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Primary biliary cirrhosis

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https://www.readbyqxmd.com/read/28640288/prognostic-factors-for-transplant-free-survival-and-validation-of-prognostic-models-in-chinese-patients-with-primary-biliary-cholangitis-receiving-ursodeoxycholic-acid
#1
Ka-Shing Cheung, Wai-Kay Seto, James Fung, Ching-Lung Lai, Man-Fung Yuen
OBJECTIVES: We aimed to validate the prognostic models for primary biliary cholangitis (PBC) in Chinese patients receiving ursodeoxycholic acid (UCDA), and to compare their performances in predicting the long-term survival. METHODS: Chinese patients with PBC from a tertiary center were identified via electronic search of hospital medical registry. Risk factors associated with adverse events (liver transplantation or death from liver-related causes including hepatocellular carcinoma (HCC) and liver decompensation) were determined...
June 22, 2017: Clinical and Translational Gastroenterology
https://www.readbyqxmd.com/read/28630805/dermatitis-herpetiformis-as-the-initial-presentation-of-primary-biliary-cholangitis-in-a-male-with-gluten-sensitivity
#2
Cyriac Philips, Rajaguru Paramaguru, Divya A Indiran, Philip Augustine
Celiac disease is commonly associated with elevated liver enzymes that normalize on a gluten-free diet. Celiac disease is rarely described in patients with primary biliary cholangitis. Dermatitis herpetiformis is the skin manifestation of the celiac disease that is very rarely associated with primary biliary cirrhosis. We present the case of a 62-year-old man who presented with severe chronic pruritus, in whom a diagnosis of primary biliary cholangitis was made initially. However, in the presence of atypical skin lesions, not confirming to chronic cholestasis, an in-depth evaluation including histopathological examination led to the diagnosis of dermatitis herpetiformis associated with gluten sensitivity...
May 14, 2017: Curēus
https://www.readbyqxmd.com/read/28624334/seasonality-and-autoimmune-diseases-the-contribution-of-the-four-seasons-to-the-mosaic-of-autoimmunity
#3
REVIEW
Abdulla Watad, Shir Azrielant, Nicola Luigi Bragazzi, Kassem Sharif, Paula David, Itay Katz, Gali Aljadeff, Mariana Quaresma, Galya Tanay, Mohammad Adawi, Howard Amital, Yehuda Shoenfeld
Autoimmune diseases (ADs) are a heterogeneous groups of diseases that occur as a results of loss of tolerance to self antigens. While the etiopathogeneis remain obscure, different environmental factors were suggested to have a role in the development of autoimmunity, including infections, low vitamin D levels, UV radiation, and melatonin. Interestingly, such factors possess seasonal variation patterns that could influence disease development, severity and progression. Vitamin D levels which reach a nadir during late winter and early spring is correlated with increased disease activity, clinical severity as well as relapse rates in several disease entities including multiple sclerosis (MS), non-cutaneous flares of systemic lupus erythematosus (SLE), psoriasis, and rheumatoid arthritis (RA)...
June 14, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28614198/the-almost-normal-liver-biopsy-presentation-clinical-associations-and-outcome
#4
Thomas W Czeczok, John S Van Arnam, Laura D Wood, Michael S Torbenson, Taofic Mounajjed
Liver biopsies obtained for abnormal liver enzymes or unexplained ascites occasionally appear histologically almost normal. The differential diagnosis for these cases is challenging because literature addressing this topic is lacking. We aimed to establish a differential diagnosis and determine clinical associations and outcomes for almost-normal liver biopsies. Ninety-seven histologically almost-normal liver biopsies were collected from 2 institutions. All cases lacked significant inflammation, fatty change, biliary tract disease, vascular disease, nodular regenerative hyperplasia, iron overload, inherited metabolic or storage disorder, viral hepatitis, or fibrosis...
