Biliary atresia: 50 years after the first kasai.

Détails

ID Serval
serval:BIB_FC91CE0DE3EF
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Synthèse (review): revue aussi complète que possible des connaissances sur un sujet, rédigée à partir de l'analyse exhaustive des travaux publiés.
Collection
Publications
Titre
Biliary atresia: 50 years after the first kasai.
Périodique
Isrn Surgery
Auteur(s)
Wildhaber B.E.
ISSN
2090-5793 (Electronic)
ISSN-L
2090-5785
Statut éditorial
Publié
Date de publication
2012
Volume
2012
Pages
132089
Langue
anglais
Notes
Publication types: Journal Article Publication Status: ppublish; pdf : Review Article
Résumé
Biliary atresia is a rare neonatal disease of unknown etiology, where obstruction of the biliary tree causes severe cholestasis, leading to biliary cirrhosis and death in the first years of life, if the condition is left untreated. Biliary atresia is the most frequent surgical cause of cholestatic jaundice in neonates and should be evoked whenever this clinical sign is associated with pale stools and hepatomegaly. The treatment of biliary atresia is surgical and currently recommended as a sequence of, eventually, two interventions. During the first months of life a hepatoportoenterostomy (a "Kasai," modifications of which are discussed in this paper) should be performed, in order to restore the biliary flow to the intestine and lessen further damage to the liver. If this fails and/or the disease progresses towards biliary cirrhosis and life-threatening complications, then liver transplantation is indicated, for which biliary atresia represents the most frequent pediatric indication. Of importance, the earlier the Kasai is performed, the later a liver transplantation is usually needed. This warrants a great degree of awareness of biliary atresia, and the implementation of systematic screening for this life-threatening pathology.
Pubmed
Open Access
Oui
Création de la notice
21/02/2015 13:27
Dernière modification de la notice
20/08/2019 17:27
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