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Intrahepatic Cholestasis

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https://www.readbyqxmd.com/read/29445068/lower-plasma-levels-of-il-35-in-patients-with-primary-biliary-cirrhosis
#1
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
https://www.readbyqxmd.com/read/29431335/open-label-study-of-ademetionine-for-the-treatment-of-intrahepatic-cholestasis-associated-with-alcoholic-liver-disease
#2
Vladimir T Ivashkin, Marina V Maevskaya, Zhanna D Kobalava, Yuriy P Uspenskiy, Julia A Fominih, Alexander V Rozanov, Veronica V Tolkacheva, Tatiana I Sotnikova, Bagdadi A Alikhanov, Irina A Gorbacheva, Olga B Ershova, Antonina A Znakhyrenko, Kirill A Sokolov, Suntje Sander-Struckmeier
BACKGROUND: The effect of oral and/or parenteral ademetionine (500 mg intravenous [IV] and tablet formulation) on clinical symptoms and biochemical markers of intrahepatic cholestasis (IHC) was investigated in subjects with alcoholic liver disease (ALD) and compensated liver function. METHODS: Prospective, multicentre, open-label study consisting of a screening period and an 8-week treatment period and performed in subjects (18-75 years) with compensated ALD and confirmed IHC...
February 8, 2018: Minerva Gastroenterologica e Dietologica
https://www.readbyqxmd.com/read/29419856/-analysis-of-slc25a13-gene-mutations-in-five-infants-with-neonatal-intrahepatic-cholestasis-caused-by-citrin-deficiency
#3
Junjie Xu, Min Gao, Yuqiang Lyu, Yunping Tang, Xuxia Wei, Lu Yang, Kaihui Zhang, Yi Liu, Zhongtao Gai
OBJECTIVE To identify potential mutations in five infants with neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD). METHODS The SLC25A13 gene was analyzed by next-generation sequencing. Suspected mutations were confirmed by PCR and Sanger sequencing in the probands and their parents. Impact of novel mutations was predicted with PolyPhen-2 software. RESULTS All neonates have harbored mutations of the SLC25A13 gene. Eight mutations were discovered, which included two novel mutations (c.1357A>G and c...
February 10, 2018: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
https://www.readbyqxmd.com/read/29419378/does-ursodeoxycholic-acid-improve-perinatal-outcomes-in-women-with-intrahepatic-cholestasis-of-pregnancy
#4
Lucy C Chappell, Jenny Chambers, Jim G Thornton, Catherine Williamson
No abstract text is available yet for this article.
February 1, 2018: BMJ: British Medical Journal
https://www.readbyqxmd.com/read/29412511/comprehensive-bile-acid-profiling-in-hereditary-intrahepatic-cholestasis-genetic-and-clinical-correlations
#5
Teng Liu, Ren-Xue Wang, Jun Han, Chen-Zhi Hao, Yi-Ling Qiu, Yan-Yan Yan, Li-Ting Li, Neng-Li Wang, Jing-Yu Gong, Yi Lu, Mei-Hong Zhang, Xin-Bao Xie, Jun-Cong Yang, Yi-Jie You, Jia-Qi Li, A S Knisely, Christoph H Borchers, Victor Ling, Jian-She Wang
BACKGROUND & AIMS: Genetic defects causing dysfunction in bile salt export pump (BSEP/ABCB11) lead to liver diseases. ABCB11 mutations alter the bile acid metabolome. We asked if profiling plasma bile acids could reveal compensatory mechanisms and track genetic and clinical status. METHODS: We compared plasma bile acids in 17 ABCB11-mutated patients, 35 healthy controls, and 12 genetically undiagnosed cholestatic patients by ultrahigh-performance liquid chromatography/multiple-reaction monitoring-mass spectrometry (UPLC/MRM-MS)...
