Jacquemin Emmanuel
Hépatologie pédiatrique et Centre de référence de l'atrésie des voies biliaires, CHU de Bicêtre, AP-HP, Le Kremlin-Bicêtre (94), France.
Presse Med. 2007 Jun;36(6 Pt 2):945-8. doi: 10.1016/j.lpm.2007.03.018. Epub 2007 Apr 16.
Biliary atresia is a rare disease, but it is the major cause of neonatal cholestasis and the major indication for liver transplantation in children. Kasaï procedure can restore bile flow and prevent or slow progression of disease in a proportion of patients. Data show that the earlier the Kasaï procedure is performed, the better is the outcome. Therefore, rapid referral to an experienced center, for prompt diagnosis and surgery, is strongly recommended. Unfortunately, the disease is often detected late and Kasaï procedure is performed after 60 days of age. In an attempt to achieve earlier diagnosis and better outcome of Kasaï procedure, we propose to institute routine screening for biliary atresia using a stool colorometric scale. This should help to identify earlier children who have acholic stools and may have biliary atresia. We postulate that this screening method will allow to improve the results of the Kasaï procedure and provide children with the best chance of survival with their native liver.
胆道闭锁是一种罕见疾病,但它是新生儿胆汁淤积的主要病因以及儿童肝移植的主要指征。葛西手术可以恢复胆汁流动,并在一定比例的患者中预防或减缓疾病进展。数据显示,葛西手术实施得越早,预后越好。因此,强烈建议迅速转诊至有经验的中心,以便及时诊断和手术。不幸的是,该病常常在晚期才被发现,葛西手术在患儿60日龄后才实施。为了实现更早诊断并改善葛西手术的预后,我们建议采用粪便比色法对胆道闭锁进行常规筛查。这应有助于更早识别出粪便无胆汁且可能患有胆道闭锁的患儿。我们推测,这种筛查方法将改善葛西手术的效果,并为患儿提供依靠自身肝脏获得最佳生存机会。