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Quoted phrase not found in phrase index: "Cholestasis, progressive familial intrahepatic, 6"
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. ...The percentage of patients with serum bile acid response was also signif …
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. ...There were no …
BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a heterogenous group of inherited hepatocell …
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 …
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 …
Long-Term Outcomes of Patients With Progressive Familial Intrahepatic Cholestasis After Biliary Diversion.
Öztürk H, Sarı S, Sözen H, Eğritaş Gürkan Ö, Dalgıç B, Dalgıç A. Öztürk H, et al. Exp Clin Transplant. 2022 May;20(Suppl 3):76-80. doi: 10.6002/ect.PediatricSymp2022.O26. Exp Clin Transplant. 2022. PMID: 35570606 Free article.
OBJECTIVES: Progressive familial intrahepatic cholestasis is a heterogeneous group of genetic disorders characterized by disrupted bile homeostasis. ...The clinical symptoms that patients with progressive familial intrahepatic c …
OBJECTIVES: Progressive familial intrahepatic cholestasis is a heterogeneous group of genetic disorders characte …
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 …
Genetic determinants of cholangiopathies: Molecular and systems genetics.
Reichert MC, Hall RA, Krawczyk M, Lammert F. Reichert MC, et al. Biochim Biophys Acta Mol Basis Dis. 2018 Apr;1864(4 Pt B):1484-1490. doi: 10.1016/j.bbadis.2017.07.029. Epub 2017 Jul 27. Biochim Biophys Acta Mol Basis Dis. 2018. PMID: 28757171 Free article. Review.
Their spectrum ranges from fairly benign conditions as, for example, benign recurrent intrahepatic cholestasis to low-phospholipid associated cholelithiasis and progressive familial intrahepatic cholestasis (PFIC). ...In recent years our …
Their spectrum ranges from fairly benign conditions as, for example, benign recurrent intrahepatic cholestasis to low-phosphol …
Progressive intrahepatic cholestasis: mechanisms, diagnosis and therapy.
Shneider BL. Shneider BL. Pediatr Transplant. 2004 Dec;8(6):609-12. doi: 10.1111/j.1399-3046.2004.00240.x. Pediatr Transplant. 2004. PMID: 15598335 Review.
Progressive intrahepatic cholestasis (PIHC, also known as progressive familial intrahepatic cholestasis) is a general term encompassing a devastating group of illnesses manifest by severe morbidity and potential mortality. ...The m …
Progressive intrahepatic cholestasis (PIHC, also known as progressive familial intrahepatic cho …
Hepatic immunohistochemistry of bile transporters in progressive familial intrahepatic cholestasis.
El-Guindi MA, Sira MM, Hussein MH, Ehsan NA, Elsheikh NM. El-Guindi MA, et al. Ann Hepatol. 2016 Mar-Apr;15(2):222-9. doi: 10.5604/16652681.1193718. Ann Hepatol. 2016. PMID: 26845599 Free article.
Background. Diagnosis of progressive familial intrahepatic cholestasis (PFIC) is a challenging matter that involves the summation of clinical, laboratory, radiological, and liver histological parameters; in addition to specific investigations to exclud …
Background. Diagnosis of progressive familial intrahepatic cholestasis (PFIC) is a challenging matter that invol …
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 …
68 results