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https://www.readbyqxmd.com/read/28987259/primary-sclerosing-cholangitis-what-the-gastroenterologist-and-hepatologist-needs-to-know
#1
REVIEW
Andrea A Gossard, Gregory J Gores
Primary sclerosing cholangitis (PSC) is a chronic, idiopathic biliary tract disease characterized by segmental strictures. The disease is progressive with no proven treatments and may eventually lead to cirrhosis and end-stage liver disease. Abrupt changes in liver biochemistries, pain, and/or cholangitis may suggest a dominant stricture amenable to endoscopic therapy or the development of cholangiocarcinoma. Patients with PSC are at increased risk of cholangiocarcinoma. There is a strong association with inflammatory bowel disease, and an associated increased risk of colorectal cancer...
November 2017: Clinics in Liver Disease
https://www.readbyqxmd.com/read/28987258/an-update-on-the-treatment-and-follow-up-of-patients-with-primary-biliary-cholangitis
#2
REVIEW
Blaire E Burman, Manan A Jhaveri, Kris V Kowdley
Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by chronic granulomatous lymphocytic cholangitis of the small bile ducts. PBC was a leading indication for liver transplant in the United States; with early diagnosis and treatment, the majority of patients with PBC have a normal life expectancy. Pathogenesis involves inflammatory damage of bile duct epithelium secondary to innate and adaptive immune responses, and toxicity from accumulated bile acids. Cholestasis and disease progression can lead to cirrhosis...
November 2017: Clinics in Liver Disease
https://www.readbyqxmd.com/read/28983725/liver-transplantation-for-biliary-atresia-a-systematic-review
#3
REVIEW
Mureo Kasahara, Koji Umeshita, Seisuke Sakamoto, Akinari Fukuda, Hiroyuki Furukawa, Shinji Uemoto
Biliary atresia (BA) is an idiopathic neonatal cholangiopathy characterized by progressive inflammatory obliteration of the intrahepatic or extrahepatic bile ducts. Although the Kasai operation has dramatically improved the outcomes in children with BA, most patients with BA eventually require liver transplantation (LT) even after undergoing a successful Kasai procedure. The Japanese LT Society (JLTS) was established in 1980 to characterize and follow trends in patient characteristics and the graft survival among all liver transplant patients in Japan...
October 5, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28983658/newborn-screening-for-biliary-atresia-in-the-united-states
#4
Cat Goodhue, Michael Fenlon, Kasper S Wang
Despite advances in our understanding of the pathogenesis of biliary atresia (BA), BA remains the most common cause of end-stage liver disease in children and the leading indication for pediatric liver transplantation. Age at time of Kasai portoenterostomy (KPE), performed to provide bile drainage, strongly correlates with transplant-free survival, mostly due to progression of intrahepatic fibrosis to cirrhosis. Unfortunately, challenges remain in recognizing that a jaundiced infant may have BA. To better diagnose infants with BA at an earlier age, population-based screening programs in countries such as Taiwan, Japan, and China have utilized stool color cards...
October 5, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28980051/surgical-modifications-additions-and-alternatives-to-kasai-hepato-portoenterostomy-to-improve-the-outcome-in-biliary-atresia
#5
REVIEW
Shilpa Sharma, Devendra K Gupta
Kasai hepato-portoenterostomy (HPE) is the most widely used surgical technique to restore bile flow in biliary atresia (BA). We aimed to review literature on HPE substitutes and additions to Kasai especially in advanced BA (ABA). A PubMed search was done for surgical procedures apart from or along with Kasai HPE for BA. Additional procedures to prevent cholangitis were also reviewed. Procedures and outcome were analysed. Alternative procedures done by the authors have also been described briefly. Results have been compiled in this review article...
October 4, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28975833/the-role-of-diagnosis-and-treatment-of-underlying-liver-disease-for-the-prognosis-of-primary-liver-cancer
#6
Ashok Shiani, Shreya Narayanan, Luis Pena, Mark Friedman
Hepatocellular carcinoma (HCC) is the third leading cause of cancer-related deaths worldwide. Underlying chronic liver disease has been associated with an increased risk of developing HCC. This study is a review of the current literature regarding the diagnosis, prognostic significance, and role of treating underlying liver disease in patients who are at risk of primary liver cancer. Relevant peer review of the English literature between 1980 and 2017 within PubMed and the Cochrane library was conducted for scientific content on current advances in managing chronic liver diseases and the development of hepatocellular carcinoma...
