropathy, is more frequently reported also the association with other mucocutaneous diseases. Among these there are both autoimmune, allergic, and inflammatory diseases, but also a more heterogeneous group called miscellaneous. The knowledge about pathogenic, epidemiological, clinical, and diagnostic aspects of CD is increasing in recent years as well as those about DH, but some aspects still remain to be defined, in particular the possible pathogenetic mechanisms involved in the association between both CD and DH and CD and other immunological skin diseases. The aim of this paper is to describe the skin diseases frequently associated with CD, distinguishing them from those which have a relationship probably just coincidental. PMID:22693492

  9. A Case of Hemophagocytic Syndrome due to Tuberculosis: Uncommon Manifestation of a Common Disease

    PubMed Central

    Singha, Arijit; Mukherjee, Adreesh; Dasgupta, Riddhi; Das, Tapas

    2014-01-01

    Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is the manifestation of immune dysregulation. It is associated with ineffective but exaggerated immune response and infiltration of active lymphocytes and histiocytes in various organs. This devastating clinical condition has myriad of clinical and biochemical manifestations such as fever, splenomegaly, pancytopenia, hypertrygliceridemia, and hyperferritinemia. It can be either primary or secondary. Primary HLH usually presents in childhood. Secondary HLH occurs due to infection mostly viruses but other aetiologies are also important as early detection and treatment may improve survival. Hemophagocytosis due to tuberculosis is uncommon. Only handful of cases have been reported mostly in immunocompromised patients. We report a case of hemophagocytic syndrome associated with disseminated tuberculosis in an immunocompetent women highlighting early diagnosis and treatment is a demanding need in this devastating disease. PMID:25404945

  10. Werner syndrome: a changing pattern of clinical manifestations in Japan (1917~2008).

    PubMed

    Goto, M; Ishikawa, Y; Sugimoto, M; Furuichi, Y

    2013-02-01

    As ~75% of the Werner syndrome (WS) patients recognized between 1904 and 2008 all over the world are of Japanese origin, the most case reports and clinical studies on WS has been published in Japanese journals. Thus, the detailed English-written clinical review on the recent WS case reports has been warranted. Although WS has been characterized by a variety of clinical manifestations mimicking premature aging, the recent longevity and delayed age-associated manifestations observed both from Japanese WS and general population may suggest a common environmental effect on some gene(s) other than WRN and may give us a newer pathophysiological look at WS and also natural aging through the molecular dysfunction of WRN. PMID:23524889

  11. Nasal manifestations of IgG4-related disease: A report of two cases.

    PubMed

    Ohno, Keiko; Matsuda, Yoko; Arai, Tomio; Kimura, Yurika

    2015-12-01

    IgG4-related disease (IgG4-RD) is a recently recognized clinical disease entity characterized by elevated serum IgG4, tumefaction, tissue infiltration of IgG4-positive plasma cells and fibrosis. IgG4-RD may occur, either synchronously or metachronously, in a variety of organs throughout the body. We describe herein two representative cases of the nasal manifestations of IgG4-RD, characterized by diffuse, crusty, erosive lesions on nasal mucosa. Oral steroid administration was effective in treating these nasal manifestations. We report a decrease in IgG4 positive plasma cell infiltrates in nasal mucosa biopsy specimens after steroid therapy, demonstrating that infiltration of IgG4-positive cells is reversible. PMID:26060132

  12. A STUDY OF PSYCHIATRIC MANIFESTATIONS OF PHYSICALLY ASYMPTOMATIC HIV - I SEROPOSITIVE INDIVIDUALS

    PubMed Central

    Satapathy, Ramanand; Krishna, Murali N.; Babu, Ashok M.; Vijayagopal, M.

    2000-01-01

    Neuropsychiatric disorders are common both secondary to the complications of immune suppression and as direct effects of HIV on the brain. A high prevalence of psychiatric disorder is reported both in physically asymptomatic and symptomatic persons. A thorough search was made in MEDLAR for the research literature available in the field of neuropsychiatric manifestations of AIDS. There is paucity of literature particularly in India and to be more specific in asymptomatic AIDS individuals, hence this study is intended to fill up the lacuna. The findings of this study show that the prevalence rate of psychiatric manifestations is 90% in HIV positive individuals and 33% in HIV negative group. This rate is significantly high though they are not suffering with physically disabling symptoms. So, the coping mechanisms and defence mechanisms in an individual who acquired HIV infection should be assessed and the significance of counselling before screening, plays a major role in the management of asymptomatic positive and negative individual. PMID:21407982

  13. Oral Manifestations of Plummer-Vinson Syndrome: A Classic Report with Literature Review

    PubMed Central

    Samad, Abdul; Mohan, N; Balaji, R V Suresh; Augustine, Dominic; Patil, Shankar Gouda

    2015-01-01

    Plummer-Vinson syndrome (PVS) is a triad of microcytic hypochromic anemia, atrophic glossitis, and esophageal webs or strictures. It is one of the syndromes associated with iron deficiency anemia. Symptoms resulting from anemia predominates the clinical picture, apart from the additional features such as glossitis, angular cheilitis, and dysphagia. Dysphagia is main clinical feature of PVS. PVS carries an increased risk of development of squamous cell carcinoma of esophagus and pharynx. A classic case report of PVS with clinical features, oral manifestations, malignant potential, differential diagnosis, investigation, dental implication, and treatment is discussed here with the literature review from the dentist’s point of view. The article carries a message that dental surgeons have to be familiar with the oral manifestations of anemia and be able to suspect PVS to aid in early diagnosis and prompt treatment. PMID:25878483

  14. Familial Q fever clustering with variable manifestations imitating infectious and autoimmune disease.

    PubMed

    Abu Rmeileh, A; Khoury, T; Meir, K; Drori, A; Shalit, M; Benenson, S; Elinav, H

    2015-05-01

    Q fever, caused by Coxiella burnetii, can present as an outbreak of acute disease ranging from asymptomatic disease, pneumonia, hepatitis or fever of unknown origin, which can progress to a chronic disease, most frequently endocarditis. The occurrence of Q fever within families is rarely described, and in most cases presents with uniform acute disease manifestations. Here we present a familial cluster of Q fever presenting as highly variable synchronous manifestations in four of five family members, including prolonged fever of unknown origin, asymptomatic carrier state, hepatitis, and chronic endocarditis developing in the absence of previous symptoms. This case series highlights the possibility of Q fever developing in cohabitated individuals with highly variable symptoms masking the common disease etiology. Screening of all exposed individuals, even those not clinically suspected to be infected, may enable to better identify, treat and prevent progression to chronic disease. PMID:25770747

  15. Ichthyosis Linearis Circumflexa as the Only Clinical Manifestation of Netherton Syndrome.

    PubMed

    Guerra, Liliana; Fortugno, Paola; Pedicelli, Cristina; Mazzanti, Cinzia; Proto, Vittoria; Zambruno, Giovanna; Castiglia, Daniele

    2015-06-24

    Ichthyosis linearis circumflexa (ILC) presents as serpiginous and migratory erythematous patches with double-edged scales. ILC is rarely an isolated skin manifestation, but most commonly a part of Netherton syndrome (NS). NS is caused by SPINK5 mutations, which lead to absent or sometimes reduced expression of the serine protease inhibitor LEKTI. NS is characterised by congenital ichthyosiform erytroderma, trichorrhexis invaginata (TI) and atopy. We report 2 children who presented since the first months of life cheek erythema followed by the appearance of sparse ILC lesions on the face, trunk and proximal extremities. Erythroderma at birth, TI and atopy were absent. LEKTI immunoreactivity was reduced in patient epidermis, and serine protease activity was modestly increased, while desmoglein-1 expression remained unaffected. SPINK5 mutation and expression analysis in patient keratinocytes revealed compound heterozygous splicing variants, which allowed residual LEKTI secretion. Our results show that ILC can be the only clinical manifestation of NS. PMID:25710899

  16. Laryngotracheal stenosis requiring emergency tracheostomy as the first manifestation of childhood-relapsing polychondritis.

    PubMed

    Buscatti, Izabel M; Giacomin, Maria Fernanda A; Silva, Marco Felipe C; Campos, Lúcia M A; Sallum, Adriana M E; Silva, Clovis A

    2013-01-01

    Relapsing polychondritis is a rare childhood disorder of unknown etiology, characterized by inflammatory, recurrent and destructive cartilage lesions. The chondritis could be widespread and involves generally laryngeal and auricular hyaline cartilages. We described a 9 years and 4 months old girl, who presented recurrent acute laryngotracheitis and laryngotracheal stenosis, which were the first manifestations of relapsing polychondritis, and was submitted to emergency tracheostomy. She also had ear condritis and arthritis, being treated with prednisolone and methotrexate. In conclusion, we reported a rare case of relapsing polychondritis that presented a life-threatening laryngo-tracheo-bronchial disorder requiring tracheostomy. We suggest that the diagnosis of relapsing polychondritis should be considered for patients who present recurrent acute laryngotracheitis with other types of condritis, as well as musculoskeletal manifestations. PMID:24149019

  17. Atypical radiological manifestations of thoracic sarcoidosis: A review and pictorial essay

    PubMed Central

    Al-Jahdali, Hamdan; Rajiah, Prabhakar; Koteyar, Shyam Sunder; Allen, Carolyn; Khan, Ali Nawaz

    2013-01-01

    Thoracic sarcoidosis is a common disease, with well-described and recognizable radiographic features. Nevertheless, most physicians are not familiar with the rare atypical often-confusing manifestations of thoracic sarcoid. Although these findings have been previously reviewed, but more recent advances in imaging and laboratory science, need to be incorporated. We present a review of literature and illustrate the review with unpublished data, intended to provide a more recent single comprehensive reference to assist with the diagnosis when atypical radiographic findings of thoracic sarcoidosis are encountered. Thoracic involvement accounts for most of morbidity and mortality associated with sarcoidosis. An accurate timely identification is required to minimize morbidity and mortality. It is essential to recognize atypical imaging findings and relate these to clinical manifestations and histology. PMID:24250731

  18. Haemolytic anaemia as first manifestation of Wilson's disease: a report of two cases.

    PubMed

    Santra, Gouranga; Paul, Rudrajit; Choudhury, Partha Sarathi; Ghosh, Sumit Kr; De, Dibyendu; Das, Shubhabrata

    2014-10-01

    Wilson's disease can have different manifestations like jaundice, cirrhosis of liver, extrapyramidal symptoms and dementia. Haemolytic anaemia may occur but it is commonly associated with florid manifestation of liver disease. Sometimes, liver cell necrosis can release huge free copper ions in blood, giving rise to oxidant damage to erythrocytes. Oxidative damage to cell membrane, haemoglobin and erythrocyte metabolism causes haemolytic crisis. In some cases, liver involvement is subclinical, but nonetheless, free copper is released from necrosed hepatocytes and causes oxidative damage to erythrocytes.We had two cases of Wilson's disease with initial presentation as severe haemolytic anaemia and no other clinical feature suggestive of Wilson's disease was present. In unclear cause of haemolytic anaemia, especially in adolescents or young adults, Wilson's disease should be considered. As Wilson disease is rare and its initial presentation with haemolytic anaemia is rarer, high level of suspicion is required to diagnose it. PMID:25906525

