Results 141 to 150 of about 63,979 (262)

Impact of cystic fibrosis transmembrane conductance regulator modulator therapies on liver stiffness and liver enzymes: An observational perspective single‐center cohort study

open access: yesJPGN Reports, EarlyView.
Abstract Objectives The efficacy of cystic fibrosis transmembrane conductance regulator (CFTR)‐modulator therapies in preventing or ameliorating cystic fibrosis liver disease (CFLD) by correcting CFTR in cholangiocytes is not well‐documented. This study aimed to assess liver function during CFTR‐modulators.
Laura Giugliano   +12 more
wiley   +1 more source

A hepatic enigma: Pediatric presentation of primary biliary cholangitis

open access: yesJPGN Reports, EarlyView.
Abstract Primary biliary cholangitis (PBC) is a chronic autoimmune condition characterized by destruction of intrahepatic bile ducts, leading to fibrosis and cirrhosis of the liver. It is an extremely rare pediatric disease with very few pediatric cases reported to date. Here, we report the case of a 14‐year‐old female who presented with elevated liver
Sindhura Kasturi   +3 more
wiley   +1 more source

Effects of post‐hepatic portoenterostomy adjuvant therapy on liver transplantation in children with biliary atresia: A systematic review

open access: yesJPGN Reports, EarlyView.
Abstract Objectives Biliary atresia (BA) is a cholangiopathy characterized by obstruction of the intrahepatic and extrahepatic bile ducts. Hepatic portoenterostomy (HPE) is the primary palliative treatment and there is still an urgent need to improve post‐HPE management.
Bianca Ferraz de Almeida Silva   +7 more
wiley   +1 more source

The Brazilian Alagille syndrome study: New insights from a multicenter national cohort

open access: yesJPGN Reports, EarlyView.
Abstract Objectives To elucidate the natural history of liver disease and identify the predictors of native liver survival (NLS) in a Brazilian cohort of children with Alagille syndrome (ALGS). Methods Multicenter retrospective cohort study of children with ALGS. Descriptive statistics summarized clinical data.
Elisa Carvalho   +34 more
wiley   +1 more source

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