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Quoted phrase not found in phrase index: "Cholestasis, progressive familial intrahepatic, 5"
Page 1
Molecular overview of progressive familial intrahepatic cholestasis.
Amirneni S, Haep N, Gad MA, Soto-Gutierrez A, Squires JE, Florentino RM. Amirneni S, et al. World J Gastroenterol. 2020 Dec 21;26(47):7470-7484. doi: 10.3748/wjg.v26.i47.7470. World J Gastroenterol. 2020. PMID: 33384548 Free PMC article. Review.
This condition could be caused by defects of the hepatocytes, which are responsible for the complex process of bile formation and secretion, and/or caused by defects in the secretory machinery of cholangiocytes. Several mutations and pathways that lead to cholestasis have …
This condition could be caused by defects of the hepatocytes, which are responsible for the complex process of bile formation and secretion, …
Systematic review of progressive familial intrahepatic cholestasis.
Baker A, Kerkar N, Todorova L, Kamath BM, Houwen RHJ. Baker A, et al. Clin Res Hepatol Gastroenterol. 2019 Feb;43(1):20-36. doi: 10.1016/j.clinre.2018.07.010. Epub 2018 Sep 17. Clin Res Hepatol Gastroenterol. 2019. PMID: 30236549 Free article.
BACKGROUND AND AIMS: Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous group of rare genetic disorders associated with bile acid secretion or transport defects. ...Patients reported that pruritus was often severe and led to dermal …
BACKGROUND AND AIMS: Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous group of rare genet …
Odevixibat treatment in progressive familial intrahepatic cholestasis: a randomised, placebo-controlled, phase 3 trial.
Thompson RJ, Arnell H, Artan R, Baumann U, Calvo PL, Czubkowski P, Dalgic B, D'Antiga L, Durmaz Ö, Fischler B, Gonzalès E, Grammatikopoulos T, Gupte G, Hardikar W, Houwen RHJ, Kamath BM, Karpen SJ, Kjems L, Lacaille F, Lachaux A, Lainka E, Mack CL, Mattsson JP, McKiernan P, Özen H, Rajwal SR, Roquelaure B, Shagrani M, Shteyer E, Soufi N, Sturm E, Tessier ME, Verkade HJ, Horn P. Thompson RJ, et al. Lancet Gastroenterol Hepatol. 2022 Sep;7(9):830-842. doi: 10.1016/S2468-1253(22)00093-0. Epub 2022 Jul 1. Lancet Gastroenterol Hepatol. 2022. PMID: 35780807 Free article. Clinical Trial.
BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a group of inherited paediatric liver diseases resulting from mutations in genes that impact bile secretion. ...FINDINGS: Between June 21, 2018, and Feb 10, 2020, 62 patients (median a …
BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a group of inherited paediatric liver diseas …
Pediatric hepatocellular carcinoma.
Khanna R, Verma SK. Khanna R, et al. World J Gastroenterol. 2018 Sep 21;24(35):3980-3999. doi: 10.3748/wjg.v24.i35.3980. World J Gastroenterol. 2018. PMID: 30254403 Free PMC article. Review.
Perinatally acquired hepatitis-B virus, hepatorenal tyrosinemia, progressive familial intrahepatic cholestasis, glycogen storage disease, Alagille's syndrome and congenital portosystemic shunts are important predisposing factors. Majority of chi …
Perinatally acquired hepatitis-B virus, hepatorenal tyrosinemia, progressive familial intrahepatic cholestasis, …
Extrahepatic manifestations of progressive familial intrahepatic cholestasis syndromes: Presentation of a case series and literature review.
Pfister ED, Dröge C, Liebe R, Stalke A, Buhl N, Ballauff A, Cantz T, Bueltmann E, Stindt J, Luedde T, Baumann U, Keitel V. Pfister ED, et al. Liver Int. 2022 May;42(5):1084-1096. doi: 10.1111/liv.15200. Epub 2022 Mar 15. Liver Int. 2022. PMID: 35184362 Review.