  19. Differential Contributions of Polycystic Ovary Syndrome (PCOS) Manifestations to Psychological Symptoms.

    PubMed

    McCook, Judy G; Bailey, Beth A; Williams, Stacey L; Anand, Sheeba; Reame, Nancy E

    2015-07-01

    The purpose of this study was to investigate the relative contributions of previously identified Polycystic ovary syndrome (PCOS) manifestations (infertility, hirsutism, obesity, menstrual problems) to multiple psychological symptoms. Participants were 126 female endocrinology patient volunteers diagnosed with PCOS who completed a cross-sectional study of PCOS manifestations and psychological symptoms. Participants had significantly elevated scores on nine subscales of psychological symptoms. Menstrual problems were significantly associated with all symptom subscales as well as the global indicator, while hirsutism and obesity were significantly related to five or more subscales. After controlling for demographic factors, menstrual problems were the strongest predictor of psychological symptoms. Findings suggest features of excess body hair, obesity, and menstrual abnormalities carry unique risks for adverse psychologic symptoms, but menstrual problems may be the most salient of these features and deserve particular attention as a marker for psychological risk among women with PCOS. PMID:24390359

  20. Psychiatric manifestations as primary symptom of neurosyphilis among HIV-negative patients.

    PubMed

    Lin, Li-Rong; Zhang, Hui-Lin; Huang, Song-Jie; Zeng, Yan-Li; Xi, Ya; Guo, Xiao-Jing; Liu, Gui-Li; Tong, Man-Li; Zheng, Wei-Hong; Liu, Li-Li; Yang, Tian-Ci

    2014-01-01

    This study characterizes psychiatric manifestations as a primary symptom of neurosyphilis (NS). Fifty-two of the 169 NS patients presented with psychiatric manifestations, many patients had characteristics of more than one syndrome, including cognitive impairment, personality disorders, delirium, hostility, dysarthria, confusion, disruption of their sleep-wake cycle, fecal and urinary incontinence, dysphoria, paranoia, hallucinations, expansive mood, and mania. Fifty-two patients had positive sera RPR and T. pallidum particle agglutination (TPPA), 75% had positive CSF RPR, 96.2% had positive CSF TPPA, 44.2% had CSF pleocytosis and elevated CSF proteins, and 70.0% had nonspecific, abnormal brain MRIs. These results indicate that NS mimics almost all psychiatric disorders. PMID:24737221

  1. Restless Legs Syndrome as a First Manifestation of a Cerebral Infarct

    PubMed Central

    Ruppert, Elisabeth; Kilic-Huck, Ulker; Wolff, Valérie; Tatu, Laurent; Ghobadi, Marion; Bataillard, Marc; Bourgin, Patrice

    2014-01-01

    The onset of restless legs syndrome (RLS) is usually progressive and the neural substrates underlying its pathophysiology remain to be identified. Here we report on a patient presenting with acute-onset RLS that was symptomatic of a right anteromedial pontine infarction. This case is exceptional because RLS appeared several hours before the occurrence of a regressive dysarthria clumsy-hand syndrome. Additionally, millimetric MRI sections showed that the structures possibly involved in RLS pathogenesis were the corticospinal tract, the pontine nuclei, and the pontocerebellar fibers. Although this is uncommon, clinicians should be aware that RLS characterized by a sudden onset can be a clinical manifestation related to stroke. Citation: Ruppert E, Kilic-Huck U, Wolff V, Tatu L, Ghobadi M, Bataillard M, Bourgin P. Restless legs syndrome as a first manifestation of a cerebral infarct. J Clin Sleep Med 2014;10(9):1037-1038. PMID:25142775

  2. Pet killing as a manifestation of impulse control disorder secondary to pramipexol.

    PubMed

    Micheli, Federico; Pellene, Alejandro; Arcushin, Daniela; Calzinari, Aldo; Farret, Michel Sáenz

    2015-01-01

    Impulse control disorders are frequent in Parkinson disease and are commonly associated with dopamine agonists intake. Typical manifestations include punding, hypersexuality, pathological gambling, and other compulsive behaviors. Symptoms often promptly disappear when dopamine agonists are discontinued, but if the origin is misinterpreted, symptoms may become a problem. We here report the case of a patient with juvenile Parkinson disease treated with 4.5 mg/d of pramipexol, who developed the need to adopt cats. He adopted almost 50 of them, but after he adopted them, he felt the need to kill them. The case became well known, and he became depressed and isolated. He was admitted to a psychiatric hospital and thought that he was a psychotic. However, when pramipexol was replaced by levodopa, the symptoms disappeared. This case illustrates the wide range of manifestations of impulse control disorders and warrants the inclusion of violent behaviors among them. PMID:25768852

  3. The Mucocutaneous Manifestations and Pathergy Reaction in Behçet’s Disease

    Microsoft Academic Search

    M. Cem Mat; Dongsik Bang; Melike Meliko?lu

    \\u000a The diagnosis of Behçet’s disease is based on clinical features. There is no specific diagnostic test. Skin-mucosa lesions\\u000a like recurrent aphtous stomatitis, genital ulcers, and various cutaneous lesions are its foremost manifestations. The presence\\u000a of erythema nodosum-like lesions, pseudofolliculitis, papulopustular lesions, acneiform nodules, subcutaneous thrombophlebitis,\\u000a and cutaneous hyperreactivity (pathergy) are included in the criteria of the International Study Group for

  4. Neuro-ophthalmological disorders in HIV infected subjects with neurological manifestations

    PubMed Central

    Mwanza, J-C; Nyamabo, L K; Tylleskär, T; Plant, G T

    2004-01-01

    Aims: To determine the frequency and features of neuro-ophthalmological manifestations in neurologically symptomatic HIV infected patients and to assess whether or not the visual evoked potential (VEP) features in these patients differ from those of neurologically asymptomatic HIV infected patients. Methods: Neuro-ophthalmological evaluation was performed in 166 neurologically symptomatic confirmed HIV positive patients, of whom 75 with normal ophthalmological examination were further studied by means of VEPs. The VEPs values were compared to those obtained from 53 other confirmed HIV positive subjects with neither ophthalmological nor neurological manifestations, who served as a comparison group and to the references values of our laboratory. Results: An abnormal neuro-ophthalmological examination was noted in 99/166 patients (60%). Eye movement disorders were present in 99 patients (51%). Visual field defects were detected in 39% of the patients. Optic neuropathy was noted in 31%, papilloedema in 27% and ocular motor nerve palsies in 26% of the patients. Toxoplasmosis and cryptococcosis were the most frequent associated pathologies, though in some patients the HIV itself was the presumed cause. VEPs were abnormal in 57% and 42% of patients with and without neurological manifestations, respectively. Compared to asymptomatic patients, symptomatic patients had a significantly increased mean latency; however, both groups had significant increase in mean latency compared to reference values. Conclusion: Neuro-ophthalmological manifestations are common in neurologically symptomatic HIV infected patients. Subclinical dysfunction in the visual pathways is a common phenomenon in both HIV infected patients with and without neurological symptoms, but neurologically symptomatic patients seem to have more damage in their visual pathways. PMID:15489493

  5. [Infiltrating perineal and scrotal inflammation: rare cutaneous manifestation of familial Mediterranean fever or acne inversa?].

    PubMed

    Huber, J; Lagally, C; Buse, S; Hatiboglu, G; Höfner, T; Hach, C; Hohenfellner, M

    2008-03-01

    Familial Mediterranean fever (FMF) can present cutaneous symptoms. In the reported case, infiltrating perineal and scrotal inflammation were attributed to FMF and treated by systemic medication with colchicine. The poor outcome of this conservative approach and pathognomonic axillary dermatological findings allowed the diagnosis of acne inversa to be made. Knowledge of this clinical picture possibly including genital manifestations is crucial, as early excision of all affected regions is the therapy of choice that enables healing. PMID:18210069

  6. Protein-Losing Enteropathy as the Principal Manifestation of Constrictive Pericarditis

    PubMed Central

    Nikolaidis, Nikolaos; Tziomalos, Konstantinos; Giouleme, Olga; Gkisakis, Dimitrios; Kokkinomagoulou, Amalia; Karatzas, Nikolaos; Papanikolaou, Athanassios; Tsitourides, Ioannis; Eugenidis, Nikolaos; Kontopoulos, Athanassios

    2005-01-01

    Constrictive pericarditis represents a rare cause of protein-losing enteropathy resulting from intestinal lymphangiectasia. We report the case of a patient with an atypical clinical presentation of constrictive pericarditis and protein-losing enteropathy as its principal manifestation; he was successfully treated with pericardiectomy. We conclude that constrictive pericarditis should be considered in the presence of protein-losing enteropathy and also, protein-losing enteropathy should be considered in the differential diagnosis of hypoalbuminemia. PMID:16191147

  7. Envenoming caused by a Portuguese man-o'-war (Physalia physalis) manifesting as purpuric papules.

    PubMed

    Risk, Yamin José; Cardoso, João Luiz Costa; Haddad Junior, Vidal

    2012-01-01

    We report the case of a 42-year old woman who was envenomed by a Portuguese man-o'-war (Physalia physalis). She presented an anomalous reaction manifested by purpuric papules that appeared after the initial phase of envenoming (around 24 hours later), when linear erythematous and edematous papules were observed. Late-onset reactions in accidents involving cnidarians commonly include chronic eruptions and local pigmentation. PMID:22892787

  8. Molecular analysis and association with clinical and laboratory manifestations in children with sickle cell anemia

    PubMed Central

    Camilo-Araújo, Roberta Faria; Amancio, Olga Maria Silverio; Figueiredo, Maria Stella; Cabanãs-Pedro, Ana Carolina; Braga, Josefina Aparecida Pellegrini

    2014-01-01

    Objectives To analyze the frequency of ?S-globin haplotypes and alpha-thalassemia, and their influence on clinical manifestations and the hematological profile of children with sickle cell anemia. Method The frequency of ?S-globin haplotypes and alpha-thalassemia and any association with clinical and laboratorial manifestations were determined in 117 sickle cell anemia children aged 3–71 months. The confirmation of hemoglobin SS and determination of the haplotypes were achieved by polymerase chain reaction-restriction fragment length polymorphism, and alpha-thalassemia genotyping was by multiplex polymerase chain reaction (single-tube multiplex-polymerase chain reaction). Results The genotype distribution of haplotypes was 43 (36.7%) Central African Republic/Benin, 41 (35.0%) Central African Republic/Central African Republic, 20 (17.0%) Rare/atypical, and 13 (11.1%) Benin/Benin. The frequency of the ?3.7 deletion was 1.71% as homozygous (??3.7/??3.7) and 11.9% as heterozygous (??3.7/??). The only significant association in respect to haplotypes was related to the mean corpuscular volume. The presence of alpha-thalassemia was significantly associated to decreases in mean corpuscular volume, mean corpuscular hemoglobin and reticulocyte count and to an increase in the red blood cell count. There were no significant associations of ?S-globin haplotypes and alpha-thalassemia with clinical manifestations. Conclusions In the study population, the frequency of alpha-thalassemia was similar to published data in Brazil with the Central African Republic haplotype being the most common, followed by the Benin haplotype. ?S-globin haplotypes and interaction between alpha-thalassemia and sickle cell anemia did not influence fetal hemoglobin concentrations or the number of clinical manifestations. PMID:25305165

