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https://www.readbyqxmd.com/read/29467115/the-pancreatic-and-extrapancreatic-manifestations-of-igg4-related-disease
#1
Jaspreet Singh Sangha Brar, Saurav Gupta, Salahudeen M Haja Mohideen, Lishya Liauw, Narayan Lath
The association between immunoglobulin IgG4 and autoimmune pancreatitis was first shown in 2001. Since then many previously established fibrosclerotic diseases demonstrating synchronous or metachronous multiorgan involvement have been included within the ambit of IgG4-related disease. Diagnostic criteria have been proposed involving 1) serum IgG4 level elevated beyond 135 mg/dL, 2) IgG4+ to IgG+ plasma cell ratio >40% and >10 IgG4+ cells per high power field of biopsy sample and 3) a constellation of imaging features which involve a variety of organ systems...
February 21, 2018: Diagnostic and Interventional Radiology: Official Journal of the Turkish Society of Radiology
https://www.readbyqxmd.com/read/29466428/hemagglutinin-specific-neutralization-of-subacute-sclerosing-panencephalitis-viruses
#2
Miguel Ángel Muñoz-Alía, Claude P Muller, Stephen J Russell
Subacute sclerosing panencephalitis (SSPE) is a progressive, lethal complication of measles caused by particular mutants of measles virus (MeV) that persist in the brain despite high levels of neutralizing antibodies. We addressed the hypothesis that antigenic drift is involved in the pathogenetic mechanism of SSPE by analyzing antigenic alterations in the MeV envelope hemagglutinin protein (MeV-H) found in patients with SSPE in relation to major circulating MeV genotypes. To this aim, we obtained cDNA for the MeV-H gene from tissue taken at brain autopsy from 3 deceased persons with SSPE who had short (3-4 months, SMa79), average (3...
2018: PloS One
https://www.readbyqxmd.com/read/29465558/analysis-of-clinical-characteristics-and-treatment-of-immunoglobulin-g4-associated-cholangitis-a-retrospective-cohort-study-of-39-iac-patients
#3
Jianchun Xiao, Peiran Xu, Binglu Li, Tao Hong, Wei Liu, Xiaodong He, Chaoji Zheng, Yupei Zhao
Immunoglobulin (Ig)G4-associated cholangitis (IAC) is one of the common organ manifestations of IgG4-related systemic disease (ISD). IAC and autoimmune pancreatitis (AIP) may mimic sclerosing cholangitis, cholangiocarcinoma, or pancreatic carcinoma. Diagnosis is based on a combination of clinical, biochemical, radiological, and histological findings.To study the clinical presentation of and treatment strategy for IAC, we reviewed clinical, serologic, and imaging characteristics, as well as treatment response, in 39 patients with IAC...
February 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29463726/structures-of-the-prefusion-form-of-measles-virus-fusion-protein-in-complex-with-inhibitors
#4
Takao Hashiguchi, Yoshinari Fukuda, Rei Matsuoka, Daisuke Kuroda, Marie Kubota, Yuta Shirogane, Shumpei Watanabe, Kouhei Tsumoto, Daisuke Kohda, Richard Karl Plemper, Yusuke Yanagi
Measles virus (MeV), a major cause of childhood morbidity and mortality, is highly immunotropic and one of the most contagious pathogens. MeV may establish, albeit rarely, persistent infection in the central nervous system, causing fatal and intractable neurodegenerative diseases such as subacute sclerosing panencephalitis and measles inclusion body encephalitis. Recent studies have suggested that particular substitutions in the MeV fusion (F) protein are involved in the pathogenesis by destabilizing the F protein and endowing it with hyperfusogenicity...
February 20, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29431255/short-term-side-effects-and-patient-reported-outcomes-of-bleomycin-sclerotherapy-in-vascular-malformations
#5
Joana M Mack, Gresham T Richter, David Becton, Omar Salem, Sarah E M Hill, Shelley E Crary
BACKGROUND: Vascular malformations (VM) are congenital lesions that can be debilitating and cause significant aesthetic and functional limitations. The chemotherapeutic agent bleomycin has been utilized as a sclerosant, directly injected percutaneously into the VM. Unfortunately, little is known about the benefits and short-term side effects of bleomycin with intralesional injections. PROCEDURE: An IRB approved, retrospective chart review was performed on patients with VM who had been treated with intralesional bleomycin...
