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Quoted phrase not found in phrase index: "Cholestasis, progressive familial intrahepatic, 5"
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. ...Two primary endpoints were evaluated: proportion of positive pruritus as …
BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a group of inherited paediatric liver diseas …
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. ...RESULTS: Of 60 …
BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a heterogenous group of inherited hepatocell …
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 …
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. ...When stratif …
BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a spectrum of rare genetic diseases characte …
Effects of odevixibat on pruritus and bile acids in children with cholestatic liver disease: Phase 2 study.
Baumann U, Sturm E, Lacaille F, Gonzalès E, Arnell H, Fischler B, Jørgensen MH, Thompson RJ, Mattsson JP, Ekelund M, Lindström E, Gillberg PG, Torfgård K, Soni PN. Baumann U, et al. Clin Res Hepatol Gastroenterol. 2021 Sep;45(5):101751. doi: 10.1016/j.clinre.2021.101751. Epub 2021 Jun 26. Clin Res Hepatol Gastroenterol. 2021. PMID: 34182185 Clinical Trial.
PURPOSE: Ileal bile acid transporter inhibition is a novel therapeutic concept for cholestatic pruritus and cholestatic liver disease progression. Odevixibat, a potent, selective, reversible ileal bile acid transporter inhibitor, decreases enteric bile acid reuptake …
PURPOSE: Ileal bile acid transporter inhibition is a novel therapeutic concept for cholestatic pruritus and cholestatic liver disease …
Clinical and genetic characterization of pediatric patients with progressive familial intrahepatic cholestasis type 3 (PFIC3): identification of 14 novel ABCB4 variants and review of the literatures.
Chen R, Yang FX, Tan YF, Deng M, Li H, Xu Y, Ouyang WX, Song YZ. Chen R, et al. Orphanet J Rare Dis. 2022 Dec 22;17(1):445. doi: 10.1186/s13023-022-02597-y. Orphanet J Rare Dis. 2022. PMID: 36550572 Free PMC article. Review.
BACKGROUND: Progressive familial intrahepatic cholestasis type 3 (PFIC3) is an autosomal recessive disease caused by pathogenic variants of the gene ABCB4. ...Positive responses at varied degrees to oral ursodeoxycholic acid (UDCA) treatment wer …
BACKGROUND: Progressive familial intrahepatic cholestasis type 3 (PFIC3) is an autosomal recessive disease …
Long-term Outcomes of Living-donor Liver Transplantation for Progressive Familial Intrahepatic Cholestasis Type 1.
Okamoto T, Sonoda M, Ogawa E, Ito S, Togawa T, Hayashi H, Okajima H, Uemoto S. Okamoto T, et al. J Pediatr Gastroenterol Nutr. 2021 Mar 1;72(3):425-429. doi: 10.1097/MPG.0000000000002983. J Pediatr Gastroenterol Nutr. 2021. PMID: 33264179
OBJECTIVES: Progressive familial intrahepatic cholestasis type 1 (PFIC-1), an autosomal recessive disorder, is characterized by cholestasis, jaundice, and refractory pruritus. In some patients with PFIC-1, liver cirrhosis and end-stage liver …
OBJECTIVES: Progressive familial intrahepatic cholestasis type 1 (PFIC-1), an autosomal recessive disorder, is c …
Validation of the PRUCISION Instruments in Pediatric Patients with Progressive Familial Intrahepatic Cholestasis.
Gwaltney C, Ivanescu C, Karlsson L, Warholic N, Kjems L, Horn P. Gwaltney C, et al. Adv Ther. 2022 Nov;39(11):5105-5125. doi: 10.1007/s12325-022-02262-7. Epub 2022 Sep 6. Adv Ther. 2022. PMID: 36066745 Free PMC article.
INTRODUCTION: Patients with cholestatic liver disease, including progressive familial intrahepatic cholestasis (PFIC) and Alagille syndrome, may have debilitating pruritus, and reducing pruritus is a key therapeutic goal. ...CONCLUSION: The PRUC …
INTRODUCTION: Patients with cholestatic liver disease, including progressive familial intrahepatic cholestasi …
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. ...In both, pruritus varied and elevated serum bile acids were observed. Of the …
OBJECTIVES: Children with progressive familial intrahepatic cholestasis (PFIC) rarely benefit from medical treat …
Optimal liver transplant procedure in progressive familial intrahepatic cholestasis type 1 treated with biliary diversion or intestinal transplantation: Lessons learned from three cases treated with different approaches.
Uchida H, Sakamoto S, Komine R, Kodama T, Nakao T, Yanagi Y, Shimizu S, Abbas SH, Fukuda A, Kasahara M. Uchida H, et al. Pediatr Transplant. 2023 Sep;27(6):e14566. doi: 10.1111/petr.14566. Epub 2023 Jul 7. Pediatr Transplant. 2023. PMID: 37417206
BACKGROUND: Progressive familial intrahepatic cholestasis type 1 (PFIC1) is an autosomal recessive cholestatic liver disorder caused by ATP8B1 gene mutations. ...CASES: The first patient had jaundice, pruritus, diarrhea, and growth retardation (weight …
BACKGROUND: Progressive familial intrahepatic cholestasis type 1 (PFIC1) is an autosomal recessive cholestatic l …
56 results