  9. Les manifestations ophtalmologiques de la maladie de Behçet, à propos de 33 cas

    PubMed Central

    Andaloussi, Idriss Benatiya; Alami, Bouchra; Abdellaoui, Meryem; Bhallil, Salima; Bono, Wafae; Tahri, Hicham

    2012-01-01

    Les manifestations ophtalmologiques au cours de la maladie de Behçet sont dominées par les uvéites, les vascularites rétiniennes et les thromboses veineuses rétiniennes. Nous rapportons une étude rétrospective portant sur tous les malades atteints de la maladie de Behçet, hospitalisés au sein du service d'Ophtalmologie de Fès de Janvier 2007 à Décembre 2009. Au total ce sont 33 patients qui sont inclut dans l'étudeLa moyenne d’âge est de 28,4 ans. Le délai moyen de consultation varie entre 1 jour et 3 ans. L'atteinte oculaire est bilatérale chez 26 patients (78,8% des yeux). L'AV est très basse avant le traitement: 28,8% à moins de 1/10. Les manifestations oculaires sont dominées par l'uvéite avec 77,3%, suivie de la vascularite rétinienne 54, 5% et la maculopathie 51, 5%. Le pourcentage des yeux dont l'AV était inférieur à 1/10 est passé à 19,7% après traitement. La fréquence de l'atteinte oculaire au cours de la maladie de Behçet est diversement appréciée selon les auteurs et selon le mode de recrutement des patients: elle va de 29% à 100%. L'atteinte uvéale est la plus fréquente des manifestations ophtalmologiques. Les lésions vasculaires rétiniennes sont dominées par la vascularite rétinienne essentiellement la périphlébite aussi bien au pôle postérieur qu’à la périphérie rétinienne. En l'absence de traitement et de mauvaise observance, le pronostic oculaire de la maladie de Behçet est très mauvais. La cécité s'installe dans 13 à 32%. Les manifestations ophtalmologiques au cours de la maladie de Behçet ont une valeur considérable, tant sur le plan diagnostique que pronostique. Notre étude a permis de décrire les aspects cliniques de l'atteinte oculaire de la maladie de Behçet dans un service d'Ophtalmologie tertiaire au Maroc. PMID:23396991

  10. Primär extragenitale Manifestation eines malignen Müller-Mischtumors im Omentum majus

    Microsoft Academic Search

    B. Illert; M. Sailer; A. M. Gassel; A. Thiede

    1996-01-01

    \\u000a Summary.   Malignant mixed Müllerian tumours (MMMT) are tumours of the female genital tract which rarely occur in extragenital sites.\\u000a We report a case of a 46-year-old female with MMMT with primary occurrence in the greater omentum. Only 19 cases of primary\\u000a extragenital manifestation of this tumour are described in the literature. The clinical appearance, diagnosis, therapy, histological\\u000a evaluation and histogenesis

  11. Membranous nephropathy as a rare renal manifestation of IgG4-related disease

    PubMed Central

    Kurien, A. A.; Raychaudhury, A.; Walker, P. D.

    2015-01-01

    IgG4-related disease, a newly described immune-mediated disorder with tissue infiltration of IgG4-positive plasma cells, has been reported in nearly every organ. In the kidney, it manifests as IgG4-related tubulointerstitial nephritis (TIN) but may also present as membranous nephropathy. We report a patient with IgG4 renal disease who had membranous nephropathy as well as TIN.

  12. Preliminary Evidence of Sturgeon Density and Other Stressors on Manifestation of White Sturgeon Iridovirus Disease

    Microsoft Academic Search

    S. E. LaPatra; J. M. Groff; T. L. Patterson; W. D. Shewmaker; M. Casten; J. Siple; A. K. Hauck

    1996-01-01

    Two studies were conducted using 5- to 6-month-old juvenile (mean weight: 3-4 g) white sturgeon, Acipenser transmontanus, to examine the effects of density and other stressors on manifestation of disease caused by the white sturgeon iridovirus (WSIV). In the first study, replicate groups of Snake River white sturgeon were stocked at three densities (953, 1,907, and 3,178 fish\\/m) in 0.31-m

  13. Clinical Manifestations of Impaired GnRH Neuron Development and Function

    Microsoft Academic Search

    Hyung-Goo Kim; Balasubramanian Bhagavath; Lawrence C. Layman

    2008-01-01

    Gonadotropin-releasing hormone (GnRH) and olfactory neurons migrate together in embryologic development, and disruption of this process causes idiopathic hypogonadotropic hypogonadism (IHH) with anosmia (Kallmann syndrome (KS)). Patients with IHH\\/KS generally manifest irreversible pubertal delay and subsequent infertility due to deficient pituitary gonadotropins or GnRH. The molecular basis of IHH\\/KS includes genes that: (1) regulate GnRH and olfactory neuron migration; (2)

  14. Adherence to guideline-recommended therapies among patients with diverse manifestations of vascular disease

    PubMed Central

    Chen, Debbie C; Armstrong, Ehrin J; Singh, Gagan D; Amsterdam, Ezra A; Laird, John R

    2015-01-01

    Background Current guidelines recommend aspirin, statins, angiotensin-converting enzyme inhibitors (ACEIs), and smoking abstinence for all patients with vascular disease. There is little data on the variation in adherence to guideline-recommended therapies among patients with different clinical manifestations of vascular disease. Purpose To analyze the variation in adherence to guideline-recommended therapies among patients with diverse manifestations of vascular disease. Methods We analyzed a comprehensive database of all patients with critical limb ischemia, claudication, acute limb ischemia, carotid artery stenosis, subclavian artery stenosis, renal artery stenosis, or mesenteric ischemia who underwent angiography between 2006 and 2013 at a multidisciplinary vascular center. Results Among 1,114 patients with vascular disease, adherence to guideline-recommended therapy at time of angiography included use of aspirin in 936 (84%), statins in 753 (68%), ACEIs in 673 (60%), and smoking abstinence in 788 (71%). A total of 335 (30%) patients utilized all four guideline-recommended therapies. Adherence to four guideline-recommended therapies was lowest among patients with acute limb ischemia (14%) and highest among patients with renal artery stenosis (37%). Among all patients with vascular disease, the range of adherence to individual guidelines was 64%–91% for aspirin, 43%–83% for statins, 49%–66% for ACEIs, and 47%–78% for smoking abstention. Conclusion The majority of patients with diverse manifestations of vascular disease take aspirin and abstain from smoking while fewer patients are prescribed ACEIs and statins. Among the current recommendations, statins have the widest variation in adherence. Less than one-third of patients with diverse manifestations of vascular disease are prescribed all four guideline-recommended therapies. PMID:25767395

  15. Clinical and Imaging Manifestations of Hemorrhagic Pulmonary Leptospirosis: A State-of-the-Art Review

    Microsoft Academic Search

    Edson Marchiori; Sílvia Lourenço; Sérgio Setúbal; Gláucia Zanetti; Taisa Davaus Gasparetto; Bruno Hochhegger

    2011-01-01

    Leptospirosis, a spirochetal zoonosis, is frequently unrecognized due to its manifestation as an undifferentiated fever. It\\u000a is an emerging infectious disease that has changed from an occupational disease of veterinarians, farmers, butchers, and other\\u000a animal handlers to a cause of epidemics in poor and decayed urban communities in developing countries. Humans are infected\\u000a when mucous membranes or abraded skin come

  16. Cocaine use and its rheumatic manifestations: a case report and discussion

    Microsoft Academic Search

    Sumeet K. Bhinder; Vikas Majithia

    2007-01-01

    Cocaine use can be associated with a wide spectrum of rheumatic manifestations. It poses a diagnostic challenge as the patients\\u000a usually withhold the information of cocaine use, and no serological tests are available to establish this diagnosis. We report\\u000a a patient with vasculopathic syndrome secondary to cocaine use. Despite initial denial of drug abuse, skin biopsy suggested\\u000a the diagnosis, which

  17. Radiologic manifestations of bronchoscopic lung volume reduction in severe chronic obstructive pulmonary disease.

    PubMed

    Grabenhorst, Matthias; Schmidt, Bernd; Liebers, Uta; Oestmann, Jörg-Wilhelm

    2015-03-01

    OBJECTIVE. Bronchoscopic lung volume reduction promises to become an effective treatment option in severe chronic obstructive pulmonary disease. Several techniques are currently being investigated, including implantation of devices into the lung and instillation of hot water vapor or polymer. This article reviews the spectrum of radiologic manifestations on chest radiography and CT that occur after the intervention. CONCLUSION. Familiarity with the intended effects and adverse events will aid the radiologist in supporting bronchoscopic lung volume reduction. PMID:25714276

  18. Intestinal Pseudo-Obstruction as an Initial Manifestation of Systemic Lupus Erythematosus.

    PubMed

    Oh, Dong Jun; Yang, Jae Nam; Lim, Yun Jeong; Kang, Ji Hyuk; Park, Jung Hyun; Kim, Mal Young

    2015-07-01

    Intestinal pseudo-obstruction (IPO) is an uncommon, severe complication that occurs in a small subgroup of patients with systemic lupus erythematosus (SLE). To our knowledge, approximately 30 cases of IPO in SLE have been reported in the literature. Moreover, IPO is rare as an initial manifestation of SLE. We report a case of a 43-year-old woman with SLE who initially presented with IPO. PMID:26131004

  19. Oral Manifestation of Autosomal Recessive Congenital Ichthyosis in a 2-Year-Old Patient

    PubMed Central

    Ramar, Kavitha; Annamalai, Sankar; Hariharavel, V. P.; Aravindhan, R.; Ganesh, C.; Ieshwaryah, K.

    2014-01-01

    Ichthyosis is a heterogeneous family of hereditary keratinisation disorders mostly characterized by variable erythema of the whole body and different scaling patterns. Although these disorders affect tissues of epidermal origin, there is little evidence regarding the oral and dental manifestations of Lamellar Ichthyosis. A case report of early childhood caries in lamellar ichthyosis is presented and the dental consideration and management is discussed in this paper. PMID:24995134

  20. Adjuvant induced autoimmune/inflammatory syndrome presenting as joint and lung manifestations.

    PubMed

    Flores Padilla, Guillermo; Mora Mendoza, Benjamín; Pedraza Montenegro, Axel

    2014-01-01

    A 59 year-old female with a history of injection of an oily material in the buttocks 11 years ago. She developed symmetric aditive polyarthritis as well as superior and inferior airways involvement. There was no evidence of granulomatosis with polyangiitis (Wegener). She had several serum autoantibodies and a skin biopsy showed a foreign body granuloma. The diagnosis of adjuvant induced autoimmune/inflammatory syndrome was made. The pulmonary involvement was an atypical manifestation at the onset of disease. PMID:24685295

  1. Towards a Self-Managing Software Patching Process Using BlackBox Persistent-State Manifests

    Microsoft Academic Search

    John Dunagan; Roussi Roussev; Brad Daniels; Aaron Johnson; Chad Verbowski; Yi-min Wang

    2004-01-01

    We describe a new approach to self-managing software patching. We identify visibility into patch impact as the key missing component in automating the current patching process, and we present a suite of components that provides this visibility by constructing black-box persistent-state manifests through self-monitoring of dependencies. Additionally, we use the component suite to measure the actual impact of recent patches

  2. Manifestations of Space-Time Variation of Coupling Constants and Fundamental Masses

    NASA Astrophysics Data System (ADS)

    Flambaum, V. V.; Berengut, J. C.

