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Primary sclerosing cholangitis

Syeda Naqvi, Syed Askari Hasan, Sameen Khalid, Aamer Abbass, Melanie Albors-Mora
Ulcerative colitis is an autoimmune disorder leading to chronic intestinal inflammation. It can present with a wide range of associated extra-intestinal manifestations. We present a case of an 18-year-old man diagnosed with ulcerative colitis, autoimmune hemolytic anemia and primary sclerosing cholangitis during the same hospitalization. The unique triad of these diseases gives important clues to the immunological factors involved in the pathogenesis of these diseases.
January 15, 2018: Curēus
Axel Andres, Aldo Montano-Loza, Russell Greiner, Max Uhlich, Ping Jin, Bret Hoehn, David Bigam, James Andrew Mark Shapiro, Norman Mark Kneteman
Deciding who should receive a liver transplant (LT) depends on both urgency and utility. Most survival scores are validated through discriminative tests, which compare predicted outcomes between patients. Assessing post-transplant survival utility is not discriminate, but should be "calibrated" to be effective. There are currently no such calibrated models. We developed and validated a novel calibrated model to predict individual survival after LT for Primary Sclerosing Cholangitis (PSC). We applied a software tool, PSSP, to adult patients in the Scientific Registry of Transplant Recipients (n = 2769) who received a LT for PSC between 2002 and 2013; this produced a model for predicting individual survival distributions for novel patients...
2018: PloS One
Yoshihide Ueda, Toshimi Kaido, Hideaki Okajima, Koichiro Hata, Takayuki Anazawa, Atsushi Yoshizawa, Shintaro Yagi, Kojiro Taura, Toshihiko Masui, Noriyo Yamashiki, Hironori Haga, Miki Nagao, Hiroyuki Marusawa, Hiroshi Seno, Shinji Uemoto
Background: Primary sclerosing cholangitis (PSC) is a progressive cholestatic liver disease, with liver transplantation being the sole life-saving treatment for end-stage PSC-related liver disease. However, recurrence of PSC after liver transplantation is a common complication, with the risk factors for recurrence being controversial. Methods: We conducted a retrospective chart review of 45 patients who had undergone liver transplantation for PSC at our institute...
December 2017: Transplantation Direct
Eleni Theocharidou, Michael A Heneghan
Autoimmune hepatitis occurs in genetically susceptible individuals as a result of loss of immunological tolerance to hepatic autoantigens that can be precipitated by environmental triggers. The clinical manifestation is usually insidious but can be also acute with liver failure. The diagnosis is made on the basis of antibody positivity, elevated immunoglobulin G levels and interface hepatitis on liver histology. Induction of remission is achieved with high-dose steroids in the majority of cases, and maintenance of remission with azathioprine...
March 2, 2018: British Journal of Hospital Medicine
Mohamad A Mouchli, Siddharth Singh, Lisa Boardman, David H Bruining, Amy L Lightner, Charles B Rosen, Julie K Heimbach, Bashar Hasan, John J Poterucha, Kymberly D Watt, Sunanda V Kane, Laura E Raffals, Edward V Loftus
Background: The course of inflammatory bowel disease (IBD) after liver transplantation (LT) for primary sclerosing cholangitis (PSC) is poorly understood. We describe the natural history of established IBD after LT (including risk of disease progression, colectomy, and neoplasia) and de novo IBD. Methods: In a retrospective cohort, we identified all patients with PSC who underwent LT for advanced PSC at Mayo Clinic, Rochester, Minnesota. Risk factors were identified using multivariate Cox proportional hazard analysis...
March 7, 2018: Inflammatory Bowel Diseases
Martine A Aardoom, Maria E Linda Joosse, Andrica C H de Vries, Arie Levine, Lissy de Ridder
Background: Cancer and death are the most severe outcomes that affect patients with inflammatory bowel disease (IBD). These outcomes are even more severe if they occur at a young age but are rare, even in the general population. We conducted a systematic review to provide an overview of all reported pediatric (PIBD) patients with severe outcome. Methods: A literature search identified publications that reported development of cancer or fatal outcome in PIBD patients...
March 7, 2018: Inflammatory Bowel Diseases
M Tieleman, A P van den Berg, B van Hoek, W G Polak, J Dubbeld, R J Porte, C Konijn, R A de Man, B E Hanssen, H J Metselaar
OBJECTIVE: To calculate the chance of receiving a liver transplant for patients on the liver transplant waiting list in the Netherlands. DESIGN: Retrospective cohort research. METHOD: Data of all patients in the Netherlands on the waiting list for liver transplantation, from the introduction of the model of end-stage liver disease score on 16th December 2006 through to 31st December 2013 were collected. Survival analysis was computed with competing risk analyses...