February 7, 2018: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/29407775/different-effects-of-ursodeoxycholic-acid-on-intrahepatic-cholestasis-in-acute-and-recovery-stages-induced-by-alpha-naphthylisothiocyanate-in-mice
#6
Linlin Zhang, Huizong Su, Yue Li, Yujuan Fan, Qian Wang, Jian Jiang, Yiyang Hu, Gaofeng Chen, Bo Tan, Furong Qiu
The aim of this study was to determine the effect of ursodeoxycholic acid (UDCA) on the alpha-naphthylisothiocyanate (ANIT)-induced acute and recovery stage of cholestasis model mice. In the acute stage of model mice, pretreatment with UDCA (25, 50, and 100 mg·kg-1, ig) for 12 days prior to ANIT administration (50 mg·kg-1, ig) resulted in the dramatic increase in serum biochemistry, with aggrevation of bile infarcts and hepatocyte necrosis. The elevation of beta-muricholic acid (β-MCA), cholic acid (CA), and taurocholic acid (TCA) in serum and liver, and reduction of these bile acids (BAs) in bile was observed...
February 1, 2018: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/29404523/can-genetic-testing-guide-the-therapy-of-cholestatic-pruritus-a-case-of-benign-recurrent-intrahepatic-cholestasis-type-2-with-severe-nasobiliary-drainage-refractory-itch
#7
Robert Holz, Andreas E Kremer, Dieter Lütjohann, Hermann E Wasmuth, Frank Lammert, Marcin Krawczyk
Benign recurrent intrahepatic cholestasis (BRIC) is a peculiar familial disease caused by mutations of the genes encoding hepatocanalicular flippase for phosphatidylserine (ATP8B1; BRIC type 1) or the bile salt export pump (ABCB11; BRIC type 2). Here, we report on a patient with nasobiliary drainage-refractory BRIC type 2 who improved under plasma separation and anion absorption therapy. We also suggest that nasobiliary drainage might be an ineffective approach in carriers of severe loss-of-function mutations of the bile salt export pump ABCB11...
February 2018: Hepatology Communications
https://www.readbyqxmd.com/read/29390323/liver-transplantation-for-decompensated-liver-cirrhosis-caused-by-progressive-familial-intrahepatic-cholestasis-type-3-a-case-report
#8
Deng Xiang, Jiannan He, Hongmei Wang, Fangfang Xiong, Hao Cheng, Junhua Ai, Renfeng Shan, Renhua Wan, Lunli Zhang, Jun Shi
RATIONALE: Progressive familial intrahepatic cholestasis (PFIC) type 3, characterized by high gamma glutamyl transferase (GGT), is an autosomal recessive genetic disease. It often occurs in patients' first years of age. However, high GGT type PFIC is still rare. PATIENT CONCERNS: The present study reports a case of liver transplantation for decompensated liver cirrhosis caused by PFIC type 3. An 18-year-old male presented with a history of abdominal distension and jaundice for 2 months...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29388792/transcription-factor-gata6-a-novel-marker-and-putative-inducer-of-ductal-metaplasia-in-biliary-atresia
#9
Tea Soini, Marjut Pihlajoki, Noora Andersson, Jouko Lohi, Kari A Huppert, David A Rudnick, Stacey S Huppert, David B Wilson, Mikko P Pakarinen, Markku Heikinheimo
Biliary atresia (BA), a neonatal liver disease, is characterized by obstruction of extrahepatic bile ducts with subsequent cholestasis, inflammation, and progressive liver fibrosis. To gain insights into the pathophysiology of BA, we focused attention on GATA6, a transcription factor implicated in biliary development. Early in fetal development GATA6 expression is evident in cholangiocytes and hepatocytes, but by late gestation it is extinguished in hepatocytes. Utilizing a unique set of BA liver samples collected before and after successful portoenterostomy (PE), we found that GATA6 expression is markedly upregulated in hepatocytes of patients with BA compared to healthy and cholestatic disease controls...
February 1, 2018: American Journal of Physiology. Gastrointestinal and Liver Physiology
https://www.readbyqxmd.com/read/29384912/intrahepatic-cholestasis-of-pregnancy-or-azithromycin-induced-intrahepatic-cholestasis-a-case-report
#10
Baoshi Han, Yan Sheng, Li Wang, Hao Feng, Xianzeng Hou, Yan Li
RATIONALE: Azithromycin-induced liver injury has been rarely reported in adult individuals, let alone in a pregnant woman. Here, we describe the clinical features and outcomes of azithromycin-induced liver injury in a pregnant woman. PATIENT CONCERNS: A 30-year-old pregnant woman presented with generalized pruritus and elevated serum bile acid level (123.6 μmol/L) on day 4 of azithromycin administration. A diagnosis of intrahepatic cholestasis of pregnancy was made, and cesarean section was performed immediately...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29372374/cysto-cholecystostomy-a-more-physiological-procedure-for-hepatic-cysts-with-biliary-communications-and-cystic-dilatations-of-main-intrahepatic-ducts
#11
Mei Diao, Long Li, Wei Cheng
OBJECTIVE: Hepatic cysts with biliary communications (HC) and cystic dilatations of main intrahepatic ducts (CIHD) can cause biliary obstruction, cholestasis, stone formation, cholangitis, liver damage and carcinoma. Conventionally, Roux-Y cysto-jejunostomy is employed to manage these conditions. However, it is technically demanding and may be complicated with major biliary disruption and bacteria migration from intestine to intrahepatic duct. We have carried out laparoscopic cysto-cholecystostomies for HC with biliary communication and CIHD and evaluated outcomes...