BACKGROUND AND AIMS: 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. Whi …
BACKGROUND AND AIMS: Progressive familial intrahepatic cholestasis (PFIC) is a collective term for a heterogenou …
Outcomes of pediatric liver transplantation for progressive familial intrahepatic cholestasis.
Vasudevan AK, Shanmugam N, Rammohan A, Valamparampil JJ, Rinaldhy K, Menon J, Thambithurai R, Namasivayam S, Kaliamoorthy I, Rela M. Vasudevan AK, et al. Pediatr Transplant. 2023 Dec;27(8):e14600. doi: 10.1111/petr.14600. Epub 2023 Sep 7. Pediatr Transplant. 2023. PMID: 37675889
BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a heterogenous group of inherited hepatocellular disorders and the clinical aspects, role of liver transplantation (LT), and its outcomes remain largely unelucidated. ...Catch-up growt …
BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a heterogenous group of inherited hepatocell …
Biliary diversion in progressive familial intrahepatic cholestasis: a systematic review and meta-analysis.
Bolia R, Goel AD, Sharma V, Srivastava A. Bolia R, et al. Expert Rev Gastroenterol Hepatol. 2022 Feb;16(2):163-172. doi: 10.1080/17474124.2022.2032660. Epub 2022 Feb 15. Expert Rev Gastroenterol Hepatol. 2022. PMID: 35051344
BACKGROUND: Biliary diversion (BD) is indicated in progressive familial intrahepatic cholestasis (PFIC) with refractory pruritus. ...RESULTS: 25 studies [424 children (PEBD-301, PIBD-93, IE-30)] were included. Pruritus resolved in 59.5% [PIBD:72 …
BACKGROUND: Biliary diversion (BD) is indicated in progressive familial intrahepatic cholestasis (PFIC) with ref …
Cholestasis Due to USP53 Deficiency.
Bull LN, Ellmers R, Foskett P, Strautnieks S, Sambrotta M, Czubkowski P, Jankowska I, Wagner B, Deheragoda M, Thompson RJ. Bull LN, et al. J Pediatr Gastroenterol Nutr. 2021 May 1;72(5):667-673. doi: 10.1097/MPG.0000000000002926. J Pediatr Gastroenterol Nutr. 2021. PMID: 33075013 Free PMC article.
METHODS: Whole exome sequencing (WES) was performed in DNA from patients diagnosed with cholestasis, at different points on the continuum from progressive familial intrahepatic cholestasis to benign recurrent intrahepatic cholestasis …
METHODS: Whole exome sequencing (WES) was performed in DNA from patients diagnosed with cholestasis, at different points on the conti …
Single-center experience in management of progressive familial intrahepatic cholestasis.
Varol Fİ, Selimoğlu MA, Güngör Ş, Yılmaz S, Tekedereli İ. Varol Fİ, et al. Arab J Gastroenterol. 2021 Dec;22(4):310-315. doi: 10.1016/j.ajg.2021.05.021. Epub 2021 Nov 25. Arab J Gastroenterol. 2021. PMID: 34840097
BACKGROUND AND STUDY AIMS: Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessively inherited disease that causes intrahepatic-hepatocellular cholestasis. ...Liver transplantation was performed in 12 (35.3%) …
BACKGROUND AND STUDY AIMS: Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessively inhe …
Current and future therapies for inherited cholestatic liver diseases.
van der Woerd WL, Houwen RH, van de Graaf SF. van der Woerd WL, et al. World J Gastroenterol. 2017 Feb 7;23(5):763-775. doi: 10.3748/wjg.v23.i5.763. World J Gastroenterol. 2017. PMID: 28223721 Free PMC article. Review.
Familial intrahepatic cholestasis (FIC) comprises a group of rare cholestatic liver diseases associated with canalicular transport defects resulting predominantly from mutations in ATP8B1, ABCB11 and ABCB4. Phenotypes range from benign recurrent intrahepat …
Familial intrahepatic cholestasis (FIC) comprises a group of rare cholestatic liver diseases associated with canalicula …
94 results