Abstract
Biliary atresia (BA), a uniquely pediatric liver disease, is the leading cause of liver-related death in children and the most frequent indication for liver transplantation in the pediatric population. Early intervention with a Kasai procedure (KP) is the current standard of care for this condition. The single most important and well-established prognostic factor for the KP outcome is the patient‘s age at the time of the KP. The older the infant, the less successful the operation and the less favourable is the post-KP survival with native liver. There remains in Canada, and throughout the world, a problem of late referral, delayed diagnosis and older age at surgery. Early disease detection and intervention has been hampered by the lack of an effective screening strategy for BA. Recently, however, novel programs for the early identification of BA in the first month of life, but after two weeks of age, have been successfully implemented and evaluated in some countries, with significantly improved outcomes for affected infants. Whether any of these programs should be adopted to improve the timing of referral and treatment for Canadian infants affected with this devastating liver disease deserves consideration and study.
Key words: Pediatric liver disease, neonatal cholestasis, newborn screening
Résumé
L’atrésie des voies biliaires (AVB), une maladie du foie qui n’atteint que les enfants, est la principale cause de décès par maladie du foie chez l’enfant et l’indication la plus fréquente de transplantation hépatique dans la population pédiatrique. Une intervention de Kasaï (IK) précoce est la norme actuelle de traitement pour cette maladie. L’âge du patient au moment de l’intervention est le facteur le plus important et le mieux attesté pour un pronostic favorable. Plus le nourrisson est vieux, moins l’intervention est couronnée de succès et moins les chances de survie après l’IK sont bonnes avec le foie d’origine. Au Canada comme dans le reste du monde, il y a encore des retards dans l’aiguillage et le diagnostic des patients, ce qui fait que les nourrissons sont trop âgés au moment de la chirurgie. La détection et l’intervention précoces sont compliquées par l’absence d’une stratégie de dépistage efficace de l’AVB. Mais récemment, certains pays ont mis en oeuvre et évalué avec succès des programmes novateurs de détection rapide de l’AVB entre deux semaines et un mois de vie; ces programmes ont significativement amélioré les résultats cliniques des nourrissons atteints. Faudrait-il les adopter afin d’accélérer l’aiguillage et le traitement des nourrissons canadiens qui présentent cette atteinte hépatique dévastatrice? La question mérite d’être étudiée.
Mots clés: maladies du foie de l’enfant, cholestase néonatale, dépistage chez le nouveau-né
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