July 2017: Cancer Control: Journal of the Moffitt Cancer Center
https://www.readbyqxmd.com/read/28953318/terlipressin-versus-other-vasoactive-drugs-for-hepatorenal-syndrome
#7
REVIEW
Mads Israelsen, Aleksander Krag, Andrew S Allegretti, Manol Jovani, Alison H Goldin, Rachel W Winter, Lise Lotte Gluud
BACKGROUND: Hepatorenal syndrome is defined as severe renal failure occurring in people with cirrhosis and ascites. Systematic reviews of randomised clinical trials found that, compared with placebo, terlipressin may reduce mortality and improve renal function in people with hepatorenal syndrome, but we need current evidence from systematic reviews on the benefits and harms of terlipressin versus other vasoactive drugs. OBJECTIVES: To evaluate the beneficial and harmful effects of terlipressin versus other vasoactive drugs for people with hepatorenal syndrome...
September 27, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28951510/increased-cholestatic-enzymes-in-two-patients-with-long-term-history-of-ulcerative-colitis-consider-primary-biliary-cholangitis-not-always-primary-sclerosing-cholangitis
#8
Erietta Polychronopoulou, Vasiliki Lygoura, Nikolaos K Gatselis, George N Dalekos
Several hepatobiliary disorders have been reported in ulcerative colitis (UC) patients with primary sclerosing cholangitis (PSC) being the most specific. Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, rarely occurs in UC. We present two PBC cases of 67 and 71 years who suffered from long-standing UC. Both patients were asymptomatic but they had increased cholestatic enzymes and high titres of antimitochondrial antibodies (AMA)-the laboratory hallmark of PBC. After careful exclusion of other causes of cholestasis by MRI/magnetic resonance cholangiopancreatography (MRCP), virological and microbiological investigations, a diagnosis of PBC associated with UC was established...
September 25, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28951229/effect-of-nucleos-t-ide-analogue-therapy-on-risk-of-intra-hepatic-cholangioarcinoma-in-patients-with-chronic-hepatitis-b
#9
Teng-Yu Lee, Yao-Chun Hsu, Shi-Hang Yu, Jaw-Town Lin, Ming-Shiang Wu, Chun-Ying Wu
BACKGROUND & AIMS: Chronic infection with hepatitis B virus (HBV) increases risk of intrahepatic cholangiocarcinoma (ICC), but it is not clear whether antiviral therapy reduces risk. We investigated the association between nucleos(t)ide analogue therapy and ICC risk. METHODS: We performed a nationwide long-term cohort study using Taiwan's National Health Insurance Research Database to obtain data on 185,843 patients with chronic HBV infection from October 1, 2003 through December 31, 2012...
September 22, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28948408/association-between-primary-biliary-cholangitis-and-osteoporosis-meta-analysis
#10
Junyu Fan, Qian Wang, Lingyun Sun
The relationship between primary biliary cholangitis (PBC, previously termed primary biliary cirrhosis) and risks of osteoporosis remains controversial. This meta-analysis was designed to comprehensively analyze the association between PBC and osteoporosis. We conducted a systematic literature search of the PubMed, EMBASE, and Web of Science. Either fixed or random effects models were applied to assess bone mineral density (BMD), osteoporosis rates, and fractures in PBC patients and normal controls. A total of eight studies were included (including 1643 PBC patients and 10,921 controls)...
November 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28940041/a-review-of-long-term-outcome-and-quality-of-life-of-patients-after-kasai-operation-surviving-with-native-livers
#11
REVIEW
Kenneth K Y Wong, Carol W Y Wong
Biliary atresia (BA) is a rare neonatal cholestatic disease which leads to progressive obliterative cholangiopathy, resulting in biliary obstruction and jaundice. The standard surgical treatment is hepatoportoenterostomy (Kasai operation). Although approximately 50% of the affected infants would require liver transplantation within the first 2 years of life, the other 50% of the patients can live for years with their native liver, despite the progression of cirrhosis and chronic liver disease. Many of these patients will be affected by long-term complications such as repeated cholangitis, portal hypertension, variceal bleeding, growth problems, biochemical abnormalities, and hepatic osteodystrophy...