June 13, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28602220/inhibition-of-microrna-24-increases-liver-fibrosis-by-enhanced-menin-expression-in-mdr2-mice
#5
Chad Hall, Laurent Ehrlich, Fanyin Meng, Pietro Invernizzi, Francesca Bernuzzi, Terry C Lairmore, Gianfranco Alpini, Shannon Glaser
BACKGROUND: Liver transplantation remains the primary treatment for primary sclerosing cholangitis (PSC). Mdr2(-/-) mice provide a reliable in vivo model of PSC and develop characteristic biliary inflammation and fibrosis. We tested the hypothesis that the tumor suppressor protein menin is implicated in the progression of liver fibrosis and that menin expression can be regulated in the liver via microRNA-24 (miR-24). MATERIALS AND METHODS: Menin expression was measured in human PSC and Mdr2(-/-) mice...
May 11, 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/28580726/the-first-case-of-domino-split-liver-transplantation-in-maple-syrup-urine-disease
#6
Uta Herden, Jun Li, Lutz Fischer, Florian Brinkert, Martin Blohm, René Santer, Bjoern Nashan, Enke Grabhorn
The enzymatic defect in MSUD results in accumulation of neurotoxic metabolites of BCAAs. LTX has shown to be a feasible strategy in patients non-responsive to diet. Because of sufficient enzyme activity in extrahepatic tissues in healthy people, the MSUD liver graft is a suitable domino organ. We present the first case of a technical challenging ex situ split of a MSUD domino organ transplanted into two pediatric recipients. The domino graft donor was a 21-year-old female (58 kg) suffering from MSUD with recurrent metabolic decompensation despite strict diet...
June 5, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28570655/circulating-fgf19-closely-correlates-with-bile-acid-synthesis-and-cholestasis-in-patients-with-primary-biliary-cirrhosis
#7
Zhanyi Li, Bingliang Lin, Guoli Lin, Yuankai Wu, Yusheng Jie, Xiangyong Li, Brian Ko, Yutian Chong, Jian Luo
BACKGROUND AND AIM: Bile acid (BA) synthesis in the liver is regulated by Fibroblast Growth Factor 19 (FGF19) secreted from the ileum as an enterohepatic feedback mechanism. Although FGF19 mRNA is absent in normal liver, FGF19 gene expression was reported to increase in response to both extrahepatic and intrahepatic cholestasis. The impact of upregulated FGF19 expression on BA synthesis is unclear and the overall role of circulating FGF19 and BA synthesis under cholestatic conditions needs to be further investigated...
2017: PloS One
https://www.readbyqxmd.com/read/28566919/xanthoma-striatum-palmare-in-a-patient-of-primary-biliary-cirrhosis-with-autoimmune-hepatitis
#8
Min-Young Yang, Jeong-Min Kim, Gun-Wook Kim, Hoon-Soo Kim, Byung-Soo Kim, Moon-Bum Kim, Hyun-Chang Ko
No abstract text is available yet for this article.
June 2017: Annals of Dermatology
https://www.readbyqxmd.com/read/28547740/biliary-duodenal-anastomosis-using-magnetic-compression-following-massive-resection-of-small-intestine-due-to-strangulated-ileus-after-living-donor-liver-transplantation-a-case-report
#9
Ryusuke Saito, Hiroyuki Tahara, Seiichi Shimizu, Masahiro Ohira, Kentaro Ide, Kohei Ishiyama, Tsuyoshi Kobayashi, Hideki Ohdan
BACKGROUND: Despite the improvements of surgical techniques and postoperative management of patients with liver transplantation, biliary complications are one of the most common and important adverse events. We present a first case of choledochoduodenostomy using magnetic compression following a massive resection of the small intestine due to strangulated ileus after living donor liver transplantation. CASE PRESENTATION: The 54-year-old female patient had end-stage liver disease, secondary to liver cirrhosis, due to primary sclerosing cholangitis with ulcerative colitis...
December 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/28546061/primary-biliary-cholangitis-advances-in-management-and-treatment-of-the-disease
#10
Pietro Invernizzi, Annarosa Floreani, Marco Carbone, Marco Marzioni, Antonio Craxi, Luigi Muratori, Umberto Vespasiani Gentilucci, Ivan Gardini, Antonio Gasbarrini, Paola Kruger, Francesco Saverio Mennini, Virginia Ronco, Elena Lanati, Pier Luigi Canonico, Domenico Alvaro
Primary Biliary Cholangitis, previously known as Primary Biliary Cirrhosis, is a rare disease, which mainly affects women in their fifth to seventh decades of life. It is a chronic autoimmune disease characterized by a progressive damage of interlobular bile ducts leading to ductopenia, chronic cholestasis and bile acids retention. Even if the disease usually presents a long asymptomatic phase and a slow progression, in many patients it may progress faster toward cirrhosis and its complications. The 10year mortality is greater than in diseases such as human immunodeficiency virus/Hepatitis C Virus coinfection and breast cancer...