2018: Nederlands Tijdschrift Voor Geneeskunde
Ifeyinwa E Obiorah, Alicia Henao Velasquez, Bhaskar Kallakury, Metin Özdemirli
BACKGROUND: Langerhans cell histiocytosis is characterized by abnormal proliferation of neoplastic Langerhans cells. Langerhans cell histiocytosis commonly affects the pediatric population and presentation in adults remain a rare event. The presentation of langerhans cell histiocytosis is highly variable but skin, bone and lung involvement are very common. Langerhans cell histiocytosis presenting as a bile duct mass is rare and usually occurs as part of a multi-organ system disease. CASE REPORT: We present a case of langerhans cell histiocytosis confined to the extrahepatic bile duct in a 62-year-old female with sclerosing cholangitis...
March 8, 2018: Balkan Medical Journal
Sarah Keller, Annette Aigner, Roman Zenouzi, Anne C Kim, Arnoud Meijer, Sören A Weidemann, Till Krech, Ansgar W Lohse, Gerhard Adam, Christoph Schramm, Jin Yamamura
OBJECTIVE: To evaluate magnetic resonance imaging (MRI) parameters T2 signal, contrast enhancement (CE), and relative liver enhancement (RLE) of extracellular gadolinium-based contrast agent (GBCA)-enhanced MRI as a marker for hepatic fibrosis and inflammation in patients with primary sclerosing cholangitis (PSC). METHODS: 3.0-Tesla MRI scans and liver biopsies of 40 patients (41.2 ± 17.1 years) were retrospectively reviewed. Biopsies were obtained within a mean time of 54 ± 55 days to MRI scans and specimens were categorized according to Ishak modified hepatic activity index (mHAI) and Scheuer staging of fibrosis...
2018: PloS One
Christian Rupp, Alexander Rössler, Taotao Zhou, Conrad Rauber, Kilian Friedrich, Andreas Wannhoff, Karl-Heinz Weiss, Peter Sauer, Peter Schirmacher, Caner Süsal, Wolfgang Stremmel, Daniel N Gotthardt
Background: The median age of diagnosis of primary sclerosing cholangitis (PSC) is ∼30-40 years. Objective: We aimed to analyse disease progression and liver-dependent survival in patients diagnosed with PSC after 50 years of age. Methods: Patients with PSC were analysed with regard to their age at diagnosis. Patients with a first diagnosis of PSC after the age of 50 years were considered as the late-onset group. Results: A total of 32/215 (14...
March 2018: United European Gastroenterology Journal
Urs Christen, Edith Hintermann
Autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) are serious autoimmune liver diseases that are characterized by a progressive destruction of the liver parenchyma and/or the hepatic bile ducts and the development of chronic fibrosis. Left untreated autoimmune liver diseases are often life-threatening, and patients require a liver transplantation to survive. Thus, an early and reliable diagnosis is paramount for the initiation of a proper therapy with immunosuppressive and/or anticholelithic drugs...
2018: Frontiers in Immunology
Melahat Kul, Diğdem Kuru Öz, Ayşe Erden
No abstract text is available yet for this article.
March 3, 2018: Abdominal Radiology
Keigo Murakami, Shuko Hata, Yasuhiro Miki, Hironobu Sasano
Background A potential correlation between sex hormones, such as androgens and estrogens, and the development and progression of hepatocellular carcinoma (HCC) has been proposed. However, its details, in particular, aromatase status in diseased human liver has remained largely unknown. Materials and methods We immunolocalized aromatase, 17β-hydroxysteroid dehydrogenase (17β-HSD) type 1 and 17β-HSD type 2 in a total of 155 cases, consisting of normal liver (n = 10), nonalcoholic steatohepatitis (NASH) (n = 18), primary sclerosing cholangitis (PSC) (n = 6), primary biliary cholangitis (PBC) (n = 13), biliary atresia (n = 18), alcoholic hepatitis (n = 11), hepatitis C virus (HCV) (n = 31), HCV sustained virologic response (HCV-SVR) (n = 10), hepatitis B virus (HBV) (n = 20), HBV sustained virologic response (HBV-SVR) (n = 8) and infants (n = 10)...
February 28, 2018: Hormone Molecular Biology and Clinical Investigation
Harveen Singh, Fariha Balouch, Charlton Noble, Peter Lewindon
OBJECTIVES: Autoimmune liver disease (AILD) incorporates primary sclerosing cholangitis (PSC), autoimmune hepatitis (AIH), and autoimmune sclerosing cholangitis (ASC). ASC is a condition that includes overlap of AIH and PSC. We investigate changes in practice in relation to diagnosis and phenotype over 2 time periods. METHODS: Retrospective chart review was conducted from January 2000 to 2016. Data were divided into two 8-year cohorts, CI and C2. RESULTS: Data were collected in 75 children, 29 in 2000-2007 (C1) and 46 in 2008-2016 (C2)...