February 12, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29396158/an-institution-wide-algorithm-for-direct-stick-embolization-of-peripheral-venous-malformations
#6
Naiem Nassiri, Lauren A Huntress, Mitchell Simon, Susan Murphy
OBJECTIVE: No standardized therapeutic algorithm or embolic agent of choice has yet been identified for management of congenital peripheral venous malformations (VMs). Treatment options and reported outcomes therefore vary widely. Herein, we present an institution-wide algorithm for management of symptomatic congenital peripheral VMs using a single embolotherapeutic modality. METHODS: During 36 months, patients with symptomatic congenital peripheral VMs underwent contrast-enhanced magnetic resonance imaging...
January 29, 2018: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/29386816/aqueous-intralesional-bleomycin-sclerotherapy-in-lymphatic-malformation-our-experience-with-children-and-adult
#7
Ankur Bhatnagar, Vijai Datta Upadhyaya, Basant Kumar, Zafar Neyaz, Ajay Kushwaha
Objectives: Lymphatic malformations (LMs) are aberrant proliferation of sequestrated lymphatic vessels during early embryogenesis and do not communicate directly with the general lymphatic system. The absence of vascular flow is the hallmark of LMs and is usually symptomless apart from painless disfiguring mass with concerns regarding cosmesis. Design: Sclerotherapy has gained prominence as a preferred treatment modality for macrocystic lesions. Here, we present our experience with use of aqueous bleomycin as intralesional sclerosing agent, an economical first-line treatment for macrocystic variant of LMs in children and adults...
July 2017: National Journal of Maxillofacial Surgery
https://www.readbyqxmd.com/read/29356767/lack-of-correlation-of-liver-tests-with-fibrosis-stage-at-diagnosis-in-pediatric-primary-sclerosing-cholangitis
#8
Jillian M Cotter, Lorna P Browne, Kelley E Capocelli, Annette McCoy, Cara L Mack
OBJECTIVES: The aims of this study were to characterize pediatric primary sclerosing cholangitis (PSC) at a regional referral-based institution, including scoring of biliary stricturing and liver fibrosis and correlation analyses of scores with serum liver tests, to identify biomarkers of disease severity. METHODS: A retrospective review of 39 PSC subjects was performed, with collection of demographic and outcomes data. Magnetic resonance cholangiopancreaticogram (MRCP) and liver biopsies were re-reviewed and scores of stricturing and fibrosis were correlated with serum liver tests...
February 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29332510/localized-autoimmune-pancreatitis-mimicking-pancreatic-cancer-case-report-and-literature-review
#9
Wen-Ling Hsu, Shu-Min Chang, Pei-Yin Wu, Chin-Chuan Chang
Autoimmune pancreatitis (AP) is a rare autoimmune pancreatic manifestation of systemic immunoglobulin G4 (IgG4)-related sclerosing disease. Distinguishing between AP and pancreatic cancer is crucial because the clinical courses, treatments, and prognoses of these two disease entities are quite different. We herein report a case involving a 52-year-old man with subacute epigastralgia who visited our hospital for evaluation of a suspicious pancreatic mass found during esophagogastroduodenoscopy. Enhanced computed tomography (CT) revealed an enlarged lesion in the pancreatic head with encasement of hepatic vessels...
January 1, 2018: Journal of International Medical Research
https://www.readbyqxmd.com/read/29312864/risk-factors-and-clinical-indicators-for-the-development-of-biliary-strictures-post-liver-transplant-significance-of-bilirubin
#10
Elizabeth Ann Forrest, Janske Reiling, Geraldine Lipka, Jonathan Fawcett
AIM: To identify risk factors associated with the formation of biliary strictures post liver transplantation over a period of 10-year in Queensland. METHODS: Data on liver donors and recipients in Queensland between 2005 and 2014 was obtained from an electronic patient data system. In addition, intra-operative and post-operative characteristics were collected and a logistical regression analysis was performed to evaluate their association with the development of biliary strictures...
