Biliary atresia and other cholestatic childhood diseases: advances and future challenges
| Autor: | Richard A. Schreiber, Mark Davenport, Marianne Samyn, Jan B F Hulscher, G. Mieli-Vergani, Claus Petersen, Jorge A. Bezerra, Deirdre Kelly, Benno M. Ure, Ronald J. Sokol, Peter F. Whitington, Henkjan J. Verkade |
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| Jazyk: | angličtina |
| Rok vydání: | 2016 |
| Předmět: |
medicine.medical_specialty
Pediatrics Biliary diversion medicine.medical_treatment BILE-DUCT Progressive familial intrahepatic cholestasis PROGNOSTIC SUBGROUP Disease Liver transplantation 03 medical and health sciences FAMILIAL INTRAHEPATIC CHOLESTASIS 0302 clinical medicine Cholestasis QUALITY-OF-LIFE Biliary Atresia Biliary atresia HEPATOCELLULAR-CARCINOMA Alagille syndrome medicine SURGICAL-APPROACH Humans Choledochal cysts ALAGILLE-SYNDROME Hepatology business.industry Bile duct Choledochal cyst General surgery INTRACTABLE PRURITUS LIVER-TRANSPLANTATION medicine.disease Choledochal malformation EXPORT PUMP DEFICIENCY Alagille Syndrome Europe medicine.anatomical_structure 030220 oncology & carcinogenesis North America 030211 gastroenterology & hepatology business |
| Zdroj: | Verkade, H J, Bezerra, J A, Davenport, M, Schreiber, R A, Mieli-Vergani, G, Hulscher, J B, Sokol, R J, Kelly, D A, Ure, B, Whitington, P F, Samyn, M & Petersen, C 2016, ' Biliary atresia and other cholestatic childhood diseases: advances and future challenges ', Journal of Hepatology . https://doi.org/10.1016/j.jhep.2016.04.032 |
| DOI: | 10.1016/j.jhep.2016.04.032 |
| Popis: | Biliary Atresia and other cholestatic childhood diseases are rare conditions affecting the function and/or anatomy along the canalicular-bile duct continuum, characterised by onset of persistent cholestatic jaundice during the neonatal period. Biliary atresia (BA) is the most common among these, but still has an incidence of only 1 in 10-19,000 in Europe and North America. Other diseases such as the genetic conditions, Alagille syndrome (ALGS) and Progressive Familial Intrahepatic Cholestasis (PFIC), are less common. Choledochal malformations are amenable to surgical correction and require a high index of suspicion. The low incidence of such diseases hinder patient-based studies that include large cohorts, while the limited numbers of animal models of disease that recapitulate the spectrum of disease phenotypes hinders both basic research and the development of new treatments. Despite their individual rarity, collectively BA and other cholestatic childhood diseases are the commonest indications for liver transplantation during childhood. Here, we review the recent advances in basic research and clinical progress in these diseases, as well as the research needs. For the various diseases, we formulate current key questions and controversies and identify top priorities to guide future research. (C) 2016 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved. |
| Databáze: | OpenAIRE |
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