    2011-11-01

    We present an overview of recent works that discuss observable manifestations of space-time variation of fundamental "constants" in a variety of physical systems. Studies in this direction are motivated by theories unifying gravity with other interactions; these often suggest the possibility of temporal and spatial variation of the fundamental constants in an expanding Universe. The effects of such variations could be observable in phenomena covering the lifespan of the Universe, from Big Bang nucleosynthesis to modern atomic clocks.

  3. a Manifestly Gauge Invariant and Universal Calculus for Su(n) Yang-Mills

    NASA Astrophysics Data System (ADS)

    Rosten, Oliver J.

    Within the framework of the Exact Renormalization Group, a manifestly gauge invariant calculus is constructed for SU(N) Yang-Mills. The methodology is comprehensively illustrated with a proof, to all orders in perturbation theory, that the ? function has no explicit dependence on either the seed action or details of the covariantization of the cutoff. The cancellation of these nonuniversal contributions is done in an entirely diagrammatic fashion.

  4. Role of clinical and paraclinical manifestations of methanol poisoning in outcome prediction

    PubMed Central

    Shadnia, Shahin; Rahimi, Mojgan; Soltaninejad, Kambiz; Nilli, Amir

    2013-01-01

    Background: Methanol poisoning is one of the most important poisoning due to drinking of illegal and non-standard alcoholic beverage in some countries. Relatively limited studies have been carried out to identify the prognostic factors in methanol poisoning. Materials and Methods: We performed a retrospective study on acute methanol-intoxicated patients, which were admitted on the Loghman Hakim Hospital Poison Center (Tehran, Iran) over a 24-month period. The demographic data, clinical manifestations and paraclinical findings, therapeutic interventions and outcome (survivors with or without complications and non-survivors) were extracted. Results: We evaluated 30 patients with methanol poisoning during 2 years. All of the patients were male; with the median age of 25.5 years. Visual disturbances, respiratory manifestations, and loss of consciousness were the most common clinical manifestations on admission time. The results of paraclinical manifestations on admission time were as following: The median of blood methanol level was 20 mg/dL. The median of pH, PaCO2 and HCO3 was 7.15, 22.35 mmHg and 7.2 mEq/L, respectively. Hyperglycemia was observed in 70% of the patients and the median of blood glucose was 184.5 mg/dL. In fourteen of the patients hemodialysis was performed. Median duration of hospitalization was 48 h. Nine of the patients died. There was a significant difference between survivors and non-survivors with regard to coma, blood methanol level, and PaCO2 and blood glucose. Furthermore, we did not observe a significant difference between these two groups regarding to pH, HCO3 level, and time interval between alcohol ingestion to hospital admission and beginning of hemodialysis. Conclusion: Regarding the results of this study, it can be suggested that coma, PaCO2 and hyperglycemia on admission time could be used as strong predictors of poor outcome. PMID:24497857

  5. Les manifestations d’allure auto-immune après allogreffe de moelle osseuse

    Microsoft Academic Search

    S Vinzio; B Lioure; F Grunenberger; J.-L Schlienger; B Goichot

    2004-01-01

    Introduction. – Bone marrow transplantation (BMT) is based on destruction of the patient’s bone marrow with rescue of haematopoietic stem cells from a donor. Chronic graft-vs-host disease (GVH) is the major complication post-BMT and mimics some autoimmune diseases, such as scleroderma, sicca syndrome, primary biliary cirrhosis and an increased prevalence of various autoantibodies. Other autoimmune-like manifestations have been reported as

  6. Ichtyose acquise et hyperkératose livédoïde palmoplantaire : deux manifestations cutanées initiales atypiques de lupus systémique

    Microsoft Academic Search

    C. Biver-Dalle; H. Gil; N. Méaux-Ruault; I. Mermet-Ginet; F. Aubin; P. Humbert; N. Magy-Bertrand

    Acquired ichthyosis and livedoid palmoplantar keratoderma have rarely been described in systemic lupus erythematosus (SLE). In the first case, a 51-year-old man presented with generalized acquired ichthyosis associated with renal glomurelosclerosis and neurolupus. rituximab allowed total resolution of the acquired ichthyosis. A livedoid palmoplantar keratoderma was observed in a 45-year-old woman as the initial clinical manifestation of a systemic lupus

  7. Neuro-cysticercosis presenting with single delusion: A rare psychiatric manifestation

    PubMed Central

    Chakraborty, Suhash; Singi, Shilpa R; Pradhan, Gyanaranjan; Anantha Subramanya, HC

    2014-01-01

    We report a case of multiple parenchymal neurocysticercosis in an elderly lady without raised intracranial tension which caused diagnostic confusion. The initial manifestation was only psychological as delusional disorder without any neurological symptoms or focal neurological deficit. Plain computed tomography scan showed mild bilateral periventricular and subcortical hypodensities. The development of hemiplegia during the course of psychiatric treatment prompted us to go for magnetic resonance imaging brain which clinched the diagnosis. PMID:25143893

  8. Parental Attachment and Its Impact on the Development of Psychiatric Manifestations in School-Aged Children

    Microsoft Academic Search

    Han Ik Yoo; Boong-Nyun Kim; Min Sup Shin; Soo Churl Cho; Kang-E Hong

    2006-01-01

    Background: Few studies have examined the relationship between the attachment styles of the primary caregivers and the behavioral problems of their school-aged children. This study was performed to identify the impact of insecure attachment patterns of parents on the development of their children’s psychiatric manifestations and disorders. Samplings and Methods: We included 494 2nd to 3rd graders and their primary

  9. Relapsing pancreatitis as initial manifestation of cystic fibrosis in a young man without pulmonary disease

    Microsoft Academic Search

    Volker Gross; Juergen Schoelmerich; Klaus Denzel; Wolfgang Gerok

    1989-01-01

    Summary  In young adults with acute pancreatitis a wide etiologic spectrum has to be considered. Among the possible causes, cystic\\u000a fibrosis is rare. Besides the typical clinical triad of pancreatic exocrine insufficiency, chronic obstrue-tive pulmonary\\u000a disease, and elevated sweat chloride levels, there is a wide spectrum of variants of the cystic fibrosis syndrome. Especially\\u000a mild manifestations of the disease may, therefore,

  10. Evidence against manifest right-handed currents in neutron beta decay

    Microsoft Academic Search

    G. Sanchez-Colon; A. Garcia

    2010-01-01

    The bounds and presence of manifest right-handed currents in neutron beta decay are reviewed. Assuming the unitarity of the Cabibbo-Kobayashi-Maskawa matrix, the current experimental situation imposes very stringent limits on the mixing angle, -0.000 77<ζ<0.000 89, and on the mass eigenstate, Mâ (GeV) is an element of (291.4,439.9), in contradiction with the established lower bound on Mâ.

  11. Evidence against manifest right-handed currents in neutron beta decay

    E-print Network

    A. Garcia; G. Sanchez-Colon

    2010-06-28

    Bounds and presence of manifest right handed currents in neutron beta decay are reviewed. Assuming the unitarity of the Cabibbo-Kobayashi-Maskawa matrix, current experimental situation imposes very stringent limits on the mixing angle, $-0.00077<\\zeta<0.00089$, and on the mass eigenstate, $M_2({\\rm GeV})\\in(291.4,439.9)$, in contradiction with the established lower bound on $M_2$.

  12. Evidence against manifest right-handed currents in neutron beta decay

    Microsoft Academic Search

    A. García; G. Sánchez-Colón

    2010-01-01

    The bounds and presence of manifest right-handed currents in neutron beta decay are reviewed. Assuming the unitarity of the Cabibbo-Kobayashi-Maskawa matrix, the current experimental situation imposes very stringent limits on the mixing angle, -0.00077

  13. Bounds on manifest left-right symmetry from neutron beta decay

    Microsoft Academic Search

    M. Aquino; A. Fernandez; A. Garcia

    1991-01-01

    The effect of manifest left-right symmetry in right-handed currents is studied in neutron beta decay. An especially strong bound on the mixing angle is obtained, - 0.0006 < zeta < 0.0028, when the unitarity of the KMC matrix is taken into account. This complements the muon-decay data, increasing the lower bound on M2 to 496 GeV.

  14. Intestinal Pseudo-Obstruction as an Initial Manifestation of Systemic Lupus Erythematosus

    PubMed Central

    Oh, Dong Jun; Yang, Jae Nam; Kang, Ji Hyuk; Park, Jung Hyun; Kim, Mal Young

    2015-01-01

    Intestinal pseudo-obstruction (IPO) is an uncommon, severe complication that occurs in a small subgroup of patients with systemic lupus erythematosus (SLE). To our knowledge, approximately 30 cases of IPO in SLE have been reported in the literature. Moreover, IPO is rare as an initial manifestation of SLE. We report a case of a 43-year-old woman with SLE who initially presented with IPO. PMID:26131004

  15. CLCA4 variants determine the manifestation of the cystic fibrosis basic defect in the intestine

    PubMed Central

    Kolbe, Ernst-Wolfgang; Tamm, Stephanie; Hedtfeld, Silke; Becker, Tim; Tümmler, Burkhard; Stanke, Frauke

    2013-01-01

    The manifestation of the monogenic disease cystic fibrosis results from the cystic fibrosis transmembrane conductance regulator (CFTR)-mediated basic defect defined as an altered chloride transport. An association study using contrasting endophenotypes was conducted with 17 markers to allow fine-mapping of a previously reported association signal within the CLCA gene cluster. Markers were analyzed for association with the manifestation of the basic defect in the patient population of the European CF Twin and Sibling Study composed of 101 families with a total of 171 patients. The manifestation of the basic defect was associated with markers rs11807298–rs6684219, encompassing the CLCA4 promoter (Praw=0.0013; Pcorr=0.0157). Refined analysis of the CLCA4 association signal among F508del homozygous CF patients who exhibit either no, CFTR-mediated or Ca2+-mediated residual chloride conductance revealed that allele distributions for markers rs11807298–rs113894048–rs6684219 differed significantly among these three patient groups. Our data strongly argue that CLCA4 modulates the capability to express residual chloride secretion in colonic tissue. The latter finding is in consistency with the now favored role of the CLCA proteins in signal transduction in epithelial cells. PMID:23073314

  16. Animal Models That Best Reproduce the Clinical Manifestations of Human Intoxication with Organophosphorus Compounds

    PubMed Central

    Pereira, Edna F. R.; Aracava, Yasco; DeTolla, Louis J.; Beecham, E. Jeffrey; Basinger, G. William; Wakayama, Edgar J.