May 8, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28538834/characteristics-of-liver-fibrosis-with-different-etiologies-using-a-fully-quantitative-fibrosis-assessment-tool
#11
Q Wu, X Zhao, H You
This study aimed to test the diagnostic performance of a fully quantitative fibrosis assessment tool for liver fibrosis in patients with chronic hepatitis B (CHB), primary biliary cirrhosis (PBC) and non-alcoholic steatohepatitis (NASH). A total of 117 patients with liver fibrosis were included in this study, including 50 patients with CHB, 49 patients with PBC and 18 patients with NASH. All patients underwent liver biopsy (LB). Fibrosis stages were assessed by two experienced pathologists. Histopathological images of LB slices were processed by second harmonic generation (SHG)/two-photon excited fluorescence (TPEF) microscopy without staining, a system called qFibrosis (quantitative fibrosis) system...
May 18, 2017: Brazilian Journal of Medical and Biological Research, Revista Brasileira de Pesquisas Médicas e Biológicas
https://www.readbyqxmd.com/read/28535947/geoepidemiology-clinical-manifestations-and-outcome-of-primary-biliary-cholangitis-in-greece
#12
Nikolaos K Gatselis, Kalliopi Zachou, Vasiliki Lygoura, Kalliopi Azariadis, Pinelopi Arvaniti, Elias Spyrou, Georgia Papadamou, George K Koukoulis, George N Dalekos, Eirini I Rigopoulou
BACKGROUND & AIMS: Primary biliary cholangitis (PBC) is a disease with rising prevalence and considerable geographical variation. To describe the prevalence, spatial and time distribution, baseline characteristics, response to treatment, outcome and the validity of GLOBE score in a large cohort of Greek PBC patients as an independent validation of this score has not been done so far. METHODS: The last 16years, 482 PBC patients (86.5% females) were evaluated and analysed retrospectively, using a prospectively collected database...
May 20, 2017: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/28522403/multiplex-autoantibody-detection-for-autoimmune-liver-diseases-and-autoimmune-gastritis
#13
Joris Vanderlocht, Mart van der Cruys, Frans Stals, Liesbeth Bakker-Jonges, Jan Damoiseaux
Autoantibody detection for autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and autoimmune gastritis (AIG) is traditionally performed by IIF on a combination of tissues. Multiplex line/dot blots (LIA/DIA) offer multiple advantages, i.e. automation, objective reading, no interfering reactivities, no coincidental findings. In the current study we evaluated automated DIA (D-Tek) for detecting autoantibodies related to autoimmune diseases of the gastrointestinal tract. We tested samples of the Dutch EQC program and compared the results with the consensus of the participating labs...
May 16, 2017: Journal of Immunological Methods
https://www.readbyqxmd.com/read/28518283/branched-chain-amino-acids-for-people-with-hepatic-encephalopathy
#14
REVIEW
Lise Lotte Gluud, Gitte Dam, Iñigo Les, Giulio Marchesini, Mette Borre, Niels Kristian Aagaard, Hendrik Vilstrup
BACKGROUND: Hepatic encephalopathy is a brain dysfunction with neurological and psychiatric changes associated with liver insufficiency or portal-systemic shunting. The severity ranges from minor symptoms to coma. A Cochrane systematic review including 11 randomised clinical trials on branched-chain amino acids (BCAA) versus control interventions has evaluated if BCAA may benefit people with hepatic encephalopathy. OBJECTIVES: To evaluate the beneficial and harmful effects of BCAA versus any control intervention for people with hepatic encephalopathy...
