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Quoted phrase not found in phrase index: "Cholestasis, progressive familial intrahepatic, 6"
Page 1
Progressive familial intrahepatic cholestasis.
Hori T, Nguyen JH, Uemoto S. Hori T, et al. Hepatobiliary Pancreat Dis Int. 2010 Dec;9(6):570-8. Hepatobiliary Pancreat Dis Int. 2010. PMID: 21134824 Review.
BACKGROUND: Three types of progressive familial intrahepatic cholestasis (PFIC) have been identified, but their etiologies include unknown mechanisms. DATA SOURCES: A PubMed search on "progressive familial intrahepatic cholestas …
BACKGROUND: Three types of progressive familial intrahepatic cholestasis (PFIC) have been identified, but their …
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. ...Although varying degrees of improvement in response to UDCA therapy were observed, 41. …
BACKGROUND: Progressive familial intrahepatic cholestasis type 3 (PFIC3) is an autosomal recessive disease …
Long-term outcome following cholecystocolostomy in 41 patients with progressive familial intrahepatic cholestasis.
Li Q, Chong C, Sun R, Yin T, Huang T, Diao M, Li L. Li Q, et al. Pediatr Surg Int. 2021 Jun;37(6):723-730. doi: 10.1007/s00383-021-04871-9. Epub 2021 Mar 2. Pediatr Surg Int. 2021. PMID: 33651176
PURPOSE: Progressive familial intrahepatic cholestasis (PFIC) is a cohort of autosomal recessive syndromes which presents with jaundice, severe pruritus and liver derangement. Without treatments, patients progress to liver failure in early child …
PURPOSE: Progressive familial intrahepatic cholestasis (PFIC) is a cohort of autosomal recessive syndromes which …
Laparoscopic button cholecystostomy for progressive familial intrahepatic cholestasis in two children.
Schukfeh N, Gerner P, Paul A, Kathemann S, Metzelder M. Schukfeh N, et al. Eur J Pediatr Surg. 2014 Oct;24(5):433-6. doi: 10.1055/s-0033-1360457. Epub 2013 Dec 10. Eur J Pediatr Surg. 2014. PMID: 24327224
BACKGROUND: Untreated, progressive familial intrahepatic cholestasis (PFIC) results in fibrosis, cirrhosis, and liver failure. ...Beside the suitability of the procedure, end points included course of serum bile acids, total bilirubin, liver enz …
BACKGROUND: Untreated, progressive familial intrahepatic cholestasis (PFIC) results in fibrosis, cirrhosis, and …
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). ...All had hepatocellular …
Progressive familial intrahepatic cholestasis, type 2 (PFIC2), characterized by cholestasis in infancy th …
Progressive familial intrahepatic cholestasis type 3: Report of four clinical cases, novel ABCB4 variants and long-term follow-up.
Lipiński P, Ciara E, Jurkiewicz D, Płoski R, Wawrzynowicz-Syczewska M, Pawłowska J, Jankowska I. Lipiński P, et al. Ann Hepatol. 2021 Nov-Dec;25:100342. doi: 10.1016/j.aohep.2021.100342. Epub 2021 Mar 20. Ann Hepatol. 2021. PMID: 33757843 Free article.
INTRODUCTION AND OBJECTIVES: Progressive familial intrahepatic cholestasis type 3 (PFIC-3) is a rare autosomal recessive cholestatic liver disorder caused by mutations in the ABCB4 gene. ...MATERIALS AND METHODS: The study included 4 patients with c …
INTRODUCTION AND OBJECTIVES: Progressive familial intrahepatic cholestasis type 3 (PFIC-3) is a rare autosomal r …
Nasobiliary drainage prior to surgical biliary diversion in progressive familial intrahepatic cholestasis type II.
Jannone G, Stephenne X, Scheers I, Smets F, de Magnée C, Reding R, Sokal EM. Jannone G, et al. Eur J Pediatr. 2020 Oct;179(10):1547-1552. doi: 10.1007/s00431-020-03646-z. Epub 2020 Apr 14. Eur J Pediatr. 2020. PMID: 32291498
Progressive familial intrahepatic cholestasis (PFIC) can cause intense pruritus that is refractory to medical therapy. ...What is Known: Surgical biliary diversion techniques have been developed to relieve intractable pruritus in progressive …
Progressive familial intrahepatic cholestasis (PFIC) can cause intense pruritus that is refractory to medical th …
Neurodevelopmental Outcomes in Children With Inherited Liver Disease and Native Liver.
Leung DH, Sorensen LG, Ye W, Hawthorne K, Ng VL, Loomes KM, Fredericks EM, Alonso EM, Heubi JE, Horslen SP, Karpen SJ, Molleston JP, Rosenthal P, Sokol RJ, Squires RH, Wang KS, Kamath BM, Magee JC; Childhood Liver Disease Research Network (ChiLDReN). Leung DH, et al. J Pediatr Gastroenterol Nutr. 2022 Jan 1;74(1):96-103. doi: 10.1097/MPG.0000000000003337. J Pediatr Gastroenterol Nutr. 2022. PMID: 34694263 Free PMC article.
OBJECTIVE: To evaluate neurodevelopmental status among children with inherited cholestatic liver diseases with native liver and variables predictive of impairment. METHODS: Participants with Alagille syndrome (ALGS), progressive familial intrahepatic …
OBJECTIVE: To evaluate neurodevelopmental status among children with inherited cholestatic liver diseases with native liver and variables …
Allograft steatohepatitis in progressive familial intrahepatic cholestasis type 1 after living donor liver transplantation.
Miyagawa-Hayashino A, Egawa H, Yorifuji T, Hasegawa M, Haga H, Tsuruyama T, Wen MC, Sumazaki R, Manabe T, Uemoto S. Miyagawa-Hayashino A, et al. Liver Transpl. 2009 Jun;15(6):610-8. doi: 10.1002/lt.21686. Liver Transpl. 2009. PMID: 19479804 Free article.
We studied histological features and long-term outcomes in patients with progressive familial intrahepatic cholestasis type 1 (PFIC1) after liver transplantation (LT). ...In conclusion, allograft steatosis was present in patients with PFIC1, progres …
We studied histological features and long-term outcomes in patients with progressive familial intrahepatic cholestas …
ATP8B1, ABCB11, and ABCB4 Genes Defects: Novel Mutations Associated with Cholestasis with Different Phenotypes and Outcomes.
Al-Hussaini A, Lone K, Bashir MS, Alrashidi S, Fagih M, Alanazi A, AlYaseen S, Almayouf A, Alruwaithi M, Asery A. Al-Hussaini A, et al. J Pediatr. 2021 Sep;236:113-123.e2. doi: 10.1016/j.jpeds.2021.04.040. Epub 2021 Apr 27. J Pediatr. 2021. PMID: 33915153
OBJECTIVES: To characterize the clinical, laboratory, histologic, molecular features, and outcome of gene-confirmed progressive familial intrahepatic cholestasis (PFIC) 1-3 among Arabs and to evaluate for "genotype-phenotype" correlations. STUDY DESIGN …
OBJECTIVES: To characterize the clinical, laboratory, histologic, molecular features, and outcome of gene-confirmed progressive fa …
48 results