February 21, 2018: Journal of Pediatric Gastroenterology and Nutrition
G J Webb, S R Rahman, C Levy, G M Hirschfield
BACKGROUND: The use of rifampicin for cholestatic pruritus is accompanied by concerns over safety, but the availability of real-world prescribing data is relatively limited. AIM: We sought to describe the rate and characteristics of rifampicin-induced hepatitis in a mixed aetiology cohort of patients with established liver disease and cholestatic pruritus. METHODS: Retrospective review of records for out-patients commenced on rifampicin for pruritus 2012-2016 inclusive...
February 22, 2018: Alimentary Pharmacology & Therapeutics
Jonathan H Chen, Vikram Deshpande
Primary sclerosing cholangitis (PSC) is a cholangiopathy-usually associated with inflammatory bowel disease-that leads to cirrhosis and liver failure. Based on a multitude of clinical trials, there is general consensus that PSC progression is unchanged by current therapies, including steroids. However, there are scattered reports in the literature of PSC patients responsive to steroids. Recently, several steroid-responsive PSC mimics have been described, most notably immunoglobulin G4-related sclerosing cholangitis...
February 1, 2018: International Journal of Surgical Pathology
Yohei Yamada, Ken Hoshino, Yasushi Fuchimoto, Kentaro Matsubara, Taizo Hibi, Hiroshi Yagi, Yuta Abe, Masahiro Shinoda, Minoru Kitago, Hideaki Obara, Takahito Yagi, Hideaki Okajima, Toshimi Kaido, Shinji Uemoto, Tatsuya Suzuki, Keiichi Kubota, Tomoharu Yoshizumi, Yoshihiko Maehara, Yukihiro Inomata, Yuko Kitagawa, Hiroto Egawa, Tatsuo Kuroda
Background: Multiple studies have failed to reveal an effective method for preventing the recurrence of primary sclerosing cholangitis (PSC) after liver transplantation (LTx). A national study conducted in Japan revealed several risk factors for the recurrence after living donor LTx (LDLTx); however, recipients of ABO-blood type incompatible (ABO-I) LTx were excluded from the previous analysis. In the present study, we investigated the efficacy of an immunosuppressive protocol in ABO-I LTx on the recurrence of PSC after LDLTx...
February 2018: Transplantation Direct
Saeed Azizi, Hussein Al-Rubaye, Mohammed Adil Turki, Muhammad R Sameem Siddiqui, Arun P Shanmuganandan, Bushra Ehsanullah, Ranjeet Brar, Al-Mutaz Abulafi
BACKGROUND: Endoscopic examinations are a vital diagnostic tool for dysplasia. Establishing the precision of different modes of examination is essential due to the disparate pick-up rates of dysplasia. OBJECTIVE: The aim of this article was to establish the pick-up rates of dysplastic or cancerous lesions using white light endoscopy (WLE) and random/targeted biopsies, or chromoendoscopy (CE), in patients with ulcerative colitis (UC) without primary sclerosing (PSC) or Crohn's disease (CD)...
February 17, 2018: International Journal of Surgery
Caroline Nordenvall, Ola Olén, Per Johan Nilsson, Anders Ekbom, Matteo Bottai, Pär Myrelid, Annika Bergquist
Background: Studies on surgical procedures in patients with concomitant primary sclerosing cholangitis (PSC) and ulcerative colitis (UC) have mainly been restricted to single centers. The aim was to compare surgical treatment of UC with or without PSC in a nationwide study. Methods: A cohort study including all patients diagnosed with UC between 1987 and 2014 in Sweden was undertaken. The impact of PSC on the risk of colectomy, the chance of restorative surgery, and risk of failure (presence of a stoma) following restorative surgery were estimated...
February 15, 2018: Inflammatory Bowel Diseases
Dana Tedesco, Manoj Thapa, Chui-Yoke Chin, Yong Ge, Minghao Gong, Jing Li, Sanjeev Gumber, Patrick Speck, Elizabeth J Elrod, Eileen M Burd, William H Kitchens, Joseph F Magliocca, Andrew B Adams, David S Weiss, Mansour Mohamadzadeh, Arash Grakoui
BACKGROUND & AIMS: Variants at the ATP binding cassette subfamily B member 4 gene (ABCB4 or MDR2) locus, which encodes a biliary transport protein, are associated with a spectrum of cholestatic liver diseases. Exacerbation of liver disease has been linked to increased hepatic levels of interleukin 17 (IL17), yet the mechanisms of this increase are not understood. We studied mice with disruption of Mdr2 to determine how defects in liver and alteration in the microbiota contribute to production of IL17 by intrahepatic γδ T cells...
February 15, 2018: Gastroenterology
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