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Sclerose systemic

Christian Tröbinger, Christian J Wiedermann
OBJECTIVE: To describe the association of bodybuilding abdominal exercise with the development of superficial sclerosing thrombophlebitis of the anterolateral thoracoabdominal wall. DESIGN: A single case study. SETTING: University-affiliated regional community hospital. CASE DESCRIPTION: A 54-year-old man presented with an otherwise unremarkable past medical history 4 weeks after the start of left-sided chest discomfort...
September 4, 2016: Physical Therapy in Sport
Jésus Gonzalez-Bermejo, Capucine Morélot-Panzini, Marie-Laure Tanguy, Vincent Meininger, Pierre-François Pradat, Timothée Lenglet, Gaëlle Bruneteau, Nadine Le Forestier, Philippe Couratier, Nathalie Guy, Claude Desnuelle, Hélène Prigent, Christophe Perrin, Valérie Attali, Catherine Fargeot, Marie-Cécile Nierat, Catherine Royer, Fabrice Ménégaux, François Salachas, Thomas Similowski
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder associated with respiratory muscle weakness and respiratory failure. Non-invasive ventilation alleviates respiratory symptoms and prolongs life, but is a palliative intervention. Slowing the deterioration of diaphragm function before respiratory failure would be desirable. We aimed to assess whether early diaphragm pacing could slow down diaphragm deterioration and would therefore delay the need for non-invasive ventilation...
November 2016: Lancet Neurology
Matheus Bertanha, Paula Angeleli Bueno de Camargo, Regina Moura, Winston Bonetti Yoshida, Rafael Elias Farres Pimenta, Jamil Victor de Oliveira Mariúba, Giovana Piteri Alcantara, Dênia Reis de Paula, Marcone Lima Sobreira
BACKGROUND: Telangiectasias are defined as small venules abnormally dilated, located in the intradermal portion of the skin, of reddish or bluish tinge, their diameter not exceeding 1 mm; they are classified by the American Venous Forum as mild venous disease CEAP C1. Conventional treatment consists of chemical sclerotherapy, considered a minimally invasive technique with rapid clinical recovery. A wide variety of sclerosing solutions can be used for this purpose. METHODS/DESIGN: This project intends to include 96 patients that will be randomized to a triple-blind study...
September 2016: Medicine (Baltimore)
Yumi Ozawa, Shujiro Hayashi, Yoichiro Hamasaki, Atsushi Hatamochi
Nephrogenic systemic fibrosis (NSF) is a disease characterized by fibrosis of the systemic organs in patients with renal failure. Following the findings of recent epidemiological studies and the finding of gadolinium (Gd) in the skin tissue of NSF patients, it is now definitely known that the use of Gd contrast agents can trigger NSF. To date, however, the exact mechanism underlying the induction of fibrosis in various organs by Gd remains unexplained. This study was undertaken to evaluate the influence of Gd on the proliferation activity and collagen production of cultured fibroblasts...
September 28, 2016: Archives of Dermatological Research
B Evranos, S B Polat, H Baser, D Ozdemir, A Kilicarslan, A Yalcin, R Ersoy, B Cakir
BACKGROUND: A fine needle aspiration biopsy (FNAB) is the most valuable diagnostic procedure for pre-operative discrimination of benign and malignant nodules. The Bethesda System for Reporting Thyroid Cytopathology provides standardised reporting and cytomorphological criteria in aspiration smears. The aim of the present study was to determine malignancy rates in nodules with different cytology results and evaluate the diagnostic value of Bethesda for variants of papillary thyroid carcinoma (PTC)...
September 25, 2016: Cytopathology: Official Journal of the British Society for Clinical Cytology
Noelle M Teske, Heidi T Jacobe
Phototherapy is an effective treatment strategy for a variety of sclerosing skin conditions. There are a number of phototherapeutic modalities used for the treatment of sclerosing skin conditions, including ultraviolet (UV)A1, broadband UVA, psoralen plus UVA, and narrowband UVB phototherapy. As controlled trials with validated outcome measures are lacking for these therapies, existing evidence is largely level II for morphea and is even more minimal for scleroderma and other sclerosing disorders (scleroderma, lichen sclerosus, and chronic graft-versus-host disease, among others)...
September 2016: Clinics in Dermatology
Emma L Culver, Roger W Chapman
IgG4-related hepatobiliary diseases are part of a multiorgan fibroinflammatory condition termed IgG4-related disease, and include IgG4-related sclerosing cholangitis (IgG4-SC) and IgG4-related hepatopathy. These diseases can present with biliary strictures and/or mass lesions, making them difficult to differentiate from primary sclerosing cholangitis (PSC) or other hepatobiliary malignancies. Diagnosis is based on a combination of clinical, biochemical, radiological and histological findings. However, a gold standard diagnostic test is lacking, warranting the identification of more specific disease markers...
