Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation

Search Page

Filters

My NCBI Filters

Results by year

Table representation of search results timeline featuring number of search results per year.

Year Number of Results
1995 1
1997 1
2001 1
2003 3
2005 1
2006 1
2007 1
2008 1
2009 2
2010 3
2012 1
2013 1
2014 3
2015 3
2016 2
2017 1
2018 4
2019 3
2020 1
2021 6
2022 4
2023 4
2024 3

Text availability

Article attribute

Article type

Publication date

Search Results

48 results

Results by year

Filters applied: . Clear all
Quoted phrase not found in phrase index: "Cholestasis, progressive familial intrahepatic, 9"
Page 1
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 …
Epidemiology and burden of progressive familial intrahepatic cholestasis: a systematic review.
Jones-Hughes T, Campbell J, Crathorne L. Jones-Hughes T, et al. Orphanet J Rare Dis. 2021 Jun 3;16(1):255. doi: 10.1186/s13023-021-01884-4. Orphanet J Rare Dis. 2021. PMID: 34082807 Free PMC article. Review.
BACKGROUND: Progressive familial intrahepatic cholestasis is a rare, heterogeneous group of liver disorders of autosomal recessive inheritance, characterised by an early onset of cholestasis with pruritus and malabsorption, which rapidly prog …
BACKGROUND: Progressive familial intrahepatic cholestasis is a rare, heterogeneous group of liver disorders of a …
Management and outcomes after liver transplantation for progressive familial intrahepatic cholestasis: A systematic review and meta-analysis.
Kavallar AM, Mayerhofer C, Aldrian D, Okamoto T, Müller T, Vogel GF. Kavallar AM, et al. Hepatol Commun. 2023 Sep 27;7(10):e0286. doi: 10.1097/HC9.0000000000000286. eCollection 2023 Oct 1. Hepatol Commun. 2023. PMID: 37756114 Free PMC article.
BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous rare congenital cholestatic liver disease. Disease progression might necessitate liver transplantation (LT). ...
BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous rare congenital cholestatic …
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. ...Eventually, 27% required LT: PIBD 10.7%, PEBD32%, IE 27%. The post-operative BA (AUROC-0.9) and bilirubin (AUROC …
BACKGROUND: Biliary diversion (BD) is indicated in progressive familial intrahepatic cholestasis (PFIC) with ref …
Impact of progressive familial intrahepatic cholestasis on caregivers: caregiver-reported outcomes from the multinational PICTURE study.
Mighiu C, O'Hara S, Ferri Grazzi E, Murray KF, Schattenberg JM, Ventura E, Karakaidos M, Taylor A, Brrang H, Dhawan A, Willemse J, Finnegan A. Mighiu C, et al. Orphanet J Rare Dis. 2022 Feb 2;17(1):32. doi: 10.1186/s13023-022-02177-0. Orphanet J Rare Dis. 2022. PMID: 35109890 Free PMC article.
BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a spectrum of rare genetic diseases characterized by inadequate bile secretion that requires substantial ongoing care, though little research is published in this area. ...Most caregiv …
BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a spectrum of rare genetic diseases characte …
Diagnostic yield and novel candidate genes by next generation sequencing in 166 children with intrahepatic cholestasis.
Zheng Y, Guo H, Chen L, Cheng W, Yan K, Zhang Z, Li M, Jin Y, Hu G, Wang C, Zhou C, Zhou W, Jia Z, Zheng B, Liu Z. Zheng Y, et al. Hepatol Int. 2024 Apr;18(2):661-672. doi: 10.1007/s12072-023-10553-6. Epub 2023 Jun 14. Hepatol Int. 2024. PMID: 37314652
Of the 52 individuals, 18 (35%) had metabolic liver diseases, 9 (17%) had syndromic cholestasis, 9 (17%) had progressive familial intrahepatic cholestasis, 3 (6%) had bile acid synthesis defects, 3(6%) had infantile liver failure a …
Of the 52 individuals, 18 (35%) had metabolic liver diseases, 9 (17%) had syndromic cholestasis, 9 (17%) had progres …
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 …
NR1H4 disease: rapidly progressing neonatal intrahepatic cholestasis and early death.
Li ZD, Li YC, Jing-Zhao, Wang JS, Xie XB. Li ZD, et al. Orphanet J Rare Dis. 2024 Apr 19;19(1):171. doi: 10.1186/s13023-024-03166-1. Orphanet J Rare Dis. 2024. PMID: 38641832 Free PMC article.
BACKGROUND: Clinical studies on progressive familial intrahepatic cholestasis (PFIC) type 5 caused by mutations in NR1H4 are limited. ...CONCLUSIONS: We identified three newly-diagnosed patients and five novel mutations. NR1H4-related PFIC typically ca …
BACKGROUND: Clinical studies on progressive familial intrahepatic cholestasis (PFIC) type 5 caused by mutations …
A Report of 2 Infant Siblings with Progressive Intrahepatic Familial Cholestasis Type 1 and a Novel Homozygous Mutation in the ATP8B1 Gene Treated with Partial External Biliary Diversion and Liver Transplant.
Jankowska I, Pawłowska J, Szymczak M, Ismail H, Broniszczak D, Cielecka-Kuszyk J, Socha P, Jarzębicka D, Czubkowski P. Jankowska I, et al. Am J Case Rep. 2021 Jul 20;22:e932374. doi: 10.12659/AJCR.932374. Am J Case Rep. 2021. PMID: 34283821 Free PMC article.
BACKGROUND Current treatment options for progressive intrahepatic familial cholestasis type 1 (PFIC-1) comprise ursodeoxycholic acid (UDCA), partial external biliary diversion (PEBD), and liver transplantation (LTx). ...The older brother, after initial …
BACKGROUND Current treatment options for progressive intrahepatic familial cholestasis type 1 (PFIC-1) comprise …
Ileal exclusion in children with progressive familial intrahepatic cholestasis.
Jankowska I, Czubkowski P, Kaliciński P, Ismail H, Kowalski A, Ryżko J, Pawłowska J. Jankowska I, et al. J Pediatr Gastroenterol Nutr. 2014 Jan;58(1):92-5. doi: 10.1097/MPG.0b013e3182a9097c. J Pediatr Gastroenterol Nutr. 2014. PMID: 24385022
OBJECTIVES: Children with progressive familial intrahepatic cholestasis (PFIC) rarely benefit from medical treatment and most patients require surgical intervention. ...The aim of this study was to analyze our experience with IE in children with PFIC. …
OBJECTIVES: Children with progressive familial intrahepatic cholestasis (PFIC) rarely benefit from medical treat …
48 results