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Cholestatic liver diseases

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https://www.readbyqxmd.com/read/29343773/mitochondrial-mutations-in-cholestatic-liver-disease-with-biliary-atresia
#1
Hong Koh, Gun-Seok Park, Sun-Mi Shin, Chang Eon Park, Seung Kim, Seok Joo Han, Huy Quang Pham, Jae-Ho Shin, Dong-Woo Lee
Biliary atresia (BA) results in severe bile blockage and is caused by the absence of extrahepatic ducts. Even after successful hepatic portoenterostomy, a considerable number of patients are likely to show progressive deterioration in liver function. Recent studies show that mutations in protein-coding mitochondrial DNA (mtDNA) genes and/or mitochondrial genes in nuclear DNA (nDNA) are associated with hepatocellular dysfunction. This observation led us to investigate whether hepatic dysfunctions in BA is genetically associated with mtDNA mutations...
January 17, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29331005/pregnant-lady-with-undiagnosed-hodgkin-s-disease-presenting-as-secondary-sclerosing-cholangitis
#2
Taif Khattak, Syed Hashim Ali Shah, Waqas Khan, Syed Shahmeer Raza, Uzair Ahmad, Farhan Ullah
Hodgkin's lymphoma. A 25 years old lady, 34 weeks primigravida was referred from the Emergency Department to the Medical Unit Khyber Teaching Hospital-MTI, Peshawar with four weeks of fever, progressive jaundice, pruritus, night sweats and weight loss. LFTs showed cholestatic picture, ERCP showed scanty intrahepatic giving beading and autumn tree appearance typical of sclerosing cholangitis. CBD was normal. Doppler U/S of hepatic and portal vein reported normal. She was started on steroids, ursodeoxycholic acid and antibiotics 3rd generation cephalosporins to which she did not respond well...
October 2017: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/29328070/nerve-growth-factor-upregulates-sirtuin-1-expression-in-cholestasis-a-potential-therapeutic-target
#3
Ming-Shian Tsai, Po-Huang Lee, Cheuk-Kwan Sun, Ting-Chia Chiu, Yu-Chun Lin, I-Wei Chang, Po-Han Chen, Ying-Hsien Kao
This study investigated the regulatory role of nerve growth factor (NGF) in sirtuin 1 (SIRT1) expression in cholestatic livers. We evaluated the expression of NGF and its cognate receptors in human livers with hepatolithiasis and the effects of NGF therapy on liver injury and hepatic SIRT1 expression in a bile duct ligation (BDL) mouse model. Histopathological and molecular analyses showed that the hepatocytes of human diseased livers expressed NGF, proNGF (a precursor of NGF), TrkA and p75NTR, whereas only p75NTR was upregulated in hepatolithiasis, compared with non-hepatolithiasis livers...
January 12, 2018: Experimental & Molecular Medicine
https://www.readbyqxmd.com/read/29325602/bile-acid-metabolism-in-liver-pathobiology
#4
John Y L Chiang, Jessica M Ferrell
Bile acids facilitate intestinal nutrient absorption and biliary cholesterol secretion to maintain bile acid homeostasis, which is essential for protecting liver and other tissues and cells from cholesterol and bile acid toxicity. Bile acid metabolism is tightly regulated by bile acid synthesis in the liver and bile acid biotransformation in the intestine. Bile acids are endogenous ligands that activate a complex network of nuclear receptor farnesoid X receptor and membrane G protein-coupled bile acid receptor-1 to regulate hepatic lipid and glucose metabolic homeostasis and energy metabolism...
January 11, 2018: Gene Expression
https://www.readbyqxmd.com/read/29325287/-the-diagnostic-value-and-limits-of-diagnostic-parameters-for-wilson-s-disease
#5
X Yang
Wilson disease (WD) is a rare and treatable genetic disorder. This paper describes the new advances and author's long-term experiences in the diagnosis of WD. The characteristics in clinical and routine tests are: the age of presentation can be quite broad, the WD could not be excluded based on age only; the patients usually have mild digestive symptoms but obvious chronic liver disease signs; liver function tests may reveal normal or a mild elevation in bilirubin, ALT and AST, but quite abnormal in serum albumin and prothrombin time in most patients; Coombs-negative hemolytic anemia, normal or markedly subnormal serum alkaline phosphatase (typically < 40 IU/L) are useful for the diagnosis of fulminant WD...
December 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29325285/-primary-biliary-cholangitis-and-bile-acid-metabolism
#6
L Wang, Y C Han, Y Han
Primary biliary cholangitis (PBC) is an immune-mediated cholestatic liver disease of unknown pathogenesis. The research on immunologic injury in the past helps us to understand more about this disease, but there are still many problems and challenges in the research on PBC. With a focus on the cholestatic features of PBC, this article reviews the research advances in bile acid metabolism in the field of PBC, in order to provide new thoughts for future research.