October 2016: Nature Reviews. Gastroenterology & Hepatology
Haa-Na Song, Seok Jin Kim, Young Hyeh Ko, Won Seog Kim
BACKGROUND: Mediastinal gray zone lymphoma (MGZL) shares clinical characteristics with primary mediastinal B-cell lymphoma (PMBCL) and nodular sclerosing Hodgkin lymphoma (NSHL). However, MGZL is extremely rare, and an appropriate treatment for it has not yet been established. METHODS: We retrospectively analyzed 8 patients who were treated with systemic chemotherapy for MGZL between 2007 and 2014. RESULTS: The patients with MGZL were predominantly young and male (median age 26 years), and 62...
2016: Acta Haematologica
Masahiro Watanabe, Koichi Hashimoto, Yusaku Abe, Eiichi N Kodama, Ryota Nabika, Shinya Oishi, Shinichiro Ohara, Masatoki Sato, Yukihiko Kawasaki, Nobutaka Fujii, Mitsuaki Hosoya
Subacute sclerosing panencephalitis (SSPE) is a persistent, progressive, and fatal degenerative disease resulting from persistent measles virus (MV) infection of the central nervous system. Most drugs used to treat SSPE have been reported to have limited effects. Therefore, novel therapeutic strategies are urgently required. The SSPE virus, a variant MV strain, differs virologically from wild-type MV strain. One characteristic of the SSPE virus is its defective production of cell-free virus, which leaves cell-to-cell infection as the major mechanism of viral dissemination...
2016: PloS One
Ann M Kulungowski, Aladdin H Hassanein, Carolyn C Foster, Arin K Greene, Steven J Fishman
PURPOSE: The treatment of venous malformations is difficult because these lesions frequently recur after resection or sclerotherapy. The purpose of this study was to determine whether recanalization of sclerosed venous lumens could be prevented with systemic angiogenic inhibition using bevacizumab or peginterferon alfa-2a. METHODS: To establish an animal model of recanalization of sclerosed facial veins, 18 rabbits had ethanol sclerotherapy of 1 facial vein followed by venography after 4weeks (n=6), 12weeks (n=6), and 24weeks (n=6)...
October 2016: Journal of Pediatric Surgery
Charlotte R Grant, Rodrigo Liberal
There are several examples of liver tolerance: the relative ease by which liver allografts are accepted and the exploitation of the hepatic microenvironment by the malarial parasite and hepatotrophic viruses are notable examples. The vasculature of the liver supports a unique population of antigen presenting cells specialised to maintain immunological tolerance despite continuous exposure to gut-derived antigens. Liver sinusoidal endothelial cells and Kupffer cells appear to be key to the maintenance of immune tolerance, by promoting T cell anergy or deletion and the generation of regulatory cell subsets...
August 12, 2016: Clinics and Research in Hepatology and Gastroenterology
Sarah Keller, Harald Ittrich, Christoph Schramm, Ansgar W Lohse, Michael Amling, Gerhard Adam, Jin Yamamura
PURPOSE: Osteodystrophy is a frequent complication in primary sclerosing cholangitis (PSC). The aim was to test the feasibility of vertebral bone diffusion-weighted imaging (DWI) in routine liver MRI for detection of osteoporosis using dual-energy X-ray absorptiometry (DXA) as gold standard. MATERIALS AND METHODS: Forty PSC patients (50 ± 12.6 years) and ten controls (49.5 ± 13.0 years) were scanned using a DWI spin echo echo-planar sequence (b-factors 0-800 s/mm(2)) on a 3-T MRI system and DXA (76 kVp)...
October 2016: Japanese Journal of Radiology
Sung-Hoon Moon, Myung-Hwan Kim, Jong Kyun Lee, Seunghee Baek, Young Sik Woo, Dong Hui Cho, Dongwook Oh, Tae Jun Song, Do Hyun Park, Sang Soo Lee, Dong Wan Seo, Sung Koo Lee
BACKGROUND: Recent research has shown that a substantial number of patients with primary sclerosing cholangitis (PSC) can also have elevated serum/tissue IgG4. The aim of our study was to develop a simple scoring system for the discrimination of IgG4-related sclerosing cholangits (IgG4-SC) from PSC. METHODS: Patients with IgG4-SC (n = 39) and PSC (n = 76) who had intrahepatic/hilar strictures were included. Candidate-differentiating variables included patient age, other organ involvement (OOI), inflammatory bowel disease, serum IgG4, and cholangiographic features...
