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biliary cirrhosis

Chen-Yi Liao, Chi-Hsiang Chung, Pauling Chu, Kuang-Yu Wei, Tseng-Min Feng, Fu-Huang Lin, Chang-Huei Tsao, Chia-Chao Wu, Wu-Chien Chien
BACKGROUND: We evaluated the risk of osteoporosis in patients with primary biliary cirrhosis (PBC) using a nationwide population-based dataset. METHODS: In a cohort study of 986,713 individuals, we selected 2,493 PBC patients who were aged 18 years or older and had been diagnosed with PBC, based on the International Classification of Disease (ICD-9-CM) codes 571.6, during 20002010. The control cohort comprised 9,972 randomly selected, propensity matched patients (by age, gender, and index date), without PBC...
2018: PloS One
B M Kamath, A Baker, R Houwen, L Todorova, N Kerkar
BACKGROUND: Alagille syndrome (ALGS) is an inherited multisystem disorder typically manifesting as cholestasis, and potentially leading to end-stage liver disease and death. AIM: To perform the first systematic review of the epidemiology, natural history and burden of ALGS with a focus on the liver component. METHODS: Electronic databases and proceedings from key congresses were searched in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2009 guidelines...
March 14, 2018: Journal of Pediatric Gastroenterology and Nutrition
Lars Bossen, Henriette Ytting, Peter Jepsen, Ole Hamberg, Peter Ott, Henning Grønbæk
The name of chronic liver disease: primary biliary cirrhosis, has been changed to: primary biliary cholangitis, primarily because of the stigma associated with the word "cirrhosis", as only a minority of the patients develop cirrhosis. In this review we present data on epidemiology and discuss the current treatments with focus on ursodeoxycholic acid and the newly described effects of the farnesoid receptor agonist obeticholic acid.
March 5, 2018: Ugeskrift for Laeger
M Garrido, C Escobar, C Zamora, C Rejas, J Varas, C Córdova, C Papuzinski, M Párraga, S San Martín, S Montedonico
Purpose: Biliary atresia precedes liver cirrhosis and liver transplantation. Amniotic membrane (AM) promotes tissue regeneration, inhibits fibrosis, and reduces inflammation. Here, we test amniotic membrane potential as a therapeutic tool against cholestatic liver fibrosis. Methods: Three groups of rats were used: sham surgery (SS), bile duct ligature (BDL), and bile duct ligature plus human amniotic membrane (BDL + AM). After surgery, animals were sacrificed at different weeks...
2018: Stem Cells International
Yue Yang, Long Chen, Yuan Jia, Yang Liu, Lei Wen, Yaoxian Liang, Yuan An, Shi Chen, Yin Su, Zhanguo Li
To analyze the clinical spectrum, laboratory characteristics, and outcomes of monoclonal gammopathy (MG) in patients with rheumatic diseases. Screening for the presence of MG was performed in 872 inpatients with rheumatic diseases from January 2010 to July 2017. A total of 41 patients were enrolled. Their clinical and biological features in addition to outcomes were described. For each patient with primary Sjögren syndrome (pSS), 2 age- and sex-matched pSS patients without MG were selected as controls. Risk factors for the presence of MG and malignant hematological neoplasias were assessed...
March 13, 2018: Clinical Rheumatology
Albert Parés
Primary cholangitis (cirrhosis) is a chronic cholestatic disease with an unquestionable female predominance. It is characterised by inflammation of the small and medium size bile ducts, and can eventually progress to cirrhosis. Most patients remain asymptomatic and are diagnosed by the casual finding of an anicteric biochemical cholestasis with increased alkaline phosphatase. The pathogenesis is unknown and of presumed autoimmune origin in genetic susceptible subjects. M2-type antimitochondrial antibodies, and specific antinuclear antibodies (gp210 and Sp100) are typical and specific of the disease...
March 8, 2018: Medicina Clínica
Nehal El-Koofy, Hanan M Fouad, Mona E Fahmy, Heba Helmy, Olfat Shaker, Hanaa M El-Karaksy, Nabil Mohsen
BACKGROUND AND STUDY AIMS: Hepatobiliary cholestatic disorders produce excess copper (Cu) retention in the liver, which is toxic and may cause hepatitis, fulminant hepatic failure, cirrhosis and death. In this study, we measured hepatic Cu and tested its correlation with serum Cu (S. Cu) and serum ceruloplasmin (S. ceruloplasmin) in cholestatic infants. PATIENTS AND METHODS: 41 cholestatic infants were enrolled as cases and 11 healthy infants as control subjects...