September 22, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28935574/taurocholate-induces-biliary-differentiation-of-liver-progenitor-cells-causing-hepatic-stellate-cell-chemotaxis-in-the-ductular-reaction-role-in-pediatric-cystic-fibrosis-liver-disease
#12
Katarzyna N Pozniak, Michael A Pearen, Tamara N Pereira, Cynthia Sm Kramer, Priyakshi Kalita-De Croft, Sujeevi K Nawaratna, Manuel A Fernandez-Rojo, Geoffrey N Gobert, Janina Ee Tirnitz-Parker, John K Olynyk, Ross W Shepherd, Peter J Lewindon, Grant A Ramm
Cystic fibrosis liver disease (CFLD) in children causes progressive fibrosis leading to biliary cirrhosis, however its cause(s) and early pathogenesis are unclear. We hypothesised that a bile acid-induced Ductular Reaction (DR) drives fibrogenesis. We evaluated the DR by cytokeratin-7 immunohistochemistry in liver biopsies, staged for fibrosis, from 60 children with CFLD and demonstrated that the DR was significantly correlated with hepatic fibrosis stage and biliary taurocholate levels. To examine the mechanisms involved in DR induction liver progenitor cells (LPCs) were treated with taurocholate and key events in DR evolution were assessed: LPC proliferation, LPC biliary differentiation and hepatic stellate cell (HSC) chemotaxis...
September 18, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28933417/serum-wisteria-floribunda-agglutinin-positive-mac-2-binding-protein-could-not-always-predict-early-cirrhosis-in-non-viral-liver-diseases
#13
Yuki Haga, Tatsuo Kanda, Reina Sasaki, Masato Nakamura, Koji Takahashi, Shuang Wu, Shin Yasui, Makoto Arai, Shingo Nakamoto, Osamu Yokosuka
BACKGROUND: Wisteria floribunda agglutinin-positive human Mac-2-binding protein (WFA(+)-M2BP) is a novel non-invasive marker of liver fibrosis. The goal of the study was to investigate whether the novel serum biomarker WFA(+)-M2BP or other non-invasive markers are useful for the prediction of liver fibrosis in patients with nonalcoholic steatohepatitis (NASH), autoimmune hepatitis (AIH), and primary biliary cholangitis (PBC). METHODS: We examined a significant correlation between serum WFA(+)-M2BP levels and histological staging of fibrosis in several chronic liver diseases, such as NASH, AIH, and PBC...
December 14, 2016: Diseases (Basel)
https://www.readbyqxmd.com/read/28922436/genetic-risk-factors-for-autoimmune-thyroid-disease-might-affect-the-susceptibility-to-and-modulate-the-progression-of-primary-biliary-cholangitis
#14
Aleksander Kuś, Magdalena Arłukowicz-Grabowska, Konrad Szymański, Ewa Wunsch, Małgorzata Milkiewicz, Rafał Płoski, Zakera Shums, Gary L Norman, Piotr Milkiewicz, Tomasz Bednarczuk, Marcin Krawczyk
BACKGROUND AND AIMS: Patients with primary biliary cholangitis (PBC) frequently suffer from extrahepatic autoimmune conditions, of which autoimmune thyroid disease (AITD) is one of the most common. Previous studies identified several genetic variants increasing the odds of developing AITD. Here we investigate whether AITD-associated polymorphisms might also play a role in the development and clinical course of PBC and PBC associated with AITD (PBC-AITD). METHODS: To this end, we prospectively recruited 230 patients with PBC and 421 healthy controls...
September 2017: Journal of Gastrointestinal and Liver Diseases: JGLD
https://www.readbyqxmd.com/read/28919271/-autoimmune-hepatitis-immunological-diagnosis
#15
Imane Brahim, Ikram Brahim, Raja Hazime, Brahim Admou
Autoimmune hepatopathies (AIHT) including autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune cholangitis (AIC), represent an impressive entities in clinical practice. Their pathogenesis is not perfectly elucidated. Several factors are involved in the initiation of hepatic autoimmune and inflammatory phenomena such as genetic predisposition, molecular mimicry and/or abnormalities of T-regulatory lymphocytes. AIHT have a wide spectrum of presentation, ranging from asymptomatic forms to severe acute liver failure...