November 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29325274/-an-interpretation-of-2017-easl-clinical-practice-guidelines-the-diagnosis-and-management-of-patients-with-primary-biliary-cholangitis
#7
Y Han, Y Chen
Primary biliary cholangitis (PBC) is a chronic autoimmune cholestatic disease and may progress to liver fibrosis, liver cirrhosis, decompensated cirrhosis, and even end-stage liver disease without effective treatment. The diagnosis of PBC is mainly based on the biochemical parameters indicating cholestatic hepatitis and the presence of specific autoantibody in circulation. The goals of the treatment and management of PBC are to prevent the development of end-stage liver disease, to improve related clinical symptoms, and to improve patients' quality of life...
November 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29325271/-research-advances-in-primary-biliary-cholangitis
#8
W J Duan, J D Jia
Primary biliary cholangitis (PBC) is a chronic cholestatic disease with unknown pathogenesis. Positive anti-mitochondrial antibody has high sensitivity and specificity in the diagnosis of this disease. Ursodeoxycholic acid is mainly used for the treatment of PBC, but 40% of patients have an unsatisfactory biochemical response to this drug. 6-Ethylchenodeoxycholic acid is a new drug approved for the treatment of PBC, and liver transplantation remains the only effective method for the treatment of patients with end-stage PBC...
November 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29316113/drug-induced-liver-injury-is-frequently-associated-with-severe-cutaneous-adverse-drug-reactions-experience-from-two-australian-tertiary-hospitals
#9
Wendy C Fang, Nikki R Adler, Linda V Graudins, Caitlin Goldblatt, Michelle Sy Goh, Stuart K Roberts, Jason A Trubiano, Ar Kar Aung
BACKGROUND: Drug-induced liver injury can be associated with certain cutaneous adverse drug reactions. AIMS: We aim to demonstrate the prevalence of drug-induced liver injury in patients with cutaneous adverse drug reactions. Severity and patterns of liver injury, risk factors, causal medications and outcomes are also examined. METHODS: A retrospective cohort study of patients with cutaneous adverse drug reactions was conducted across two hospitals in Australia...
January 8, 2018: Internal Medicine Journal
https://www.readbyqxmd.com/read/29316057/-a-promising-medium-term-follow-up-of-pediatric-sclerosing-cholangitis-mild-phenotype-or-early-diagnosis
#10
F Ferrari, G Ranucci, M Aloi, L Della Volpe, F Viola, E Miele, S Cucchiara, R Iorio
BACKGROUND AND AIMS: Sclerosing cholangitis (SC) is a chronic cholestatic liver disease whose diagnosis is being increasingly recognized in childhood. Long-term course and prognosis of pediatric SC is poorly described. METHODS: We reviewed data of pediatric SC patients, followed in two referral centers during a period of up to 20 years. We aimed to evaluate long-term outcomes according to SC phenotype. RESULTS: Among 45 patients (median age: 10...
January 9, 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29315013/on-the-evolution-of-bile-salts-and-the-farnesoid-x-receptor-in-vertebrates
#11
Kim Frisch, Aage Kristian Olsen Alstrup
In recent decades, our knowledge of bile salts has undergone a vast development, and bile salts are now known not only for their detergent properties that aid in the absorption of dietary lipids but also for their interaction with specific nuclear and membrane receptors. In particular, it has been realized that the response of the farnesoid X receptor (FXR) to bile acids provides a signal bridge between the liver and small intestine, controlling the intracellular levels, biosynthesis, and enterohepatic circulation of bile acids...
March 2018: Physiological and Biochemical Zoology: PBZ
https://www.readbyqxmd.com/read/29310470/new-therapies-target-the-toxic-consequences-of-cholestatic-liver-disease
#12
Peter L M Jansen
Introduction In most cholestatic liver diseases the primary cholestasis-causing lesions are located in the biliary tree and may be of (auto)immune origin. Bile salts are responsible for the secondary toxic consequences. Bile salt and nuclear hormone directed therapies primarily aim at improving this secondary toxic injury. In primary biliary cholangitis, trials show statistically significant responses on biochemical endpoints. Preclinical studies suggest that FXR- and PPAR-agonists, inhibitors of the apical sodium-dependent bile salt transporter (ASBT-inhibitors) and the C23 UDCA derivative nor-UDCA are promising agents for the treatment of primary sclerosing cholangitis (PSC)...
January 9, 2018: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29304564/unexplained-cholestasis-in-adults-and-adolescents-diagnostic-benefit-of-genetic-examination
#13
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/29260695/-jaundice-and-liver-injury-with-cholestatic-pattern-after-treatment-with-metoprololsuccinat
#14
Tommy Hansen, Lotte Fynne
Drug-induced liver injury is a well-known adverse event to numerous medications with clinical presentations from asymptomatic liver enzyme elevation to liver failure. However, liver injury after administration with metoprolol is not common, and only few case reports have been published. This is a case report of an 80-year-old woman with liver injury with cholestatic pattern and jaundice after two months of treatment with Metoprololsuccinat. With no evidence of other disease, liver function normalized in the following months after discontinuation of Metoprololsuccinat...
