keyword
MENU ▼
Read by QxMD icon Read
search

Cholestatic liver diseases

keyword
https://www.readbyqxmd.com/read/28077274/the-role-and-regulation-of-the-peroxisome-proliferator-activated-receptor-alpha-in-human-liver
#1
REVIEW
Sander Kersten, Rinke Stienstra
The peroxisome proliferator-activated receptor α (PPARα) is a ligand-activated transcription factor that is abundantly expressed in liver. PPARα is activated by fatty acids and various other lipid species, as well as by a class of chemicals referred to as peroxisome proliferators. Studies in mice have shown that PPARα serves as the master regulator of hepatic lipid metabolism during fasting. In addition, PPARα suppresses inflammation and the acute phase response. Comparatively little is known about PPARα in human liver...
January 7, 2017: Biochimie
https://www.readbyqxmd.com/read/28065777/protective-effects-of-petroleum-ether-extracts-of-herpetospermum-caudigerum-against-%C3%AE-naphthylisothiocyanate-induced-acute-cholestasis-of-rats
#2
Wen-Rui Cao, Jing-Qiu Ge, Xin Xie, Meng-Lin Fan, Xu-Dong Fan, Hong Wang, Zhao-Yue Dong, Zhi-Hua Liao, Xiao-Zhong Lan, Min Chen
ETHNOPHARMACOLOGICAL RELEVANCE: The ripe seeds of Herpetospermum caudigerum have been used in Tibetan folk medicine for treatment of bile or liver diseases including jaundice, hepatitis, intumescences or inflammation. Previously reports suggested that the seed oil and some lignans from H. caudigerum exhibited protective effects against carbon tetrachloride (CCl4)-induced hepatic damage in rats, which may be related to their free radical scavenging effect. However, the protective effect of H...
January 5, 2017: Journal of Ethnopharmacology
https://www.readbyqxmd.com/read/28064265/a-novel-truncation-mutation-in-atp8b1-gene-in-progressive-familial-intrahepatic-cholestasis
#3
Anjali Sharma, Ujjal Poddar, Shikha Agnihotry, Rakesh Aggarwal
BACKGROUND: Progressive familial intrahepatic cholestasis has been only infrequently reported from India. CASE CHARACTERISTICS: An Indian girl with progressive cholestatic liver disease beginning during infancy, normal gamma-glutamyl transpeptidase levels, parental consanguinity, positive family history and a fatal outcome. OBSERVATION: A novel, homozygous mutation (c.[589_592inv;592_593insA]) in ATP8B1 gene, with a markedly truncated protein (p...
December 15, 2016: Indian Pediatrics
https://www.readbyqxmd.com/read/28055006/biliary-tract-instillation-of-a-smac-mimetic-induces-trail-dependent-acute-sclerosing-cholangitis-like-injury-in-mice
#4
Maria Eugenia Guicciardi, Anuradha Krishnan, Steven F Bronk, Petra Hirsova, Thomas S Griffith, Gregory J Gores
Primary sclerosing cholangitis (PSC) is a cholestatic liver disease of unknown etiopathogenesis characterized by fibrous cholangiopathy of large and small bile ducts. Systemic administration of a murine TNF-related apoptosis-inducing ligand (TRAIL) receptor agonist induces a sclerosing cholangitis injury in C57BL/6 mice, suggesting endogenous TRAIL may contribute to sclerosing cholangitis syndromes. Cellular inhibitor of apoptosis proteins (cIAP-1 and cIAP-2) are negative regulators of inflammation and TRAIL receptor signaling...
January 5, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28052628/management-of-cholestatic-disease-in-2017
#5
REVIEW
Elsemieke de Vries, Ulrich Beuers
Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the most frequent chronic cholestatic liver diseases and serve as model diseases to discuss the management of cholestasis in 2017 in the lecture that is summarized in this report. PBC and PSC are characterized by inflammation and fibrosis of small intrahepatic (PBC) or larger intra- and/or extrahepatic (PSC) bile ducts. Bile duct damage leads to cholestasis and can progress to liver fibrosis and even cirrhosis. Various genetic, environmental and endogenous factors may contribute to the development of chronic cholestatic liver diseases, but the exact pathogenesis of PBC and PSC has not been clarified...