May 18, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28517369/treatment-of-primary-biliary-cholangitis-non-responders-a-systematic-review
#15
Duminda Suraweera, Harman Rahal, Melissa Jimenez, Matthew Viramontes, Gina Choi, Sammy Saab
BACKGROUND: Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a chronic cholestatic liver disease characterized by an immune mediated destruction of intrahepatic bile ducts. Ursodeoxycholic acid (UDCA) has been the primary medication for the treatment of PBC, resulting in improved liver tests, resolution of symptoms and increased transplant free survival. However, not all patients respond to UDCA. The aim of this systematic review is to provide an evidence based assessment of the medications that have been studied in patients who are refractory to UDCA...
May 18, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28503557/epidemiology-and-risk-factors-intrahepatic-cholangiocarcinoma
#16
REVIEW
Amar Gupta, Elijah Dixon
Intrahepatic cholangiocarcinoma (ICC) is a rare entity with a distinct clinical course and epidemiology from hilar and extrahepatic cholangiocarcinoma. ICC makes up 8-10% of cholangiocarcinomas and 10-20% of all primary liver tumors. There remains a considerable amount of geographic variation in the incidence of ICC worldwide; however, the overall incidence of this malignancy appears to be rising. Several risk factors have been identified, such as infectious causes (liver flukes, viral hepatitis), biliary tract disease [primary sclerosing cholangitis (PSC), hepaticolithiasis, biliary cystic diseases], metabolic syndrome, lifestyle choices (alcohol abuse, tobacco use), and cirrhosis...
April 2017: Hepatobiliary Surgery and Nutrition
https://www.readbyqxmd.com/read/28503555/surgical-options-for-intrahepatic-cholangiocarcinoma
#17
REVIEW
Kui Wang, Han Zhang, Yong Xia, Jian Liu, Feng Shen
Intrahepatic cholangiocarcinoma (ICC) is the second most common primary liver cancer, accounting for 10-15% of primary hepatic malignancy. The incidence and cancer-related mortality of ICC continue to increase worldwide. At present, hepatectomy is still the most effective treatment for ICC patients to achieve long-term survival, although its overall efficacy may not be as good as that for patients with hepatocellular carcinoma (HCC) due to the unique pathogenesis and clinical-pathological profiles of ICC. Viral infection, lithiasis and metabolic factors may all be associated with the pathogenesis of ICC...
April 2017: Hepatobiliary Surgery and Nutrition
https://www.readbyqxmd.com/read/28496445/eosinophils-in-autoimmune-diseases
#18
REVIEW
Nicola L Diny, Noel R Rose, Daniela Čiháková
Eosinophils are multifunctional granulocytes that contribute to initiation and modulation of inflammation. Their role in asthma and parasitic infections has long been recognized. Growing evidence now reveals a role for eosinophils in autoimmune diseases. In this review, we summarize the function of eosinophils in inflammatory bowel diseases, neuromyelitis optica, bullous pemphigoid, autoimmune myocarditis, primary biliary cirrhosis, eosinophilic granulomatosis with polyangiitis, and other autoimmune diseases...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28494557/-clinicopathologic-features-of-drug-induced-vanishing-bile-duct-syndrome
#19
L H Ye, C K Wang, H C Zhang, Z Q Liu, H W Zheng
Vanishing bile duct syndrome (VBDS) manifests as progressive destruction and disappearance of the intrahepatic bile duct caused by various factors and cholestasis. VBDS associated with drug-induced liver injury (D-VBDS) is an important etiology of VBDS, and immune disorder or immune imbalance may be the main pathogenesis. According to its clinical symptoms, serological markers, and course of the disease, D-VBDS is classified into major form and minor form, and its clinical features are based on various pathomorphological findings...
April 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/28465155/effect-of-liver-disease-on-hepatic-transporter-expression-and-function
#20
REVIEW
Nilay Thakkar, Jason R Slizgi, Kim L R Brouwer
Liver disease can alter the disposition of xenobiotics and endogenous substances. Regulatory agencies such as the Food and Drug Administration (FDA) and the European Medicines Evaluation Agency (EMEA) recommend, if possible, studying the effect of liver disease on drugs under development to guide specific dose recommendations in these patients. While extensive research has been conducted to characterize the effect of liver disease on drug-metabolizing enzymes, emerging data have implicated that the expression and/or function of hepatobiliary transport proteins also are altered in liver disease...
April 29, 2017: Journal of Pharmaceutical Sciences
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