December 24, 2017: World Journal of Transplantation
https://www.readbyqxmd.com/read/29298883/cell-to-cell-measles-virus-spread-between-human-neurons-dependent-on-the-hemagglutinin-and-the-hyperfusogenic-fusion-protein
#11
Yuma Sato, Shumpei Watanabe, Yoshinari Fukuda, Takao Hashiguchi, Yusuke Yanagi, Shinji Ohno
Measles virus (MV) usually causes acute infection, but in rare cases persists in the brain, resulting in subacute sclerosing panencephalitis (SSPE). Since human neurons, an important target affected in the disease, do not express the known MV receptors (signaling lymphocyte activation molecule (SLAM) and nectin 4), how MV infects neurons and spreads between them is unknown. Recent studies have shown that many virus strains isolated from SSPE patients possess substitutions in the extracellular domain of the fusion (F) protein which confer enhanced fusion activity...
January 3, 2018: Journal of Virology
https://www.readbyqxmd.com/read/29283443/letter-on-european-dermatology-forum-s1-guideline-on-the-diagnosis-and-treatment-of-sclerosing-diseases-of-the-skin-part-2-scleromyxedema-scleredema-and-nephrogenic-systemic-fibrosis
#12
Patrick Mark, Ilona Dekkers, Peter Blankestijn, Tim Leiner, Giles Roditi
We read with interest the guidelines recently published on sclerosing diseases of the skin (Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis)[1, 2]. However, we are concerned that the guideline recommendations proposed for prevention of nephrogenic systemic fibrosis (NSF) are potentially dangerous. Although we recognise the challenges in constructing comprehensive guidelines, we are concerned that this may be because the guidelines have not involved a multidisciplinary team. This article is protected by copyright...
December 28, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29280826/an-unusual-cause-of-dysphagia
#13
Soumya Chatterjee, Brian J Hedman, Donald F Kirby
Scleredema adultorum of Buschke is a rare scleroderma mimic characterized by woody induration of the skin involving the back of the neck, interscapular region, upper and mid back, and sometimes the face, upper chest, and upper arms. Dysphagia is rare and not well characterized. We describe a case of scleredema that developed significant dysphagia affecting both the oropharyngeal phase and the esophageal phase of deglutition. Diagnostic evaluation revealed mild-moderate oropharyngeal and severe esophageal dysmotility...
December 27, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29258587/association-between-mammographic-breast-density-and-histologic-features-of-benign-breast-disease
#14
Karthik Ghosh, Robert A Vierkant, Ryan D Frank, Stacey Winham, Daniel W Visscher, Vernon S Pankratz, Christopher G Scott, Kathleen Brandt, Mark E Sherman, Derek C Radisky, Marlene H Frost, Lynn C Hartmann, Amy C Degnim, Celine M Vachon
BACKGROUND: Over 40% of women undergoing breast screening have mammographically dense breasts. Elevated mammographic breast density (MBD) is an established breast cancer risk factor and is known to mask tumors within the dense tissue. However, the association of MBD with high risk benign breast disease (BBD) is unknown. METHOD: We analyzed data for 3400 women diagnosed with pathologically confirmed BBD in the Mayo Clinic BBD cohort from 1985-2001, with a clinical MBD measure (either parenchymal pattern (PP) or Breast Imaging Reporting and Data Systems (BI-RADS) density) and expert pathology review...
December 19, 2017: Breast Cancer Research: BCR
https://www.readbyqxmd.com/read/29226107/cholangitis-diagnosis-treatment-and-prognosis
#15
REVIEW
Amir Houshang Mohammad Alizadeh
Cholangitis is a serious life-threatening situation affecting the hepatobiliary system. This review provides an update regarding the clinical and pathological features of various forms of cholangitis. A comprehensive search was performed in the PubMed, Scopus, and Web of Knowledge databases. It was found that the etiology and pathogenesis of cholangitis are heterogeneous. Cholangitis can be categorized as primary sclerosing (PSC), secondary (acute) cholangitis, and a recently characterized form, known as IgG4-associated cholangitis (IAC)...