    2014-01-01

    The translational capacity of data generated in preclinical toxicological studies is contingent upon several factors, including the appropriateness of the animal model. The primary objectives of this article are: 1) to analyze the natural history of acute and delayed signs and symptoms that develop following an acute exposure of humans to organophosphorus (OP) compounds, with an emphasis on nerve agents; 2) to identify animal models of the clinical manifestations of human exposure to OPs; and 3) to review the mechanisms that contribute to the immediate and delayed OP neurotoxicity. As discussed in this study, clinical manifestations of an acute exposure of humans to OP compounds can be faithfully reproduced in rodents and nonhuman primates. These manifestations include an acute cholinergic crisis in addition to signs of neurotoxicity that develop long after the OP exposure, particularly chronic neurologic deficits consisting of anxiety-related behavior and cognitive deficits, structural brain damage, and increased slow electroencephalographic frequencies. Because guinea pigs and nonhuman primates, like humans, have low levels of circulating carboxylesterases—the enzymes that metabolize and inactivate OP compounds—they stand out as appropriate animal models for studies of OP intoxication. These are critical points for the development of safe and effective therapeutic interventions against OP poisoning because approval of such therapies by the Food and Drug Administration is likely to rely on the Animal Efficacy Rule, which allows exclusive use of animal data as evidence of the effectiveness of a drug against pathologic conditions that cannot be ethically or feasibly tested in humans. PMID:24907067

  17. Equine phacoclastic uveitis: the clinical manifestations, light microscopic findings, and therapy of 7 cases.

    PubMed Central

    Grahn, B H; Cullen, C L

    2000-01-01

    This retrospective clinical study describes the clinical manifestations, light microscopic findings, and diagnosis and treatment of acute and chronic lens rupture in the horse. Rupture of the lens capsule in the horse usually results in a chronic, blinding inflammation (phacoclastic uveitis) unless prompt surgical and medical therapies are implemented. The clinical manifestations of acute lens capsule rupture included: cataract; intralenticular displacement of iridal pigment; lens cortical fragments attached to the perforated lens capsule, iris, and corneal endothelium; miosis; aqueous flare; and usually a corneal or scleral perforation with ulceration or focal full thickness corneal edema and scarring. The clinical signs of chronic phacoclastic uveitis include blindness, phthisis bulbi, and generalized corneal opacification related to scarring, vascularization, pigmentation, and edema. In one horse, acute phacoclastic uveitis was successfully treated with phacoemulsification to remove the ruptured lens and medical therapy to control the accompanying inflammation. The affected eyes of the horses with chronic phacoclastic uveitis were enucleated because of persistent clinical signs of nonulcerative keratitis and uveitis, despite long-term medical management. The clinical manifestations and lack of improvement with medical therapy are similar in the horse, dog, cat, and rabbit. However, the histologic findings in equine phacoclastic uveitis differ significantly from those in the dog, and rabbit. Images Figure 1. Figure 2. Figure 3. Figure 4. Figure 5. Figure 6. Figure 7. Figure 8. PMID:10816830

  18. ?? T cells regulate the expression of cytokines but not the manifestation of fungal keratitis.

    PubMed

    He, Siyu; Zhang, Hongmin; Liu, Susu; Liu, Hui; Chen, Guoming; Xie, Yanting; Zhang, Junjie; Sun, Shengtao; Li, Zhijie; Wang, Liya

    2015-06-01

    As an important immunoregulatory cell type, the role of ?? T cells in fungal keratitis (FK) is unclear. We observed the distribution of ?? T cells in infected corneas in vivo by two-photon microscopy. The ?? T cells were depleted by neutralizing antibodies. The cytokine expression profile was obtained by protein arrays to determine the cytokines regulated by ?? T cells. ICAM-1, MIP-2 and IL-17A were evaluated by ELISA assays to confirm the role of ?? T cells in FK. We counted the number of neutrophils, evaluated the volume of fungal hyphae and analyzed the manifestation of the disease. The ?? T cells increased significantly at 36 h and 72 h post fungal infection (P < 0.05) and migrated from the limbus to the infection site. The neutralizing antibodies completely depleted the ?? T cells in 24 h. The depletion of ?? T cells led to up regulation of 25 cytokines and down regulation of 3 cytokines. ICAM-1, MIP-2 and IL-17A changed significantly because of the depletion of ?? T cells (P < 0.05). However, the number of neutrophils, volume of fungal hyphae and manifestation of the disease was not affected by the depletion of ?? T cells. Our results demonstrated that ?? T cells have a role in FK via regulation of some cytokines but did not affect the manifestation of this disease, suggesting that ?? T cells are not the key regulator cells in this disease. PMID:25864785

  19. Dietary Fish Oil Blocks the Microcirculatory Manifestations of Ischemia- Reperfusion Injury in Striated Muscle in Hamsters

    NASA Astrophysics Data System (ADS)

    Lehr, Hans-Anton; Hubner, Christoph; Nolte, Dirk; Kohlschutter, Alfried; Messmer, Konrad

    1991-08-01

    Epidemiologic observations and experimental studies have demonstrated a protective effect of dietary fish oil on the clinical manifestations of ischemia-reperfusion injury. To investigate the underlying mechanisms, we used the dorsal skinfold chamber model for intravital fluorescence microscopy of the microcirculation in striated muscle of awake hamsters. In control hamsters (n = 7), reperfusion after a 4-hr pressure-induced ischemia to the muscle tissue elicited the adhesion of fluorescently stained leukocytes to the endothelium of postcapillary venules, capillary obstruction, and the breakdown of endothelial integrity. These microvascular manifestations of ischemia-reperfusion injury were significantly attenuated in animals (n = 7) when fed with a fish oil-enriched diet for 4 weeks prior to the experiments. In leukocyte total lipids, the fish oil diet resulted in a substantial displacement of arachidonic acid, the precursor of the potent adhesionpromoting leukotriene (LT) B_4, by fish oil-derived eicosapentaenoic acid, the precursor of biologically less potent LTB_5, emphasizing the mediator role of LTB_4 in ischemia-reperfusion injury. These results suggest that the preservation of microvascular perfusion by dietary fish oil contributes to its protective effects on the clinical manifestations of ischemia-reperfusion injury.

  20. Neurological manifestations of atrio-esophageal fistulas from left atrial ablation.

    PubMed

    Finsterer, J; Stöllberger, C; Pulgram, T

    2011-10-01

    Left atrial ablation (LAAB) is carried out as an alternative treatment of atrial fibrillation refractory to conservative measures. An increasingly recognized complication of LAAB is an atrio-esophageal fistula (AEF). Neurological manifestations usually dominate the presentation of an AEF and result from cardiac embolism of thrombi, transgressed esophageal contents, or air. AEFs have been reported after LAAB in 52 patients (23 men, age 35-76 years), described in 24 publications, so far. AEFs manifest clinically on the average 17 days (3-38 days) after ablation. Neurological manifestations include embolic strokes (n = 30), seizures (n = 9), transitory-ischaemic attacks (n = 6), coma (n = 6), or psychiatric abnormalities (n = 5). Imaging studies of the cerebrum most frequently show multiple embolic strokes or air embolism. The diagnosis is made upon thoracic computed tomography with contrast media. An act of swallowing, trans-esophageal echocardiography, gastroscopy, or esophageal stenting must be avoided not to enlarge the shunt. The only expedient therapy is surgical closure of the fistula, but even then, the prognosis is poor with a mortality of 71%. AEFs should be suspected if there is a history of LAAB followed by fever, thoracic pain, postprandial cerebral strokes, seizures, coma, or confusion with a latency of days to 5 weeks after ablation. Diagnostic work-up must avoid measures, which enlarge the fistula. Treatment is surgical exclusively. PMID:21426444

  1. Predictors of HIV/AIDS Related Ocular Manifestations among HIV/AIDS Patients in Felege Hiwot Referral Hospital, Northwest Ethiopia

    PubMed Central

    Sharew, Guadie

    2015-01-01

    Background. Ocular manifestations in people living with HIV/AIDS are varied and affect almost all the structures of eye leading to visual impairment or blindness. Therefore, the aim of this study was to identify the predictors of HIV related ocular manifestation among ART clinic clients. Methods. Institution based cross-sectional study was employed among ART clients at Felege Hiwot referral hospital, northwest Ethiopia. The study was conducted from 1 January 2013 to 30 January 2013. A total of 369 systematically and randomly selected clients were included in the study. Data were collected using structured questionnaires and ophthalmologic clinical examination. Data were entered and analyzed using SPSS version 16.0. Binary and multivariable logistic regression analyses were computed to identify independent predictors of HIV related ocular manifestation. Results. Twenty-five percent (25.7%) of HIV patients had ocular manifestations. The three most frequent signs were Squamoid Conjuctival growth (26.9%), ophthalmic herpes zoster (22.1%), and Bacterial Conjuctivitis (17.2%). History of eye problem, CD4 count, and visual acuity of the eye were the predictors of HIV related ocular manifestation. Conclusion. In this study, a higher proportion of ocular manifestations were detected in HIV/AIDS patients. Visual acuity and CD4 counts were the independent predictors of ocular manifestations. This finding gives an insight for policy makers and concerned body to integrate ophthalmic examination in ART clinics to improve the health condition of HIV/ADIS patients. PMID:26000175

  2. EPIDEMIOLOGY, CLINICAL MANIFESTATIONS, AND DIAGNOSIS OF CHIKUNGUNYA FEVER: LESSONS LEARNED FROM THE RE-EMERGING EPIDEMIC

    PubMed Central

    Mohan, Alladi; Kiran, DHN; Manohar, I Chiranjeevi; Kumar, D Prabath

    2010-01-01

    Chikungunya fever, caused by “Chikungunya virus,” is an arbovirus disease transmitted by the bite of infected mosquitoes belonging to the genus Aedes. Chikungunya fever epidemics have been reported from several countries around the world. The disease that was silent for nearly 32 years re-emerged in the October 2005 outbreak in India that is still ongoing. The incubation period ranges from 3 to 12 days. The onset is usually abrupt and the acute stage is characterized by sudden onset with high-grade fever, severe arthralgias, myalgias, and skin rash. Swollen tender joints and crippling arthritis are usually evident. In the chronic stage, relapses that include sensation of fever, asthenia, exacerbation of arthralgias, inflammatory polyarthritis, and stiffness may be evident. Neurological, ocular, and mucocutaneous manifestations have also been described. Chronic arthritis may develop in about 15% of the patients. Viral culture is the gold standard for the diagnosis of Chikungunya fever. Reverse transcription polymerase chain reaction and real-time loop-mediated isothermal amplification have also been found to be useful. Serodiagnostic methods for the detection of immunoglobulin M and immunoglobulin G antibodies against Chikungunya virus are more frequently used. Chikungunya is a self-limiting disease; however, severe manifestations such as meningoencephalitis, fulminant hepatitis, and bleeding manifestations may sometimes be life-threatening. Treatment is symptomatic and supportive. Prevention by educating the community and public health officials, vector control measures appear to be the best approach at controlling Chikungunya fever as no commercially available vaccine is available for public use in India for this condition presently. PMID:20418981