December 18, 2017: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/29250120/human-chorionic-plate-derived-mesenchymal-stem-cells-restore-hepatic-lipid-metabolism-in-a-rat-model-of-bile-duct-ligation
#15
Yun Bin Lee, Jong Ho Choi, Eun Nam Kim, Jin Seok, Hyun-Jung Lee, Jung-Hwan Yoon, Gi Jin Kim
In cholestatic liver diseases, impaired bile excretion disrupts lipid homeostasis. We investigated changes of lipid metabolism, including mitochondrial β-oxidation, in a rat model of bile duct ligation (BDL) in which chorionic plate-derived mesenchymal stem cells (CP-MSCs) were transplanted. Serum cholesterol level, which was elevated after BDL, was significantly decreased following CP-MSC transplantation. The expression levels of genes involved in intracellular lipid uptake, including long-chain fatty acyl-CoA synthetases and fatty acid transport proteins, were decreased in rats after BDL; however, they were not significantly changed by subsequent CP-MSC transplantation...
2017: Stem Cells International
https://www.readbyqxmd.com/read/29247427/clinical-spectrum-and-outcome-of-pediatric-drug-induced-liver-injury
#16
Aditi Kumar, Vikrant Sood, Rajeev Khanna, Sanjeev Kumar Verma, Nikhil Mehra, Dinesh Rawat, Seema Alam
Limited literature is available in pediatric population regarding drug-induced liver injury (DILI) making it a diagnostic challenge. This study was thus planned to determine the clinical spectrum and the outcome of DILI in children. All patients with DILI under 18 y of age were retrospectively reviewed and details regarding clinical presentation, Roussel Uclaf Causality Assessment Method (RUCAM) scale, drugs implicated, biochemical abnormalities and outcome were noted. DILI constituted 3.7% of all children with liver disease...
December 16, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/29245301/copper-associated-hepatitis-in-a-patient-with-chronic-myeloid-leukemia-following-hematopoietic-stem-cell-transplantation-a-case-report
#17
Ching-Fen Lee, Chi-Hua Chen, Yu-Chuan Wen, Tsung-Yen Chang, Ming-Wei Lai, Tang-Her Jaing
RATIONALE: We report a complicated case of cholestatic hepatitis with suspected autoimmune hemolytic anemia (AIHA) and copper toxicity syndrome after HSCT and donor lymphocyte infusion (DLI). PATIENT CONCERNS: A 19-year-1-month-old girl presented with a history of CML. She underwent matched unrelated donor HSCT and donor lymphocyte infusion subsequently. Three months later, yellowish discoloration of the skin was found, which was accompanied by progressive itchy skin, easy fatigability, insomnia, and dark urine output...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29238877/cryptogenic-cholestasis-in-young-and-adults-atp8b1-abcb11-abcb4-and-tjp2-gene-variants-analysis-by-high-throughput-sequencing
#18
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
https://www.readbyqxmd.com/read/29217403/transcriptome-analysis-reveals-a-positive-role-for-nerve-growth-factor-in-retinol-metabolism-in-primary-rat-hepatocytes
#19
Ying-Hsien Kao, Po-Huang Lee, Ting-Chia Chiu, Yu-Chun Lin, Cheuk-Kwan Sun, Po-Han Chen, Ming-Shian Tsai
Up-regulation of nerve growth factor (NGF) in parenchymal hepatocytes with cholestatic injury has been previously demonstrated to exert hepatoprotective effects in an autocrine manner; however, the overall impact of NGF up-regulation remains elusive. This study aimed to profile the effects of exogenous NGF on cultured primary rat hepatocytes using transcriptome analysis. Total RNA was isolated from hepatocytes with and without 24 h of NGF exposure, and subjected to RNA enrichment by PCR and RNA sequencing procedures...
December 4, 2017: Cytokine
https://www.readbyqxmd.com/read/29196240/the-pathophysiology-of-human-obstructive-cholestasis-is-mimicked-in-cholestatic-gold-syrian-hamsters
#20
Rowan F van Golen, Pim B Olthof, Lianne R de Haan, Robert-Jan Coelen, Alexandros Pechlivanis, Mark J de Keijzer, Ruud Weijer, Dirk R de Waart, André B P van Kuilenburg, Jeroen Roelofsen, Pim W Gilijamse, Martinus A Maas, Matthew R Lewis, Jeremy K Nicholson, Joanne Verheij, Michal Heger
Obstructive cholestasis causes liver injury via accumulation of toxic bile acids (BAs). Therapeutic options for cholestatic liver disease are limited, partially because the available murine disease models lack translational value. Profiling of time-related changes following bile duct ligation (BDL) in Gold Syrian hamsters revealed a biochemical response similar to cholestatic patients in terms of BA pool composition, alterations in hepatocyte BA transport and signaling, and suppression of BA production. Hamsters tolerated cholestasis well for up to 28days and progressed relatively slowly to fibrotic liver injury...
November 28, 2017: Biochimica et Biophysica Acta
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