Results 21 to 30 of about 139,121 (234)

Odevixibat treatment in progressive familial intrahepatic cholestasis: a randomised, placebo-controlled, phase 3 trial.

The Lancet Gastroenterology and Hepatology, 2022
BACKGROUND Progressive familial intrahepatic cholestasis (PFIC) is a group of inherited paediatric liver diseases resulting from mutations in genes that impact bile secretion. We aimed to evaluate the effects of odevixibat, an ileal bile acid transporter
Richard J. Thompson, H. Arnell, R. Artan, U. Baumann, P. Calvo, P. Czubkowski, B. Dalgıç, L. D’Antiga, Özlem Durmaz, B. Fischler, E. Gonzales, T. Grammatikopoulos, G. Gupte, W. Hardikar, R. Houwen, B. Kamath, S. Karpen, L. Kjems, F. Lacaille, A. Lachaux, E. Lainka, C. Mack, J. Mattsson, P. McKiernan, H. Özen, Sanjay Rajwal, B. Roquelaure, M. Shagrani, E. Shteyer, Nisreen Soufi, E. Sturm, M. Tessier, H. Verkade, Patrick Horn   +33 more
semanticscholar   +1 more source

GWAS meta-analysis of intrahepatic cholestasis of pregnancy implicates multiple hepatic genes and regulatory elements

Nature Communications, 2022
Intrahepatic cholestasis of pregnancy (ICP) is a pregnancy-specific liver disorder affecting 0.5–2% of pregnancies. The majority of cases present in the third trimester with pruritus, elevated serum bile acids and abnormal serum liver tests.
P. Dixon, A. Levine, Inês Cebola, M. Chan, Aliya Amin, Anshul Aich, M. Mozere, H. Maude, Alice L. Mitchell, Jun Zhang, J. Chambers, A. Syngelaki, J. Donnelly, S. Cooley, M. Geary, Kypros K. Nicolaides, M. Thorsell, W. Hague, M. Estiù, H. Marschall, D. Gale, C. Williamson   +21 more
semanticscholar   +1 more source

A novel homozygous mutation in the USP53 gene as the cause of benign recurrent intrahepatic cholestasis in children: a case report

The Turkish Journal of Pediatrics, 2023
Background. Benign recurrent intrahepatic cholestasis (BRIC) is a rare cause of cholestasis with recurrent episodes of jaundice and pruritus without extrahepatic bile duct obstruction.
Burcu Berberoğlu Ateş, Ahmet Cevdet Ceylan, Gülin Hızal, Fatih Duran, Hayriye Tatlı Doğan, Şamil Hızlı   +5 more
doaj   +1 more source

Bile Acids in Intrahepatic Cholestasis of Pregnancy

Diagnostics, 2022
Intrahepatic cholestasis of pregnancy (ICP) is the most common, reversible, and closely related to pregnancy condition characterized by elevated levels of bile acids (BAs) in blood serum and an increased risk of adverse perinatal outcomes.
Maciej Majsterek, Magdalena Wierzchowska-Opoka, Inga Makosz, L. Kreczyńska, Żaneta Kimber-Trojnar, B. Leszczyńska-Gorzelak   +5 more
semanticscholar   +1 more source

Effects of cytomegalovirus infection on the differential diagnosis between biliary atresia and intrahepatic cholestasis in a Chinese large cohort study

Annals of Hepatology, 2021
Introduction and objectives: Differentiating biliary atresia from other causes of neonatal cholestasis is challenging, particularly when cytomegalovirus (CMV) and biliary atresia occur simultaneously.
Dongying Zhao, Xiaohui Gong, Yahui Li, Xiaoang Sun, Yan Chen, Zhaohui Deng, Yongjun Zhang   +6 more
doaj   +1 more source

Ursodeoxycholic acid in intrahepatic cholestasis of pregnancy: a systematic review and individual participant data meta-analysis

The Lancet Gastroenterology and Hepatology, 2021
Summary Background Ursodeoxycholic acid is commonly used to treat intrahepatic cholestasis of pregnancy, yet its largest trial detected minimal benefit for a composite outcome (stillbirth, preterm birth, and neonatal unit admission).
C. Ovadia, J. Sajous, Paul Townsend Seed, Kajol Patel, Nicholas J Williamson, G. Attilakos, F. Azzaroli, Y. Bacq, L. Batsry, K. Broom, Romana Brun-Furrer, L. Bull, J. Chambers, Yue Cui, Min Ding, P. Dixon, M. Estiù, F. Gardiner, V. Geenes, M. Grymowicz, Berrin Günaydın, W. Hague, C. Haslinger, Ya-Yi Hu, U. Indraccolo, A. Juusela, S. Kane, A. Kebapcilar, L. Kebapçılar, K. Kohari, J. Kondrackienė, M. P. Koster, Richard H. Lee, Xiaohua Liu, A. Locatelli, R. Macías, R. Madazlı, Agata Majewska, K. Maksym, J. Marathe, A. Morton, M. Oudijk, D. Öztekin, M. Peek, A. Shennan, R. Tribe, V. Tripodi, Naciye Türk Özterlemez, T. Vasavan, L. Wong, Y. Yinon, Qian-Wen Zhang, K. Zloto, H. Marschall, J. Thornton, L. Chappell, C. Williamson   +56 more
semanticscholar   +1 more source

Quercetin potentiates the hepatoprotective effect of sildenafil and/or pentoxifylline against intrahepatic cholestasis: Role of Nrf2/ARE, TLR4/NF-κB, and NLRP3/IL-1β signaling pathways.

Life Science, 2022
AIM Intrahepatic cholestasis is a common pathological condition of several types of liver disorders. In this study, we aimed to investigate the regulatory effects of quercetin (QU) on selected phosphodiesterase inhibitors against alpha-naphthyl ...
M. Fawzy, Gehad Nasr, Fares E. M. Ali, M. Fathy   +3 more
semanticscholar   +1 more source

Extrahepatic manifestations of progressive familial intrahepatic cholestasis syndromes: Presentation of a case series and literature review

Liver international (Print), 2022
Progressive familial intrahepatic cholestasis (PFIC) is a collective term for a heterogenous group of rare, inherited cholestasis syndromes. The number of genes underlying the clinical PFIC phenotype is still increasing.
Eva-Doreen Pfister, Carola Dröge, R. Liebe, A. Stalke, Nicole Buhl, A. Ballauff, T. Cantz, E. Bueltmann, J. Stindt, Tom Luedde, U. Baumann, V. Keitel   +11 more
semanticscholar   +1 more source

Compound Heterozygous MYO5B Mutation, a Cause of Infantile Cholestasis: A Case Report

Journal of Nepal Medical Association, 2022
Infantile cholestasis is a common clinical problem in early infancy characterised by impairment in bile formation and/or flow. It requires prompt evaluation for underlying aetiology to initiate appropriate management.
Muna Khanal, Adarsh Kumar Jha, Arun Kumar Sharma   +2 more
doaj   +1 more source

Genetics in Familial Intrahepatic Cholestasis: Clinical Patterns and Development of Liver and Biliary Cancers: A Review of the Literature

Cancers, 2022
Simple Summary An increase in serum bile acids can generate a chronic inflammatory state and has been associated with the risk of developing hepatobiliary cancers.
G. Vitale, A. Mattiaccio, A. Conti, Laura Turco, M. Seri, F. Piscaglia, M. Morelli   +6 more
semanticscholar   +1 more source