  3. Systemic Sclerosis: Current Concepts in Pathogenesis and Therapeutic Aspects of Dermatological Manifestations

    PubMed Central

    Viswanath, Vishalakshi; Phiske, Meghana M; Gopalani, Vinay V

    2013-01-01

    Systemic sclerosis (SSc) is a chronic, multisystem connective tissue disease with protean clinical manifestations. Recent advances in understanding the pathogenic mechanisms have led to development of target-oriented and vasomodulatory drugs which play a pivotal role in treating various dermatological manifestations. An exhaustive literature search was done using Medline, Embase, and Cochrane library to review the recent concepts regarding pathogenesis and evidence-based treatment of salient dermatological manifestations. The concept of shared genetic risk factors for the development of autoimmune diseases is seen in SSc. It is divided into fibroproliferative and inflammatory groups based on genome-wide molecular profiling. Genetic, infectious, and environmental factors play a key role; vascular injury, fibrosis, and immune activation are the chief pathogenic factors. Vitamin D deficiency has been documented in SSc and correlates with the severity of skin involvement. Skin sclerosis, Raynaud's phenomenon (RP) with digital vasculopathies, pigmentation, calcinosis, and leg ulcers affect the patient's quality of life. Immunosuppressives, biologicals, and hematopoietic stem cell transplantation are efficacious in skin sclerosis. Endothelin A receptor antagonists, calcium-channel blockers, angiotensin receptor inhibitors, prostacyclin analogs, and phosphodiesterase type 5 (PDE-5) inhibitors are the mainstay in RP and digital vasculopathies. Pigmentation in SSc has been attributed to melanogenic potential of endothelin-1 (ET-1); the role of ET 1 antagonists and vitamin D analogs needs to be investigated. Sexual dysfunction in both male and female patients has been attributed to vasculopathy and fibrosis, wherein PDE-5 inhibitors are found to be useful. The future concepts of treating SSc may be based on the gene expression signature. PMID:23918994

  4. Mutation in BMPR2 Promoter: A ‘Second Hit’ for Manifestation of Pulmonary Arterial Hypertension?

    PubMed Central

    Ehlken, Nicola; Fischer, Christine; Lichtblau, Mona; Grünig, Ekkehard; Hinderhofer, Katrin

    2015-01-01

    Background Hereditary pulmonary arterial hypertension (HPAH) can be caused by autosomal dominant inherited mutations of TGF-? genes, such as the bone morphogenetic protein receptor 2 (BMPR2) and Endoglin (ENG) gene. Additional modifier genes may play a role in disease manifestation and severity. In this study we prospectively assessed two families with known BMPR2 or ENG mutations clinically and genetically and screened for a second mutation in the BMPR2 promoter region. Methods We investigated the BMPR2 promoter region by direct sequencing in two index-patients with invasively confirmed diagnosis of HPAH, carrying a mutation in the BMPR2 and ENG gene, respectively. Sixteen family members have been assessed clinically by non-invasive methods and genetically by direct sequencing. Results In both index patients with a primary BMPR2 deletion (exon 2 and 3) and Endoglin missense variant (c.1633G>A, p.(G545S)), respectively, we detected a second mutation (c.-669G>A) in the promoter region of the BMPR2 gene. The index patients with 2 mutations/variants were clinically severely affected at early age, whereas further family members with only one mutation had no manifest HPAH. Conclusion The finding of this study supports the hypothesis that additional mutations may lead to an early and severe manifestation of HPAH. This study shows for the first time that in the regulatory region of the BMPR2 gene the promoter may be important for disease penetrance. Further studies are needed to assess the incidence and clinical relevance of mutations of the BMPR2 promoter region in a larger patient cohort. PMID:26167679

  5. Effects of Atmospheric Variability on Ionospheric Manifestations of Earthquakes and Tsunamis

    NASA Astrophysics Data System (ADS)

    Godin, O. A.; Zabotin, N. A.; Zabotina, L.

    2014-12-01

    There is a large and increasing number of ground-based and satellite-borne instruments, which reliably reveal ionospheric manifestations of natural hazards such as large earthquakes, strong tsunamis, and powerful tornadoes. As the focus shifts from detecting the ionospheric features associated with the natural hazards to characterizing the hazards for the purposes of improving early warning systems and contributing to disaster recovery, it becomes imperative to relate quantitatively characteristics of the observed ionospheric disturbances and the underlying natural hazard. The relation between perturbations at the ground level and their ionospheric manifestations is strongly affected by parameters of the intervening atmosphere. In this paper, we employ the ray theory to model propagation of acoustic gravity waves in three-dimensionally inhomogeneous atmosphere. Huygens' wavefront-tracing and Hamiltonian ray-tracing algorithms are used to simulate wave propagation from an earthquake hypocenter through the earth's crust and ocean to the upper atmosphere as well as the generation of atmospheric waves by seismic surface waves and tsunamis. We quantify the influence of temperature stratification and winds, including their seasonal variability, and air viscosity and thermal conductivity on the geometry and amplitude of ionospheric disturbances. Modeling results are verified by comparing observations of the velocity fluctuations at altitudes of 150-160 km by a coastal Dynasonde HF radar system with theoretical predictions of ionospheric manifestations of background infragravity waves in the ocean. Dynasonde radar systems are shown to be a promising means for monitoring acoustic-gravity wave activity and observing ionospheric perturbations due to earthquakes and tsunamis. The effects will be discussed of background ionospheric disturbances and uncertainty in atmospheric parameters on the feasibility and accuracy of retrieval of open-ocean tsunami heights from observations of the ionosphere.

  6. Manifestation of Chaos in Real Complex Systems: Case of Parkinson's Disease

    NASA Astrophysics Data System (ADS)

    Yulmetyev, Renat M.; Demin, Sergey A.; Hänggi, Peter

    In this chapter we present a new approach to the study of manifestations of chaos in real complex system. Recently we have achieved the following result. In real complex systems the informational measure of chaotic chatacter (IMC) can serve as a reliable quantitative estimation of the state of a complex system and help to estimate the deviation of this state from its normal condition. As the IMC we suggest the statistical spectrum of the non-Markovity parameter (NMP) and its frequency behavior. Our preliminary studies of real complex systems in cardiology, neurophysiology and seismology have shown that the NMP has diverse frequency dependence. It testifies to the competition between Markovian and non-Markovian, random and regular processes and makes a crossover from one relaxation scenario to the other possible. On this basis we can formulate the new concept in the study of the manifestation of chaoticity. We suggest the statistical theory of discrete non-Markov stochastic processes to calculate the NMP and the quantitative evaluation of the IMC in real complex systems. With the help of the IMC we have found out the evident manifestation of chaosity in a normal (healthy) state of the studied system, its sharp reduction in the period of crises, catastrophes and various human diseases. It means that one can appreciably improve the state of a patient (of any system) by increasing the IMC of the studied live system. The given observation creates a reliable basis for predicting crises and catastrophes, as well as for diagnosing and treating various human diseases, Parkinson's disease in particular.

  7. Validation of the Revised Children's Manifest Anxiety Scale, Second Edition (RCMAS-2) Scores for Children with Specific Learning Disabilities

    E-print Network

    Raad, Jennifer Marie

    2013-08-31

    Specific learning disabilities are one of the most common and debilitating disorders experienced by children and adolescents, and students with specific learning disabilities may be particularly vulnerable to anxiety. The Revised Children's Manifest...

  8. 19 CFR 122.22 - Electronic manifest requirement for all individuals onboard private aircraft arriving in and...

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ...requirement for all individuals onboard private aircraft arriving in and departing from the United... AIR COMMERCE REGULATIONS Private Aircraft § 122.22 Electronic manifest requirement for all individuals onboard private aircraft arriving in and departing from the...

  9. 19 CFR 122.22 - Electronic manifest requirement for all individuals onboard private aircraft arriving in and...

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ...requirement for all individuals onboard private aircraft arriving in and departing from the United... AIR COMMERCE REGULATIONS Private Aircraft § 122.22 Electronic manifest requirement for all individuals onboard private aircraft arriving in and departing from the...

  10. 19 CFR 122.22 - Electronic manifest requirement for all individuals onboard private aircraft arriving in and...

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ...requirement for all individuals onboard private aircraft arriving in and departing from the United... AIR COMMERCE REGULATIONS Private Aircraft § 122.22 Electronic manifest requirement for all individuals onboard private aircraft arriving in and departing from the...

  11. 19 CFR 122.22 - Electronic manifest requirement for all individuals onboard private aircraft arriving in and...

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ...requirement for all individuals onboard private aircraft arriving in and departing from the United... AIR COMMERCE REGULATIONS Private Aircraft § 122.22 Electronic manifest requirement for all individuals onboard private aircraft arriving in and departing from the...

  12. 19 CFR 122.22 - Electronic manifest requirement for all individuals onboard private aircraft arriving in and...

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ...requirement for all individuals onboard private aircraft arriving in and departing from the United... AIR COMMERCE REGULATIONS Private Aircraft § 122.22 Electronic manifest requirement for all individuals onboard private aircraft arriving in and departing from the...

  13. 40 CFR 266.325 - Are you subject to the manifest and transportation condition in § 266.315(b)?

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ...40 Protection of Environment 26 2010-07-01...to the manifest and transportation condition in § 266...325 Protection of Environment ENVIRONMENTAL PROTECTION...Storage, Treatment, Transportation and Disposal....

  14. 8 CFR 231.2 - Electronic manifest and I-94 requirement for passengers and crew onboard departing vessels and...

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ...applicable to commercial carriers regarding the electronic transmission of departure manifests covering passengers and crew members under section 231 of the Act are set forth in 19 CFR 4.64 (passengers and crew members onboard vessels) and in 19...

  15. 8 CFR 231.2 - Electronic manifest and I-94 requirement for passengers and crew onboard departing vessels and...

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ...applicable to commercial carriers regarding the electronic transmission of departure manifests covering passengers and crew members under section 231 of the Act are set forth in 19 CFR 4.64 (passengers and crew members onboard vessels) and in 19...

  16. 8 CFR 231.2 - Electronic manifest and I-94 requirement for passengers and crew onboard departing vessels and...

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ...applicable to commercial carriers regarding the electronic transmission of departure manifests covering passengers and crew members under section 231 of the Act are set forth in 19 CFR 4.64 (passengers and crew members onboard vessels) and in 19...

  17. 8 CFR 231.2 - Electronic manifest and I-94 requirement for passengers and crew onboard departing vessels and...

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ...applicable to commercial carriers regarding the electronic transmission of departure manifests covering passengers and crew members under section 231 of the Act are set forth in 19 CFR 4.64 (passengers and crew members onboard vessels) and in 19...

  18. 8 CFR 231.2 - Electronic manifest and I-94 requirement for passengers and crew onboard departing vessels and...

