Outcomes following partial external biliary diversion in patients with progressive familial intrahepatic cholestasis.
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DOI: 10.1016/j.jpedsurg.2016.11.021
Abstract: BACKGROUND/PURPOSE PFIC is a family of bile acid (BA) transport disorders that may result in serious liver disease requiring transplantation. We reviewed our experience with PEBD as a method to improve liver function and avoid… read more here.
Keywords: pfic patients; partial external; pfic pfic; outcomes following ... See more keywords
Progressive Familial Intrahepatic Cholestasis Type 2 in an Indian Child.
Sign Up to like & getrecommendations! Published in 2017 at "Journal of pediatric genetics"
DOI: 10.1055/s-0036-1597912
Abstract: Progressive familial intrahepatic cholestasis (PFIC) is a chronic cholestasis syndrome that begins in infancy and usually progresses to cirrhosis within the first decade of life. There are three varieties of PFIC described: PFIC-1 occurs due… read more here.
Keywords: familial intrahepatic; progressive familial; intrahepatic cholestasis; pfic ... See more keywords
Neurodevelopmental Outcomes in Children With Inherited Liver Disease and Native Liver
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DOI: 10.1097/mpg.0000000000003337
Abstract: Supplemental Digital Content is available in the text ABSTRACT 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),… read more here.
Keywords: algs; liver; children inherited; liver disease ... See more keywords
Impact of progressive familial intrahepatic cholestasis on caregivers: caregiver-reported outcomes from the multinational PICTURE study
Sign Up to like & getrecommendations! Published in 2022 at "Orphanet Journal of Rare Diseases"
DOI: 10.1186/s13023-022-02177-0
Abstract: 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. We report health-related quality… read more here.
Keywords: picture study; familial intrahepatic; progressive familial; pfic ... See more keywords