January 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28051047/evaluation-of-the-use-of-laparoscopic-guided-cholecystocholangiography-and-liver-biopsy-in-definitive-diagnosis-of-neonatal-cholestatic-jaundice
#6
Khalid Shreef, Abdullah Alhelal
BACKGROUND: Once it is established that a jaundiced infant has direct hyperbilirubinemia, the principal diagnostic concern is to differentiate hepatocellular from obstructive cholestasis. Traditional tests such as ultrasonography, percutaneous liver biopsy and technetium 99 m hepatobiliary iminodiacetic acid (HIDA) scan are often not sufficiently discriminating. Definitive exclusion of biliary atresia (BA) in the infant with cholestatic jaundice usually requires mini-laparotomy and intra-operative cholangiography...
October 2016: African Journal of Paediatric Surgery: AJPS
https://www.readbyqxmd.com/read/28049946/ursodeoxycholic-acid-ameliorates-intrahepatic-cholestasis-independent-of-biliary-bicarbonate-secretion-in-vil2-kd-kd-mice
#7
Ryo Hatano, Kotoku Kawaguchi, Fumitaka Togashi, Masato Sugata, Shizuka Masuda, Shinji Asano
Ursodeoxycholic acid (UDCA) is a hydrophilic bile acid that possesses many pharmacological effects, including increasing bile flow, changing the hydrophobicity of the bile acid pool, and modulation of the immune response. UDCA has been approved for treating cholestatic liver disease, such as primary biliary cholangitis. However, several unanticipated severe side effects of UDCA are observed in cholestatic patients, and its pharmacological benefits remain controversial. We reported that ezrin-knockdown (Vil2(kd/kd)) mice exhibited severe hepatic injury because of a functional disorder in bile duct fluidity and alkalinity regulation, resembling human intrahepatic cholestatic disease...
2017: Biological & Pharmaceutical Bulletin
https://www.readbyqxmd.com/read/28049044/lymphocytes-contribute-to-biliary-injury-and-fibrosis-in-experimental-xenobiotic-induced-cholestasis
#8
Nikita Joshi, Anna K Kopec, Holly Cline-Fedewa, James P Luyendyk
The etiology of chronic bile duct injury and fibrosis in patients with autoimmune cholestatic liver diseases is complex, and likely involves immune cells such as lymphocytes. However, most models of biliary fibrosis are not autoimmune in nature. Biliary fibrosis can be induced experimentally by prolonged exposure of mice to the bile duct toxicant alpha-naphthylisothiocyanate (ANIT). We determined whether lymphocytes contributed to ANIT-mediated biliary hyperplasia and fibrosis in mice. Hepatic accumulation of T-lymphocytes and increased serum levels of anti-nuclear-autoantibodies were evident in wild-type mice exposed to ANIT (0...
December 31, 2016: Toxicology
https://www.readbyqxmd.com/read/28045770/clinical-variability-following-partial-external-biliary-diversion-in-familial-intrahepatic-cholestasis-1-deficiency
#9
James E Squires, Neslihan Celik, Amy Morris, Kyle Soltys, George Mazariegos, Benjamin Shneider, Robert H Squires
OBJECTIVES: Familial intrahepatic cholestasis 1 (FIC1) deficiency is caused by a mutation in the ATP8B1 gene. Partial external biliary diversion (PEBD) is pursued to improve pruritus and arrest disease progression. Our aim is to describe clinical variability after PEBD in FIC1 disease. METHODS: We performed a single-center, retrospective review of genetically confirmed FIC1 deficient patients who received PEBD. Clinical outcomes after PEBD were cholestasis, pruritus, fat-soluble vitamin supplementation, growth, and markers of disease progression that included splenomegaly and aspartate aminotransferase-to-platelet ratio index...
December 30, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28039895/genetic-profiling-of-children-with-advanced-cholestatic-liver-disease
#10
Mohammad Shagrani, Jessica Burkholder, Dieter Broering, Mohamed Abouelhoda, Tariq Faquih, Mohamed El-Kalioby, Shazia N Subhani, Ewa Goljan, Renad Albar, Dorota Monies, Nejat Mazhar, Basma S AlAbdulaziz, Khalid Al Abdelrahman, Nada Altassan, Fowzan S Alkuraya
Advanced cholestatic liver disease is a leading referral to pediatric liver transplant centers. Recent advances in the genetic classification of this group of disorders promise a highly personalized management although the genetic heterogeneity also poses a diagnostic challenge. Using a next-generation sequencing-based multi-gene panel, we performed retrospective analysis of 98 pediatric patients who presented with advanced cholestatic liver disease. A likely causal mutation was identified in the majority (61%), spanning many genes including ones that have only rarely been reported to cause cholestatic liver disease e...