March 6, 2018: Arab Journal of Gastroenterology: the Official Publication of the Pan-Arab Association of Gastroenterology
Omar Toumi, Mohamed Ali Chaouch, Abdesslem Ghedira, Ibtissem Korbi, Mohamed Nasr, Faouzi Noomene, Khadija Zouari, Randa Salem, Badii Hamida, Mondher Golli
INTRODUCTION: Congenital bile duct cysts (CBDC) is a rare congenital malformation. It results from an anomaly of the biliopancreatic junction (AJBP). This condition is often diagnosed at a young age. Although, it can be asymptomatic and discovered only at an advanced age. The aim of our work was to describe the diagnosis, therapeutic and evolutionary aspects of BVCD through a series of 11 cases diagnosed in adult cases. METHODS: This is a descriptive, retrospective and monocentric study...
June 2017: La Tunisie Médicale
Peng Zhang, Qianjin Lu
Immunological tolerance loss is fundamental to the development of autoimmunity; however, the underlying mechanisms remain elusive. Immune tolerance consists of central and peripheral tolerance. Central tolerance, which occurs in the thymus for T cells and bone marrow for B cells, is the primary way that the immune system discriminates self from non-self. Peripheral tolerance, which occurs in tissues and lymph nodes after lymphocyte maturation, controls self-reactive immune cells and prevents over-reactive immune responses to various environment factors...
March 5, 2018: Cellular & Molecular Immunology
Kyohei Abe, Hiroaki Shiba, Kenei Furukawa, Taro Sakamoto, Yuichi Ishida, Katsuhiko Yanaga
Liver transplant recipients are considered to be at high risk for Clostridium difficile infection, with an incidence of 2.7-8.0%, which is three times higher than that among other patients. A case of a patient who suffered from pseudomembranous colitis five times after living donor liver transplantation is reported. A 60-year-old woman underwent splenectomy and living donor liver transplantation using the left lobe of her spouse for primary biliary cirrhosis. The patient made a satisfactory recovery, except for splenic vein thrombosis...
March 1, 2018: Clinical Journal of Gastroenterology
Jennifer Sammon, Sandra Fischer, Ravi Menezes, Hooman Hosseini-Nik, Sara Lewis, Bachir Taouli, Kartik Jhaveri
BACKGROUND: Combined hepatocellular-cholangiocarcinoma (cHCC-CC) is a rare primary liver tumor, which has overlapping imaging features with mass forming intra-hepatic cholangiocarcinoma (ICC) and hepatocellular carcinoma (HCC). Previous studies reported imaging features more closely resemble ICC and the aim of our study was to examine the differential MRI features of cHCC-CC and ICC with emphasis on enhancement pattern observations of gadolinium enhanced MRI. METHODS: Institutional review board approval with consent waiver was obtained for this retrospective bi-centric study...
February 27, 2018: Cancer Imaging: the Official Publication of the International Cancer Imaging Society
Melisa Dirchwolf, Sebastián Marciano, Diego H Giunta, Maria L Posadas-Martínez, Scott W Biggins, Andrés E Ruf
BACKGROUND: Hepatitis C virus (HCV) related cirrhosis is a leading indication for liver transplantation (LT) worldwide. Access to effective HCV treatment is inequitable globally. We aimed to analyze whether the introduction of effective HCV treatment caused an impact in LT trends in a middle-income country. METHODS: Cross sectional analysis of all adult patients who were listed/received a LT in Argentina for HCV, alcohol-related liver disease (ALD) or autoimmune hepatitis/primary biliary cirrhosis (AIH/PBC) from 2007 to 2017...
February 27, 2018: Clinical Transplantation
Tapas K Tejura, Alejandro Pita, Christian Romero, Yuri S Genyk, Linda Sher, Suzanne L Palmer
PURPOSE: To identify long-term post-operative imaging findings resulting from right lobe liver donation. METHODS: This retrospective imaging review consisted of 42 adults (20 males, 22 females, mean age 36.6 years, range 18.7-55.9 years) who underwent right lobe liver donation with pre- and post-operative imaging between 1999 and 2006. The mean follow-up period was 30.2 months (range 12.3-69.6 months). Follow-up imaging evaluation included assessment of the biliary tree, particularly isolated bile ducts draining to and terminating at the cut surface (orphan ducts)...
February 22, 2018: Abdominal Radiology
Xin Yan, Jinglan Jin
RATIONALE: Primary cutaneous amyloidosis (PCA) is a localized skin disorder characterized by the abnormal deposition of amyloid in the extracellular matrix of the dermis. The association between PCA and other diseases, although rare, has been documented for various autoimmune diseases. PCA associated with autoimmune hepatitis-primary biliary cirrhosis (AIH-PBC) overlap syndrome and Sjögren syndrome (SS) has not been previously reported in the literature. PATIENT CONCERNS: A 50-year-old woman presented with progressive abnormal liver enzyme levels and was referred to our department...