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ...applicable to commercial carriers regarding the electronic transmission of departure manifests covering passengers and crew members under section 231 of the Act are set forth in 19 CFR 4.64 (passengers and crew members onboard vessels) and in 19...

  19. Do not forget the stool examination!-cutaneous and gastrointestinal manifestations of Blastocystis sp. infection.

    PubMed

    Bálint, Anita; Dóczi, Ilona; Bereczki, László; Gyulai, Rolland; Sz?cs, Mónika; Farkas, Klaudia; Urbán, Edit; Nagy, Ferenc; Szepes, Zoltán; Wittmann, Tibor; Molnár, Tamás

    2014-04-01

    Blastocystis sp. is one of the most common parasites in the human intestinal tract. This infection commonly is accompanied by diarrhoea and abdominal pain, but extraintestinal symptoms, such as skin lesions, may also accompany the disease. In this study, our aim was to assess the frequency, clinical symptoms and skin manifestations of confirmed positive Blastocystis sp. infections. Data of 80 patients with confirmed positive Blastocystis sp. infections were assessed retrospectively. The average age of the patients was 46.3 years of age (with a range between 13 and 85 years of age). The number of female patients was higher than the number of males (48 vs. 32; 60 vs. 40%). Gastrointestinal and dermatological symptoms and the results of routine biochemical and haematological blood tests of enrolled patients were collected and analyzed. The skin manifestations were analyzed using the data available (including descriptions, photos and histologies). We discovered that 11.25% of our enrolled patients exhibited skin manifestations associated to Blastocystis sp., mainly on the females. The occurrence of Blastocystis sp. was 6% in symptomatic patients who required medical attendance in the time period between 2005 and 2013. Of the 80 patients, 73.75% indicated that they had gastrointestinal symptoms: 40 patients complained of abdominal pain and 17 with blood in their stool, while other symptoms, such as meteorism (15 subjects), weigh loss (8 subjects), perianal pain or itching (6 subjects), passing stool with mucus (5 subjects), vomiting (2 subjects) and fever (2 subjects) were less frequent. The prevalence of abdominal pain in the cohort without skin lesions was higher compared to those patients with skin problems (p?=?0.007). The mean value of C-reactive protein showed elevated levels, but eosinophils were within a normal range. In addition, we did not find significant difference in eosinophilia between patients with vs. without skin manifestations. Thus, we suggest that eosinophilia is not an obligatory laboratory finding in protozoon infections, such as Blastocystis sp. In the light of our results, we suggest a stool parasite examination for patients with skin lesions of unknown origin. PMID:24553977

  20. Manifestations of dynamo driven large-scale magnetic field in accretion disks of compact objects

    NASA Technical Reports Server (NTRS)

    Chagelishvili, G. D.; Chanishvili, R. G.; Lominadze, J. G.; Sokhadze, Z. A.

    1991-01-01

    A turbulent dynamo nonlinear theory of turbulence was developed that shows that in the compact objects of accretion disks, the generated large-scale magnetic field (when the generation takes place) has a practically toroidal configuration. Its energy density can be much higher than turbulent pulsations energy density, and it becomes comparable with the thermal energy density of the medium. On this basis, the manifestations to which the large-scale magnetic field can lead at the accretion onto black holes and gravimagnetic rotators, respectively, are presented.

  1. Hypoparathyroidism as the First Manifestation of Kearns-Sayre Syndrome: A Case Report

    PubMed Central

    ASHRAFZADEH, Farah; GHAEMI, Nosrat; AKHONDIAN, Javad; BEIRAGHI TOOSI, Mehran; ELMI, Saghi

    2013-01-01

    Objective Kearns-Sayre syndrome is a mitochondrial myopathy, which was first described by Tomas Kearn in 1958. Diagnostic symptoms include retinitis pigmentosa, chronic and progressive external ophthalmoplegia plus one or more of following factors: heart conduction system disorders, cerebellar ataxia, or cerebrospinal fluid (CSF) protein content above 100 mg/dL. The nature of this uncommon disease is yet to be clarified. In this paper, we report a case of Kearns-Sayre syndrome. According to the previous records, the first manifestation of Kearns- Sayre syndrome as hypoparathyroidism is uncommon and in this article, we report a case with this problem. PMID:24665319

  2. Update in the management of extraintestinal manifestations of inflammatory bowel disease.

    PubMed

    Patil, Seema A; Cross, Raymond K

    2013-03-01

    Inflammatory bowel disease, comprised of Crohn's disease and ulcerative colitis, are chronic inflammatory disorders of the gastrointestinal tract. Up to 40 % of patients with inflammatory bowel disease can develop inflammation in other organ systems of the body. These extraintestinal manifestations (EIM) can affect the musculoskeletal, ocular, mucocutaneous, and hepatobiliary systems. Symptoms related to EIM can result in impaired quality of life, and complications of EIM can lead to disfigurement, functional deficits, and even life-threatening organ dysfunction. Some EIM parallel the activity of IBD, and respond to treatment of the underlying disease. Others, however, follow an independent course and require targeted treatment. PMID:23371321

  3. Chronic Progressive External Ophthalmoplegia – A Common Ocular Manifestation of Mitochondrial Disorders

    Microsoft Academic Search

    Marcus Deschauer; Stephan Zierz

    ? Extraocular muscles are predominantly affected in mitochondrial myopathies resulting in chronic progressive external ophthalmoplegia\\u000a (CPEO). \\u000a \\u000a ? CPEO is one of the most common manifestations of mitochondrial disorders and can present as an isolated disorder or as part\\u000a of syndromes with multisystemic involvement. Frequently patients suffer from exercise intolerance or proximal limb weakness.\\u000a \\u000a \\u000a \\u000a \\u000a ? The underlying pathomechanisms are alterations of

  4. Unusual Neurologic Manifestations of a Patient with Cyanotic Congenital Heart Disease after Phlebotomy

    PubMed Central

    Salimipour, Hooman; Mehdizadeh, Somayeh; Nemati, Reza; Pourbehi, Mohamad Reza; Pourbehi, Gholam Reza

    2015-01-01

    Secondary erythrocytosis in cyanotic congenital heart disease (CCHD) is a compensatory response to chronic hypoxia which should be managed with caution. CCHD patients, who have compensated erythrocytosis but do not manifest significant neurologic symptoms, may experience secondary life-threatening complications such as stroke in case of inappropriate phlebotomy. This study reports a young man with CCHD who developed frequently repeated transient neurologic deficits with various presentations after one session of phlebotomy. The symptoms resolved a few days after the hematocrit (Hct) level returned to the prephlebotomy level. PMID:25922773

  5. Gastrointestinal manifestations of Henoch-Schonlein purpura: A report of two cases

    PubMed Central

    Prathiba Rajalakshmi, Parameswaran; Srinivasan, Kalyanasundaram

    2015-01-01

    Henoch-Schonlein purpura (HSP) is a small vessel vasculitis mediated by type III hypersensitivity with deposition of IgA immune complex in the walls of vessels. It is a multi-system disorder characterized by palpable purpura, arthritis, glomerulonephritis and gastrointestinal manifestations and commonly occurs in children and young adults. The patients with gastrointestinal involvement usually present with colicky abdominal pain, vomiting and melena. The imaging findings include multifocal bowel thickening with mucosal hyperenhancement, presence of skip areas, mesenteric vascular engorgement, with involvement of unusual sites like stomach, duodenum and rectum. These imaging findings in a child or young adult with appropriate clinical findings could suggest HSP. PMID:25825636

  6. Post-operative quadriplegia as the initial manifestation of tumefactive multiple sclerosis

    PubMed Central

    Maghsoudi, Behzad; Haddad, Hossein; Vatankhah, Pooya; Rasekhi, Alireza; Jaberi, Abbas Rahimi

    2015-01-01

    Post-operative quadriplegia is a rarely encountered complication and not previously reported as the initial presentation of tumefactive multiple sclerosis. We present an unusual case of a patient with such manifestation and atypical lesions on brain magnetic resonance imaging. The patient was treated with methyl prednisolone pulse therapy and showed a dramatic response. Uncommon neurologic diseases can have very unusual presentations, which should be taken into consideration when encountered with such patients. Considering this fact will help physicians in better decision-making and proper treatment planning. PMID:26195864

  7. Pulmonary necrobiotic nodules in Crohn's disease: a rare extra-intestinal manifestation.

    PubMed

    El-Kersh, Karim; Fraig, Mostafa; Cavallazzi, Rodrigo; Saad, Mohamed; Perez, Rafael L

    2014-12-01

    Pulmonary necrobiotic nodules represent a rare extra-intestinal manifestation of Crohn's disease. Histologically, they are composed of sterile aggregates of inflammatory cells with necrosis. The differential diagnosis is broad, and exclusion of infectious etiologies is mandatory before starting immunosuppressive therapy. Here, we present the fifth reported case of pulmonary necrobiotic nodules in Crohn's disease. Our patient had new-onset Crohn's disease associated with both cavitating and non-cavitating lung nodules that were confirmed to be necrobiotic nodules by biopsy. The patient was started on mesalamine and prednisone, with subsequent improvement of his gastrointestinal symptoms and regression of the necrobiotic nodules. PMID:25233391

  8. Fractal properties of the sea surface manifested in microwave remote sensing signatures

    NASA Technical Reports Server (NTRS)

    Glazman, R. E.

    1988-01-01

    The wave spectrum of a well developed sea is discussed. It includes a broad range of wavenumbers where the spectral density is governed by a power law of the form k sup-p. When p is less than or = 4, the surface exhibits properties, such as an increased surface number density of steep and breaking wavelet events and an increased number of specular points for vertical incidence, due to the cascade (fractal) pattern in its geometry. These properties manifest themselves in error trends in wind speed measurements by scatterometer and altimeter.

  9. Effect of Kuberaksha Patra Churna, Vriddhadaru Mula Churna and Kandughna Taila in Shlipada (Manifested filariasis)

    PubMed Central

    Prasad, Goli Penchala; Naidu, M. L.