September 14, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28913747/primary-sj%C3%A3-gren-s-syndrome-extraglandular-manifestations-and-hydroxychloroquine-therapy
#16
J Demarchi, S Papasidero, M A Medina, D Klajn, R Chaparro Del Moral, O Rillo, V Martiré, G Crespo, A Secco, A Catalan Pellet, C Amitrano, C Crow, C Asnal, P Pucci, F Caeiro, N Benzanquen, J P Pirola, M Mayer, F Zazzetti, S Velez, J Barreira, N Tamborenea, L Santiago, L Raiti
The use of hydroxychloroquine (HCQ) in Primary Sjögren's Syndrome (pSS) has been assessed in different studies over the last years, with conflicting results regarding its efficacy in sicca syndrome and extraglandular manifestations (EGM). The goal of this study was to compare the incidence rate of EGM in pSS patients with and without HCQ therapy.We performed a multicenter retrospective study, including patients with pSS (European classification criteria) with at least 1 year of follow-up. Subjects with concomitant fibromyalgia, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis were excluded...
November 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28913620/a-brief-review-on-prognostic-models-of-primary-biliary-cholangitis
#17
REVIEW
Sha Chen, Weijia Duan, Hong You, Jidong Jia
Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune liver disease characterized by progressive destruction of small intrahepatic bile ducts. If left untreated, PBC may eventually result in end-stage liver disease. For better management of PBC and optimal allocation of medical resources, it is pivotal to accurately estimate the prognosis of patients with PBC. This article will briefly review the models that predict long-term outcome of PBC patients, with special focus on the applicability, strengths and limitations of the widely used models reported from 1983 to 2016...
September 14, 2017: Hepatology International
https://www.readbyqxmd.com/read/28902846/downregulation-of-microrna-145-may-contribute-to-liver-fibrosis-in-biliary-atresia-by-targeting-add3
#18
Yongqin Ye, Zhihan Li, Qi Feng, Zimin Chen, Zhouguang Wu, Jianyao Wang, Xiaoshuo Ye, Dahao Zhang, Lei Liu, Wei Gao, Lihui Zhang, Bin Wang
BACKGROUND AND OBJECTIVES: Biliary atresia (BA) is a pediatric liver disease characterized by fibro-obliteration and obstruction of the extrahepatic biliary system, that invariably leads to cirrhosis and even death, if left untreated for extended time. However, its pathology and etiology still remained unknown. In this study, we tested the expression of adducin 3 (ADD3), the gene identified as a susceptibility gene in BA by GWAS, and uncovered its upstream regulatory microRNA in the pathogenesis of BA...
2017: PloS One
https://www.readbyqxmd.com/read/28893408/inflammatory-myopathy-associated-with-antimitochondrial-antibodies-a-distinct-phenotype-with-cardiac-involvement
#19
Jemima Albayda, Aamna Khan, Livia Casciola-Rosen, Andrea M Corse, Julie J Paik, Lisa Christopher-Stine
OBJECTIVE: In the context of clinical evaluations performed on our prospective myositis cohort, we noted a striking association of severe cardiac disease in myositis patients with antimitochondrial antibodies. We sought to review all cases of antimitochondrial antibody (AMA) associated myositis in our cohort to describe the clinical features of this disease subset. METHODS: We identified 7 patients with confirmed antimitochondrial antibodies who presented as an inflammatory myopathy...
June 13, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28893353/the-cholangiocyte-adenosine-il6-axis-regulates-survival-during-biliary-cirrhosis
#20
Elise Lavoie, Michel Fausther, Jessica Goree, Jonathan Dranoff
BACKGROUND AND AIMSEpithelial response to injury is critical to the pathogenesis of biliary cirrhosis, and IL6 has been suggested as a mediator of this phenomenon. Several liver cell types can secrete IL6 following activation by various signaling molecules including circulating adenosine. The aims of this study are to assess whether adenosine can induce IL6 secretion by cholangiocytes via the A2b adenosine receptor (A2bAR) and determine the effect of A2bAR-sensitive IL6 release on injury response in biliary cirrhosis...
September 11, 2017: Gene Expression
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