February 2018: Medicine (Baltimore)
Pablo Galindo Orrego, Janeth Bejarano Rengifo, Christian Labrador López, Geovanny Hernández Cely, Oscar Alfredo Beltrán Galvis, Martín Alonso Garzón Olarte, Enrique Ponce de León Chaux, Jorge Alberto Ceballos Hurtado, Diana Carolina Salinas Gómez, Adriana Varón Puerta
Bronchobiliary fistula (BBF) is an abnormal communication between the biliary tract and the bronchial tree. Is an infrequent condition, usually secondary to a local infectious process or a traumatic event. Bilioptisis is pathognomonic. We present the case of a 37 year old woman with secondary biliary cirrhosis, in list for liver transplantation, with several episodes of cholangitis and carrier of external biliary diverivation, who presented bilioptisis and HIDA scintigraphy with SPECT confirmed BBF. This case was resolved with percutaneous derivation of the biliary tract...
October 2017: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
Diego Andrés Rodríguez Lugo, Jorge Julián Coronado Tovar, Giovana Alejandra Solano Villamarin, Wiliam Otero Regino
Primary biliary cholangitis (PBC) is a chronic autoimmune cholangiopathy characterized by a selective destruction of biliary epithelial cells of small and medium caliber hepatic ducts, which mainly affects women. The main symptoms are fatigue and pruritus, however, a large proportion of patients may be asymptomatic. The diagnosis is based on AMA titers >1:40, alkaline phosphatase >1.5 times the upper limit for more than 24 weeks and compatible liver histology. It is associated with multiple autoimmune diseases mainly extrahepatic, thyroid diseases, bone diseases, among others...
October 2017: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
Himerón Limaylla-Vega, Emilio Vega-Gonzales
Iatrogenic bile duct injuries (IBDI) represent a serious surgical complication of laparoscopic cholecystectomy (LC). Often it occurs when the bile duct merges with the cystic duct; and they have been ranked by Strasberg and Bismuth, depending on the degree and level of injury. About third of IBDI recognized during LC, to detect bile leakage. No immediate repair is recommended, especially when the lesion is near the confluence or inflammation is associated. The drain should be established to control leakage of bile and prevent biliary peritonitis, before transferring the patient to a specialist in complex hepatobiliary surgery facility...
October 2017: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
Palittiya Sintusek, Nawarat Posuwan, Piyaporn Wanawongsawad, Suttiruk Jitraruch, Yong Poovorawan, Voranush Chongsrisawat
AIM: To assess the seroprevalence of hepatitis B virus (HBV) immunity among previously vaccinated pediatric liver transplant recipients and present a case report of de novo hepatitis B infection after liver transplantation. METHODS: This study focused on children with chronic liver diseases who received primary hepatitis B immunization and had a complete dataset of anti-HBs before and after liver transplantation between May 2001 and June 2017. Medical records were retrospectively reviewed for potential factors relating to HBV immunity loss...
February 14, 2018: World Journal of Gastroenterology: WJG
Juan S Rubio, Carolina Rumbo, Pablo A Farinelli, Nicolás Aguirre, Diego A Ramisch, Hugo Paladini, Pablo D Angelo, Pablo Barros Schelotto, Gabriel E Gondolesi
Collateral circulation secondary to liver cirrhosis may cause the development of large PSSs that may steal flow from the main portal circulation. It is important to identify these shunts prior to, or during the transplant surgery because they might cause an insufficient portal flow to the implanted graft. There are few reports of "steal flow syndrome" cases in pediatrics, even in biliary atresia patients that may have portal hypoplasia as an associated malformation. We present a 12-month-old female who received an uneventful LDLT from her mother, and the GRWR was 4...
February 16, 2018: Pediatric Transplantation
Tengda Li, Yuanlan Huang, Peng Liu, Yun Liu, Jie Guo, Weiwei Zhang, Mingli Gu, Cheng Qian, Anmei Deng
Primary biliary cirrhosis (PBC) is an autoimmune liver disease. Its histological characteristics, such as progressive intrahepatic bile duct destruction, cholestasis, and liver cirrhosis, are caused by the body's autoimmune disorders. Interleukin (IL)-35 has two subunits (p35 and Ebi3) and is a member of the IL-12 family of heterodimeric cytokines. IL-35 has immunosuppressive functions and plays an important role in many autoimmune diseases. In this study, we compared plasma levels of IL-35 and relative mRNA expression levels of p35 and Ebi3 in peripheral blood mononuclear cells (PBMCs) from 70 PBC patients and 70 healthy individuals...
2018: Tohoku Journal of Experimental Medicine
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