    2012-01-01

    At present there are effective drugs in eradicating microfilariae but treatments to control the progression of manifested filariasis, periodic adenolymphangitis (ADL) and lymphedema are not available in conventional system of medicine. So far National Ayurveda Research Institute for Vector-borne diseases, Vijayawada, has conducted many clinical trails on manifested filariasis patients with the classical Ayurvedic herbal, herbo-mineral drugs and found significant results on ADL, lymphedema and other acute and chronic clinical manifestations. An effort has been made to find the effect of Kuberaksha Patra Churna [Caesalpinia bonduc (L.) Roxb.], Vriddhadaru Mula Churna [Argyreia nervosa (Burm.f.) Boj.] and Kandughna Taila (oil prepared from 10 Ayurvedic drugs) in manifested filarial patients. Based on inclusion criteria 133 patients were included in three groups (45 in Gr.I, 45 in Gr.II and 43 in Gr.III) and 120 patients completed the study (40 in each group). In Gr. I Argyreia nervosa (Burm.f.) Boj. root powder, Caesalpinia bonduc (L.) Roxb. leaf powder mixed equally was given in the dose of 5 g twice a day for 30 days. In Gr.II along with Gr. I internal drugs Kandughna Taila was applied externally in sufficient quantity once a day for 30 days. Gr. III is a control study with Ayurvedic established drug ‘Nityananda Rasa’ 1 tablet thrice daily for 30 days. Group I and II drugs showed highly significant effect on lymphedema, lymphadenitis, lymphangitis, pain, tenderness, heaviness, deformity, fever and rigors (P<0.0001). Group III drug showed highly significant (P<0.0001) effect on lymphedema, deformity and heaviness; statistically significant (P=0.0018) on pain and tenderness; Significant effect on fever (P=0.0290), rigor (P=0.0290) and in lymphangitis (P=0.0384) and non-significant effect on lymphadenitis (P=0.1033). On statistical analysis effect of treatment on Hb and eosinophil count was found non-significant in three groups. On ESR, effect of treatment was found significant in Gr. III and non-significant in Gr. I and Gr. II. PMID:23049193

  10. Dent’s disease manifesting as focal glomerulosclerosis: Is it the tip of the iceberg?

    Microsoft Academic Search

    Yaacov Frishberg; Dganit Dinour; Ruth Belostotsky; Rachel Becker-Cohen; Choni Rinat; Sofia Feinstein; Paulina Navon-Elkan; Efrat Ben-Shalom

    2009-01-01

    Dent’s disease is an X-linked proximal tubulopathy. It often manifests in childhood with symptoms of Fanconi syndrome and\\u000a low-molecular-weight proteinuria. We describe four boys from three unrelated families whose only presenting symptoms of Dent’s\\u000a disease were nephrotic-range proteinuria and histological findings of focal segmental and\\/or global glomerulosclerosis. In\\u000a all families, a causal mutation in the CLCN5 gene, encoding a voltage-gated

  11. Polyarteritis nodosa and extrahepatic manifestations of HBV infection: the case against autoimmune intervention in pathogenesis.

    PubMed

    Trepo, C; Guillevin, L

    2001-05-01

    Numerous extrahepatic manifestations have been reported in patients with both acute and chronic hepatitis B (arthralgias or arthritis, skin rashes, glomerulonephritis and neuritis), all of which are present in polyarteritis nodosa (PAN) which is the most unique and spectacular extrahepatic manifestation. In the 1970s, the frequency of PAN due to the hepatitis B (HBV) reached 30%. Immunization programs explain the decrease and it is now down to 7%. PAN usually occurs within 6 months of infection. Clinical manifestations reflect this most classic form of PAN, Hepatic manifestations including, ALT/AST elevations are mild and usually overlooked. Besides HBV, other viruses may be responsible for cases of vasculitides including PAN, HIV, Parvovirus B19, and EBV. Different pathogenic mechanisms have been identified but immune complexes are mainly thought to be responsible. In glomerulonephritis, detailed immunostaining and ultrastructural findings indicate that HBe antigen (Ag) is more likely to be the responsible antigen. In PAN, fewer reports are available and early studies with poorly defined antibodies need to be revisited. Interestingly almost all cases of HBV/PAN are associated with wild-type HBV infection, characterised by HBe antigenemia and high HBV replication, supporting the concept that lesions could result from the deposit of viral Ag/Ab complexes soluble in Ag excess, possibly involving HBe Ag. The recent observation of PAN cases associated with precore mutation which abrogates the formation of HBe Ag challenges this view. It may suggest that other, still undefined, circulating HBV-related Ag(s) distinct from HBe Ag could be involved. Remarkably, none of the HBV/PAN cases or glomerulonephritis exhibit antineutrophil cycoplasmic antibodies (ANCA) reactivity. Viral PAN can now be completely separated from other form of vasculitis mostly autoimmune in nature. Based on the efficacy of antiviral agents in chronic hepatitis B and of plasma exchanges in PAN we combined both therapies to treat HBV PAN. This was associated with swift recovery, even in the most severe forms. The perfect time correlation between inhibition of virus replication and resolution of all bioclinical signs suggest a direct pathogenic role of the virus possibly via immune complexes. Traditional immunosuppressive and steroid therapy should no longer be used for HBV PAN cases. PMID:11334492

  12. Gastrointestinal manifestations of Henoch-Schonlein purpura: A report of two cases.

    PubMed

    Prathiba Rajalakshmi, Parameswaran; Srinivasan, Kalyanasundaram

    2015-03-28

    Henoch-Schonlein purpura (HSP) is a small vessel vasculitis mediated by type III hypersensitivity with deposition of IgA immune complex in the walls of vessels. It is a multi-system disorder characterized by palpable purpura, arthritis, glomerulonephritis and gastrointestinal manifestations and commonly occurs in children and young adults. The patients with gastrointestinal involvement usually present with colicky abdominal pain, vomiting and melena. The imaging findings include multifocal bowel thickening with mucosal hyperenhancement, presence of skip areas, mesenteric vascular engorgement, with involvement of unusual sites like stomach, duodenum and rectum. These imaging findings in a child or young adult with appropriate clinical findings could suggest HSP. PMID:25825636

  13. Pericardial tamponade and coexisting pulmonary embolism as first manifestation of non-advanced lung adenocarcinoma

    PubMed Central

    Akhbour, Salwa; Khennine, Brahim Amine; Oukerraj, Latifa; Zarzur, Jamila; Cherti, Mohamed

    2014-01-01

    Pericardial effusion and pulmonary embolism are relatively common complications of malignancy and are uncommon as its initial manifestation. This report describes a case of a patient, who presented with this association, due to an underlying pulmonary adenocarcinoma. When a major pericardial effusion is associated with pulmonary hypertension, some echocardiographic signs may redress the diagnosis. This case emphasizes a challenge diagnostic which may be guided by high right ventricular pressure and on the other hand the importance of keeping both these conditions in mind when dealing with context of malignancy. PMID:25360199

  14. Occlusion of the syrinx as a manifestation of aspergillosis in Canada geese

    USGS Publications Warehouse

    Stroud, R.K.; Duncan, R.M.

    1982-01-01

    Aspergillosis has been described in many species of wild waterfowl, primarily as a disease of the respiratory tract. Typically, mycotic granulomas are found in the lungs. Air sacs may be thickened and contain discoid individual or coalescing greenish or bluish plaques resembling bread mold. Occasionally, there is systemic involvement, with granulomas in multiple organs. Carcasses often are emaciated, indicating a long-term course. In the present report, we describe a fatal acute manifestation of Aspergillus fumigatus infection that easily may be overlooked when examining wild waterfowl.

  15. Cutaneous manifestation of silicone dissemination from a PIP implant--a case for prophylactic explantation?

    PubMed

    Cawrse, N H; Pickford, M A

    2011-08-01

    The recent withdrawal of PIP (Poly Implant Prosthese, France) implants for breast augmentation enforced by the Medicines and Healthcare products Regulatory Agency (MHRA) on 31st March 2010 has ignited speculation into possible side effects relating to an unauthorized gel fill content. Local and migratory silicone granulomata and regional lymphadenopathy are well reported in the literature. Gel bleed from high cohesive gel implants with similar effect is also well known. However dissemination to sites distant from the breast manifest as cutaneous abnormalities in a patient implanted with a PIP product raises concern. We report such a case. PMID:21524950

  16. Unusual neurologic manifestations of a patient with cyanotic congenital heart disease after phlebotomy.

    PubMed

    Salimipour, Hooman; Mehdizadeh, Somayeh; Nemati, Reza; Pourbehi, Mohamad Reza; Pourbehi, Gholam Reza; Assadi, Majid

    2015-01-01

    Secondary erythrocytosis in cyanotic congenital heart disease (CCHD) is a compensatory response to chronic hypoxia which should be managed with caution. CCHD patients, who have compensated erythrocytosis but do not manifest significant neurologic symptoms, may experience secondary life-threatening complications such as stroke in case of inappropriate phlebotomy. This study reports a young man with CCHD who developed frequently repeated transient neurologic deficits with various presentations after one session of phlebotomy. The symptoms resolved a few days after the hematocrit (Hct) level returned to the prephlebotomy level. PMID:25922773

  17. Ocular manifestations of syphilis: recent cases over a 2.5-year period

    Microsoft Academic Search

    Cindy Puech; Stéphane Gennai; Patricia Pavese; Isabelle Pelloux; Max Maurin; Jean-Paul Romanet; Christophe Chiquet

    2010-01-01

    Background  The ocular manifestations of syphilis are protean and can affect every structure of the eye. There has been a recent increase\\u000a of syphilis infection in Europe. We report recent cases of ocular syphilis infection in a tertiary center.\\u000a \\u000a \\u000a \\u000a \\u000a Methods  During a 2.5-year period (2005–2007) we collected the medical records of eight male patients with ocular syphilis. The diagnosis\\u000a was based on

  18. [Ophthalmological manifestations of Cornelia de Lange syndrome : Case report and review of the literature].

    PubMed

    Avgitidou, G; Cursiefen, C; Heindl, L M

    2015-05-01

    A 2-year-old boy suffering from Cornelia de Lange syndrome, presented with mucopurulent ocular discharge and epiphora since birth. Irrigation and probing of the nasolacrimal system revealed and successfully treated bilateral nasolacrimal duct obstructions. Cornelia de Lange syndrome is characterized not only by typical facial features, visceral and urogenital anomalies but also by ophthalmological manifestations in 99?% of cases. The most common ophthalmological disorders are synophrys, blepharitis, epiphora, hypertrichosis of the eyebrows and eyelashes, myopia, ptosis and nasolacrimal duct obstruction. PMID:25566737

  19. Speech-Language and swallowing manifestations and rehabilitation in an 11-year-old girl with MELAS syndrome

    PubMed Central

    Vandana, V. P.; Bindu, Parayil Sankaran; Sonam, Kothari; Taly, Arun B.; Gayathri, N.; Madhu, N.; Sinha, S.

    2015-01-01

    Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a rare mitochondrial disease. The available studies on MELAS syndrome are limited to evaluation of radiological, audiological, genetic, and neurological findings. Among the various neurological manifestations, speech-language and swallowing manifestations are less discussed in the literature. This report describes the speech-language and swallowing function in an 11-year-old girl with MELAS syndrome. The intervention over a period of 6 months is discussed. PMID:25878740

  20. Secondary neuropsychiatric manifestations caused by Epstein-Barr virus encephalitis in a new onset systemic lupus erythematosus patient.

    PubMed

    Hongbo, Chen; Hongzhen, Ma; Lingzhi, He; Maosheng, Xu; Mei, Chen

    2012-08-01

    Abnormal manifestations of central nervous system in system lupus erythematosus (SLE) patients are mainly caused by primary neuropsychiatric SLE (NP-SLE). We reported a new onset SLE patient who had secondary neuropsychiatric manifestations caused by Epstein-Barr virus (EBV) encephalitis. Although EBV has an uncertain association with SLE, Epstein-Barr virus encephalitis occurred in active SLE patients was not reported previously. Our report may be the first case about EBV encephalitis occurred in active SLE. PMID:21607560