July 28, 2016: Journal of Gastroenterology
Tassos Grammatikopoulos, Melissa Sambrotta, Sandra Strautnieks, Pierre Foskett, A S Knisely, Bart Wagner, Maesha Deheragoda, Chris Starling, Giorgina Mieli-Vergani, Joshua Smith, University Of Washington Center For Mendelian Genomics, Laura Bull, Richard J Thompson
BACKGROUND & AIMS: Neonatal sclerosing cholangitis (NSC) is a severe neonatal-onset cholangiopathy commonly leading to liver transplantation (LT) for end-stage liver disease in childhood. Liver-biopsy findings histopathologically resemble those in biliary atresia (BA); however, in NSC extrahepatic bile ducts are patent, whilst in BA their lumina are obliterated. NSC is commonly seen in consanguineous kindreds, suggesting autosomal recessive inheritance. METHODS: From 29 NSC patients (24 families) identified, DNA was available in 24 (21 families)...
July 25, 2016: Journal of Hepatology
Terumi Kamisawa, Sawako Kuruma, Kazuro Chiba
An accurate diagnosis should be made before treatment of autoimmune pancreatitis and immunoglobulin G4-related sclerosing cholangitis. Once a diagnosis has been established, steroids are the standard therapy and symptoms are the major indications. Before steroid therapy, obstructive jaundice and hyperglycemia should be controlled. An initial dose of 0.6 mg/kg/d of oral prednisolone is administered for 2 to 4 weeks, and is gradually tapered over 2 to 3 months. After steroid therapy has begun, blood and imaging tests are performed periodically...
August 2016: Seminars in Liver Disease
Shigeyuki Kawa
Although now considered to be a member of the systemic entity of immunoglobulin G4- (IgG4-) related disease, IgG4-related pancreatitis is generally referred to as type 1 autoimmune pancreatitis (AIP). Type 1 AIP was established based on a pathological background of lymphoplasmacytic sclerosing pancreatitis, high serum IgG4 concentration, and abundant IgG4-bearing plasma cell infiltration. The characteristic clinical features of type 1 AIP, such as elderly male preponderance, obstructive jaundice, and mass-forming lesions in the pancreas, often mimic those of pancreatic cancer...
August 2016: Seminars in Liver Disease
Yoh Zen
Immunoglobulin G4-related disease (IgG4-RD) in the pancreatobiliary system manifests as sclerosing cholangitis (SC), hepatic inflammatory pseudotumors, and type 1 autoimmune pancreatitis (AIP). The pathology of IgG4-RD involves an inflammatory process and fibrogenic pathway, the combination of which damages the affected organs. Fibroinflammatory injury is characterized by three microscopic findings: a diffuse lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, obliterative phlebitis, and storiform fibrosis...
August 2016: Seminars in Liver Disease
Yasuni Nakanuma, Yoji Ishizu, Yoh Zen, Kenichi Harada, Takeji Umemura
Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease involving many organs; it includes IgG4-related sclerosing cholangitis and inflammatory pseudotumor in the hepatobiliary system. Two types of hepatic parenchymal involvement have been reported in IgG4-RD: IgG4-related autoimmune hepatitis (AIH) and IgG4-hepatopathy. Moreover, only three cases of IgG4-related AIH have been reported. Immunoglobulin G4-related AIH is clinicopathologically similar to AIH, except for an elevated serum IgG4 level and heavy infiltration of IgG4-positive plasma cells in the liver tissue...
August 2016: Seminars in Liver Disease
Nianyu Xue, Youfeng Xu, Pintong Huang, Shengmin Zhang, Hongwei Wang, Fei Yu
The present study aimed to report the shear wave elastography (SWE) findings in a patient with the diffuse sclerosing variant of papillary thyroid carcinoma (DSVPTC). Since patients with DSVPTC may present with typical clinicopathological features and initially appear to have Hashimoto's thyroiditis, a thorough clinical evaluation and an early diagnosis are important. A 20-year-old female patient presented with a 1-month history of a neck mass and sore throat. Conventional ultrasound and SWE were performed using an AIXPLORER system with 14-5 MHz linear transducer...
August 2016: Molecular and Clinical Oncology
Vivian Youngjean Park, Eun-Kyung Kim, Min Jung Kim, Jung Hyun Yoon, Hee Jung Moon
An increasing number of radial scars are detected by ultrasound (US), but their management is controversial. This study investigated the upgrade rate in mammographically occult radial scars/complex sclerosing lesions without epithelial atypia at US-guided 14-gauge core needle biopsy in asymptomatic patients. Nineteen mammographically occult benign radial scars/complex sclerosing lesions (median size, 7 mm; range, 3-23 mm) were included. Patients underwent surgical excision (n = 10) or vacuum-assisted excision, with follow-up US at least 6 mo after benign vacuum-assisted excision results (n = 8), or underwent US follow-up for 2 y after core needle biopsy (n = 1)...
October 2016: Ultrasound in Medicine & Biology
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