December 30, 2016: Clinical Genetics
https://www.readbyqxmd.com/read/28012215/real-world-experience-with-daclatasvir-plus-sofosbuvir-%C3%A2-ribavirin-for-post-liver-transplant-hcv-recurrence-and-severe-liver-disease
#11
Kerstin Herzer, Tania M Welzel, Ulrich Spengler, Holger Hinrichsen, Hartwig Klinker, Thomas Berg, Peter Ferenci, Markus Peck-Radosavljevic, Akin Inderson, Yue Zhao, Maria Jesus Jimenez-Exposito, Stefan Zeuzem
Optimizing therapy of post-transplant HCV recurrence remains important, especially in advanced liver disease. We evaluated daclatasvir (DCV) plus sofosbuvir (SOF), with or without ribavirin (RBV), in patients with post-liver transplant recurrence in a real-world European cohort at high risk of decompensation or death within 12 months. Recommended treatment was DCV 60mg plus SOF 400mg once-daily for 24 weeks; RBV use/shorter treatment duration was at physicians' discretion. Patients (N=87) were 70% male, 93% white, and mostly infected with HCV genotypes 1b (48%), 1a (32%), or 3 (9%); 37 (43%) had cirrhosis (16 decompensated), five had fibrosing cholestatic hepatitis...
December 24, 2016: Transplant International: Official Journal of the European Society for Organ Transplantation
https://www.readbyqxmd.com/read/28008402/dapagliflozin-induced-acute-on-chronic-liver-injury
#12
Joshua A Levine, Amy Ann Lo, Amisha Wallia, Melinda Rogers, Lisa B VanWagner
Sodium-glucose cotransporter 2 inhibitors are a new class of oral hypoglycemic agents, and thus safety data are limited. We present a 48-year-old woman with type 2 diabetes mellitus and Child's Class A cirrhosis secondary to nonalcoholic steatohepatitis presenting with jaundice and acute cholestatic liver injury. Other than starting dapagliflozin, she reported no medication changes or supplement use. Before treatment, her total bilirubin was 1.2 mg/dL. On admission, her liver values were elevated and liver biopsy was consistent with drug-induced liver injury...
August 2016: ACG Case Reports Journal
https://www.readbyqxmd.com/read/28003721/primary-biliary-cholangitis-disease-pathogenesis-and-implications-for-established-and-novel-therapeutics
#13
REVIEW
Amitkumar Patel, Anil Seetharam
Primary Biliary Cholangitis is a progressive, autoimmune cholestatic liver disorder. Cholestasis with disease progression may lead to dyslipidemia, osteodystrophy and fat-soluble vitamin deficiency. Portal hypertension may develop prior to advanced stages of fibrosis. Untreated disease may lead to cirrhosis, hepatocellular cancer and need for orthotopic liver transplantation. Classically, diagnosis is made with elevation of alkaline phosphatase, demonstration of circulating antimitochondrial antibody, and if performed: asymmetric destruction/nonsupperative cholangitis of intralobular bile ducts on biopsy...
December 2016: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/27997980/new-therapeutic-concepts-in-bile-acid-transport-and-signaling-for-management-of-cholestasis
#14
REVIEW
Michael Trauner, Claudia Daniela Fuchs, Emina Halilbasic, Gustav Paumgartner
The identification of the key regulators of bile acid synthesis and transport within the enterohepatic circulation has unravelled potential targets for pharmacological therapies of cholestatic liver diseases. Novel drug targets include the bile acid receptors FXR and TGR5, the bile acid-induced gut hormones FGF19 and GLP-1, and the bile acid transport systems ASBT and NTCP within the enterohepatic circulation. Moreover, bile acid derivatives undergoing cholehepatic shunting may allow improved targeting to the bile ducts...
