儿童慢性胆汁淤积可能发生与维生素E缺乏有关的,具有特征的进行性和废用性神经系统病变,但其最早体征和发病时间均不清楚。作者认为确定胆汁淤积儿童的发病率和严重程度是很重要的,故对48例儿童以前证实为胆汁淤积患者和23例儿童非胆汁淤积性吸收障碍或进行性共济失调患者进行血清维生素E测定及神经系统检查。方法将患儿分为3组。第1组48例为各类型的胆汁淤积(胆道闭锁,新生儿肝炎,Alagille综合征)其中40例曾进行Kasai型肠造口术,并定期随访,此组患儿均有血清胆酸浓度升高,结合高胆红质血症及其他肝功能损害。第2组,11例为非胆汁淤积性吸 Chronic cholestasis of children may present with characteristic progressive and disabling neurological disorders that are associated with vitamin E deficiency but their earliest signs and timing of onset are unknown. The authors concluded that it is important to determine the incidence and severity of cholestatic children, and therefore serum vitamin E was measured in 48 children previously diagnosed with cholestasis and in 23 children with non-cholestatic dysfunction or progressive ataxia And neurological examination. Methods The children were divided into 3 groups. The first group of 48 cases of various types of cholestasis (biliary atresia, neonatal hepatitis, Alagille syndrome) of which 40 cases had Kasai-type enterostomy, and regular follow-up, this group of children have serum bile acid concentration High, combined with hypercholesterolemia and other liver damage. Group 2, 11 cases of non-cholestatic suction