January 25, 2018: World Journal of Surgery
https://www.readbyqxmd.com/read/29366351/histomorphometric-changes-in-the-placenta-and-umbilical-cord-during-complications-of-pregnancy
#12
D Güven, B Z Altunkaynak, G Altun, I Alkan, I Kocak
Pregnancy complications may cause morphological changes and circulation defects in the placenta, which may lead to morbidity and mortality in fetuses and newborns. We investigated structural changes in the placenta and umbilical cord under various abnormal maternal conditions. Placenta and umbilical cord specimens were obtained from pregnant women during labor at 37 - 42 weeks gestation. Volumetric measurements were made for each placenta and umbilical cord using the Cavalieri method. Significant differences were observed for volumetric densities of total villi, syncytial knots, intervillous vessels and perivillous fibrin deposition...
January 25, 2018: Biotechnic & Histochemistry: Official Publication of the Biological Stain Commission
https://www.readbyqxmd.com/read/29317337/heart-and-bile-acids-clinical-consequences-of-altered-bile-acid-metabolism
#13
REVIEW
Tharni Vasavan, Elisa Ferraro, Effendi Ibrahim, Peter Dixon, Julia Gorelik, Catherine Williamson
Cardiac dysfunction has an increased prevalence in diseases complicated by liver cirrhosis such as primary biliary cholangitis and primary sclerosing cholangitis. This observation has led to research into the association between abnormalities in bile acid metabolism and cardiac pathology. Approximately 50% of liver cirrhosis cases develop cirrhotic cardiomyopathy. Bile acids are directly implicated in this, causing QT interval prolongation, cardiac hypertrophy, cardiomyocyte apoptosis and abnormal haemodynamics of the heart...
January 6, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29316097/splicing-analysis-of-rare-novel-synonymous-or-intronic-variants-identified-in-abcb11-heterozygotes-presenting-as-progressive-intrahepatic-cholestasis-with-low-%C3%AE-glutamyltransferase
#14
Neng-Li Wang, Yi-Ling Qiu, Wen-Cai Guan, Gang Li, Yi Lu, Mei-Hong Zhang, Wei-Sha Luan, Jian-She Wang
AIM: To analyze the pathogenicity of rare/novel synonymous or intronic variants identified in ABCB11 heterozygotes presenting as progressive intrahepatic cholestasis with low γ-glutamyltransferase (GGT). METHODS: The enrolled variants were identified in ABCB11 between October 2009 and June 2016. The effects on pre-RNA splicing were analyzed by in silico tools and minigene splicing assay. RESULTS: There were 3 intronic (c.908+5G>A, c.2815-8A>G, and c...
January 9, 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29309877/development-of-a-mechanistic-biokinetic-model-for-hepatic-bile-acid-handling-to-predict-possible-cholestatic-effects-of-drugs
#15
Sylvia Notenboom, Karl M Weigand, Johannes H Proost, Marola M van Lipzig, Evita van de Steeg, Petra H H van den Broek, Rick Greupink, Frans G M Russel, Geny M M Groothuis
Drug-induced liver injury (DILI) is a common reason for drug withdrawal from the market. An important cause of DILI is drug-induced cholestasis. One of the major players involved in drug-induced cholestasis is the bile salt efflux pump (BSEP; ABCB11). Inhibition of BSEP by drugs potentially leads to cholestasis due to increased (toxic) intrahepatic concentrations of bile acids with subsequent cell injury. In order to investigate the possibilities for in silico prediction of cholestatic effects of drugs, we developed a mechanistic biokinetic model for human liver bile acid handling populated with human in vitro data...