December 20, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/27997975/primary-biliary-cirrhosis-and-the-microbiome
#15
Eamonn M M Quigley
Primary biliary cirrhosis is a rather uncommon, slowly progressive, cholestatic liver disease that predominantly affects middle-aged women. Apart from the changes in the gut microbiome that have been described in liver disease in general, little is known of the composition of the microbiome in primary biliary cirrhosis. Nevertheless, epidemiological, clinical, and some experimental evidence points to the possible role of a bacterium (or bacteria) in the initiation of the autoimmune process that leads to the development of this unique clinical phenotype...
September 2016: Seminars in Liver Disease
https://www.readbyqxmd.com/read/27997974/the-microbiome-and-primary-sclerosing-cholangitis
#16
Ahmad H Ali, Elizabeth J Carey, Keith D Lindor
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with detrimental sequela. In many patients, PSC progresses to end-stage liver disease and hepatobiliary cancer. There is no medical therapy that is proven to halt or reverse the progression of PSC. Approximately 70 to 80% of PSC patients have inflammatory bowel disease, usually ulcerative colitis. The etiology of PSC is poorly understood. Several lines of evidence suggest that the intestinal microbiota plays an important role in the etiopathogenesis of PSC...
September 2016: Seminars in Liver Disease
https://www.readbyqxmd.com/read/27996360/liver-transplantation-for-hepatocellular-carcinoma-in-young-adults-a-united-network-for-organ-sharing-study
#17
Stefanie M Thomas, Diana Moke, Rocio Lopez, Rabi Hanna, Mohammad Nasser Kabbany, Naim Alkhouri
PURPOSE: Orthotopic liver transplantation (OLT) is curative for hepatocellular carcinoma (HCC). HCC is typically a disease of older adults (OAs); therefore, characteristics and outcomes of OLT for young adults (YAs) (ages 18-40) are not described. The objective of this study was to assess the characteristics and outcomes of YAs with HCC receiving OLT and compare these to OAs (ages >40 years). METHODS: YAs with HCC who had OLT from the United Network for Organ Sharing (UNOS) database were included in this study...
December 20, 2016: Journal of Adolescent and Young Adult Oncology
https://www.readbyqxmd.com/read/27995906/acg-clinical-guideline-evaluation-of-abnormal-liver-chemistries
#18
Paul Y Kwo, Stanley M Cohen, Joseph K Lim
Clinicians are required to assess abnormal liver chemistries on a daily basis. The most common liver chemistries ordered are serum alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase and bilirubin. These tests should be termed liver chemistries or liver tests. Hepatocellular injury is defined as disproportionate elevation of AST and ALT levels compared with alkaline phosphatase levels. Cholestatic injury is defined as disproportionate elevation of alkaline phosphatase level as compared with AST and ALT levels...
January 2017: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/27989704/the-tooth-as-a-monitor-of-cholestatic-liver-disease-in-rats
#19
Victor Edson Rogerio, Luciana Bruzadin, Ricardo Katsuya Toma, Marcos Augusto Bizeto, Leda Mugayar, Ivan Hong Jun Koh
OBJECTIVE: Measure, noninvasively, the deposition of bilirubin in the tooth by using DIAGNOdent and correlate it to liver dysfunction. STUDY DESIGN: After confirming the capacity of DIAGNOdent to measure varying bile concentrations in plaster blocks, a cholestatic liver disease model was studied to detect increasing bilirubin impregnation in the teeth of rats. Wistar-EPM rats (n = 50) were divided into three groups: (1) BDL: rats submitted to bile duct ligation (BDL); (2) Naïve: rats without procedure; and (3) Sham: rats submitted to laparotomy without BDL (n = 10/period/group)...
October 12, 2016: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
https://www.readbyqxmd.com/read/27981592/the-ascending-pathophysiology-of-cholestatic-liver-disease
#20
REVIEW
Peter L M Jansen, Ahmed Ghallab, Nachiket Vartak, Raymond Reif, Frank G Schaap, Jochen Hampe, Jan G Hengstler
In this review we develop the argument that cholestatic liver diseases, particularly PBC and PSC, evolve over time with anatomically an ascending course of the disease process. The first and early lesions are in 'downstream' bile ducts. This eventually leads to cholestasis and this causes bile salt-mediated toxic injury of the 'upstream' liver parenchyma. Bile salts are toxic in high concentration. These concentrations are present in the canalicular network, bile ducts and gallbladder. Leakage of bile from this network and ducts could be an important driver of toxicity...
December 16, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
keyword
keyword
39626
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"