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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 …
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 …
Metallothionein: a game changer in histopathological diagnosis of Wilson disease.
Wiethoff H, Mohr I, Fichtner A, Merle U, Schirmacher P, Weiss KH, Longerich T. Wiethoff H, et al. Histopathology. 2023 Dec;83(6):936-948. doi: 10.1111/his.15041. Epub 2023 Sep 4. Histopathology. 2023. PMID: 37661783
METHODS: MT immunohistochemistry was performed on liver specimens of WD patients (n = 64) and control cases (n = 160) including acute liver failure, steatotic liver disease, autoimmune hepatitis, normal liver, primary biliary cholangitis, primary and secondary sclerosing c …
METHODS: MT immunohistochemistry was performed on liver specimens of WD patients (n = 64) and control cases (n = 160) including acute liver …
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 …
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 …
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 …
The Mutational Landscape Of Genetic Cholestatic Diseases In Pakistani Children.
Cheema HA, Waheed N, Saeed A, Anjum MN, Fayyaz Z, Ijaz S. Cheema HA, et al. J Pak Med Assoc. 2023 Aug;73(8):1610-1621. doi: 10.47391/JPMA.7069. J Pak Med Assoc. 2023. PMID: 37697751 Free article.
Of the 70 variants identified, 50(71.4%) were novel variants. The ABCB11-related hereditary cholestasis was the most frequent 27(29%), followed by ABCB4 (26(27.9%). ...There was no evidence of phenotypic expression in the carrier parents despite the severe nature of …
Of the 70 variants identified, 50(71.4%) were novel variants. The ABCB11-related hereditary cholestasis was the most frequent 27(29%) …
Small intestinal bacterial overgrowth in patients with progressive familial intrahepatic cholestasis.
Lisowska A, Kobelska-Dubiel N, Jankowska I, Pawłowska J, Moczko J, Walkowiak J. Lisowska A, et al. Acta Biochim Pol. 2014;61(1):103-7. Epub 2014 Mar 17. Acta Biochim Pol. 2014. PMID: 24644547 Free article.
BACKGROUND & AIMS: To date, no studies concerning the presence of small intestinal bacterial overgrowth in patients with progressive familial intrahepatic cholestasis were published. Based upon characteristic of progressive familial …
BACKGROUND & AIMS: To date, no studies concerning the presence of small intestinal bacterial overgrowth in patients with progressive …
Morphologic findings in progressive familial intrahepatic cholestasis 2 (PFIC2): correlation with genetic and immunohistochemical studies.
Evason K, Bove KE, Finegold MJ, Knisely AS, Rhee S, Rosenthal P, Miethke AG, Karpen SJ, Ferrell LD, Kim GE. Evason K, et al. Am J Surg Pathol. 2011 May;35(5):687-96. doi: 10.1097/PAS.0b013e318212ec87. Am J Surg Pathol. 2011. PMID: 21490445 Free PMC article.
Progressive familial intrahepatic cholestasis, type 2 (PFIC2), characterized by cholestasis in infancy that may progress to cirrhosis, is caused by mutation in ABCB11, which encodes bile salt export pump (BSEP). ...Twelve patients with cl …
Progressive familial intrahepatic cholestasis, type 2 (PFIC2), characterized by cholestasis in infancy th …
51 results