January 5, 2018: European Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/29304564/unexplained-cholestasis-in-adults-and-adolescents-diagnostic-benefit-of-genetic-examination
#16
Luise Aamann, Nikolaj Ørntoft, Ida Vogel, Henning Grønbaek, Naja Becher, Hendrik Vilstrup, Peter Ott, Dorte Launholt Lildballe
OBJECTIVES: A few adult and adolescent patients with even severe cholestatic liver disease remain unexplained after standard diagnostic work-up. We studied the value of genetic examination in such patients and developed a panel of eight genes with known cholestatic associations. MATERIALS AND METHODS: Thirty-three patients with unexplained cholestasis despite a thorough clinical work-up were examined for sequence variations in the coding regions of the ABCB4, ABCB11, ABCC2, ABCG5, ATP8B1, JAG1, NOTCH2, and UGT1A1 genes and the promoter region of UGT1A1 by massive parallel sequencing of DNA extracted from whole blood...
January 5, 2018: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/29302103/babesia-odocoilei-as-a-cause-of-mortality-in-captive-cervids-in-canada
#17
Amélie Mathieu, Adriana R Pastor, Charlene N Berkvens, Carolyn Gara-Boivin, Michel Hébert, Alexandre N Léveillé, John R Barta, Dale A Smith
Nine cases of fatal infection with Babesia odocoilei were confirmed in reindeer (Rangifer tarandus tarandus) and elk (Cervus canadensis) housed in zoological institutions located in southern Quebec, Ontario, and Manitoba, Canada between 2013 and 2016. All animals died of a hemolytic crisis. Frequent postmortem findings were extensive hemorrhage, pigmenturia, and intrahepatic cholestasis. The described ante- and postmortem signs are consistent with those of previously reported cases in the United States. Diagnosis was confirmed in all cases by polymerase chain reaction performed on DNA extracted from whole blood or frozen spleen...
January 2018: Canadian Veterinary Journal. la Revue Vétérinaire Canadienne
https://www.readbyqxmd.com/read/29250459/intrahepatic-cholestasis-of-pregnancy-refractory-to-multiple-medical-therapies-and-plasmapheresis
#18
Adam John Covach, William Nicholas Rose
Objectives  We report on a patient suffering from intractable itching secondary to intrahepatic cholestasis of pregnancy (ICP) unresponsive to conventional medical therapies. She was started on a regimen of therapeutic plasma exchange (TPE), which is often efficacious in relieving patient's itching from all causes of cholestasis, including ICP. Methods  We performed a retrospective review of a patient's medical record. Results  Following initial TPE, the patient reported dramatic relief of her itching and consequent insomnia...
October 2017: American Journal of Perinatology Reports
https://www.readbyqxmd.com/read/29240010/well-preserved-renal-function-in-children-with-untreated-chronic-liver-disease
#19
Ulla B Berg, Antal Németh
OBJECTIVES: Based on studies with hepatorenal syndrome, it is widely regarded that renal function is impacted in chronic liver disease. Therefore we investigated renal function in our children with chronic liver disease. METHODS: In a retrospective study of 277 children with chronic liver disease, renal function was investigated as glomerular filtration rate (GFR) and effective renal plasma flow (ERPF), measured as clearance of inulin and para-amino hippuric acid or clearance of iohexol...
December 12, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29238877/cryptogenic-cholestasis-in-young-and-adults-atp8b1-abcb11-abcb4-and-tjp2-gene-variants-analysis-by-high-throughput-sequencing
#20
Giovanni Vitale, Stefano Gitto, Francesco Raimondi, Alessandro Mattiaccio, Vilma Mantovani, Ranka Vukotic, Antonietta D'Errico, Marco Seri, Robert B Russell, Pietro Andreone
BACKGROUND: Mutations in ATP-transporters ATPB81, ABCB11, and ABCB4 are responsible for progressive familial intrahepatic cholestasis (PFIC) 1, 2 and 3, and recently the gene for tight junction protein-2 (TJP2) has been linked to PFIC4. AIM: As these four genes have been poorly studied in young people and adults, we investigated them in this context here. METHODS: In patients with cryptogenic cholestasis, we analyzed the presence of mutations by high-throughput sequencing...
December 13, 2017: Journal of Gastroenterology
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