December 28, 2017: Journal of Clinical and Translational Hepatology
https://www.readbyqxmd.com/read/29221270/analysis-of-the-clinical-differentiation-of-pulmonary-sclerosing-pneumocytoma-and-lung-cancer
#16
Jun Zhu
Background: Pulmonary sclerosing pneumocytoma (PSP) is a rare benign lung tumor. This study investigated the diagnostic experience of PSP and lung cancer. Methods: This study is a retrospective study. We observed the locations of lung lesions, imaging form and clinical symptoms, and recorded the surgical complications through comparing patients with PSP and lung cancer. Results: From December 2012 to February 2017, 187 PSP cases and 197 lung cancer cases were collected...
September 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29196804/igg4-related-disease-with-emphasis-on-the-biopsy-diagnosis-of-autoimmune-pancreatitis-and-sclerosing-cholangitis
#17
REVIEW
Sönke Detlefsen, Günter Klöppel
In 2011, chronic fibroinflammatory processes occurring simultaneously or metachronously in various organs and associated with elevated IgG4 serum levels and/or tissue infiltration with IgG4-positive plasma cells have been recognized as manifestations of a systemic disorder called IgG4-related disease (IgG4-RD). The histologic key findings are lymphoplasmacytic infiltration rich in IgG4-positive plasma cells combined with storiform fibrosis and obliterative phlebitis. Among the organs mainly affected by IgG4-RD are the pancreas and the extrahepatic bile ducts...
December 1, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/29141796/high-dose-biotin-as-treatment-for-progressive-multiple-sclerosis
#18
Gary Birnbaum, Jessica Stulc
BACKGROUND: Published data suggested high dose biotin improved patients with progressive MS. We wished to determine benefits and side effects of administering daily high dose biotin to patients with progressive multiple sclerosis in a large MS specialty clinic. METHODS: Forty-three patients with progressive multiple scleroses were prescribed pharmaceutical grade biotin as a single daily dose of 300mg/day. Brain MRIs were performed at baseline and after one year on biotin...
November 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29129978/a-case-of-peripheral-ulcerative-keratitis-associated-with-autoimmune-hepatitis
#19
Hamoon Eshraghi, Aria Mahtabfar, Mohammad H Dastjerdi
Purpose: To describe a case of peripheral ulcerative keratitis in the setting of autoimmune hepatitis and possible overlap syndrome with primary sclerosing cholangitis. Case Report: A 48-year-old African American female with autoimmune hepatitis with possible overlap syndrome with primary sclerosing cholangitis presented with tearing, irritation, and injection of the left eye that was determined to be peripheral ulcerative keratitis. The patient was treated with topical and systemic steroids, immunosuppressant drugs (azathioprine and mycophenolate mofetil), a biologic (rituximab), and surgery (conjunctival resection), and the peripheral ulcerative keratitis epithelialized but ultimately led to corneal perforation...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/29108474/molecular-testing-for-oncogenic-gene-alterations-in-pediatric-thyroid-lesions
#20
Sogol Mostoufi-Moab, Emmanuel Labourier, Lisa Sullivan, Virginia LiVolsi, Yimei Li, Rui Xiao, Sylvie Beaudenon-Huibregtse, Ken Kazahaya, N Scott Adzick, Zubair Baloch, Andrew J Bauer
BACKGROUND: Thyroid nodules are less common in pediatric patients (i.e., those ≤18 years) than they are in adults. The Bethesda System for Reporting Thyroid Cytopathology allows for individual risk stratification, but a significant number of nodules are indeterminate. Incorporating gene mutation panels and gene expression classifiers may aid in preoperative diagnosis. The overall aim of this study was to assess the prevalence of oncogene alterations in a representative pediatric population and across a broad-spectrum of thyroid tumor diagnoses...
January 2018: Thyroid: Official Journal of the American Thyroid Association
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