肝内胆汁淤积且肝外胆道通畅的婴儿的长期预后
Long-term prognosis for infants with intrahepatic cholestasis and patent extrahepatic biliary tract.
作者信息
Odièvre M, Hadchouel M, Landrieu P, Alagille D, Eliot N
出版信息
Arch Dis Child. 1981 May;56(5):373-6. doi: 10.1136/adc.56.5.373.
Abstract
One hundred and three infants with prolonged cholestasis beginning before 3 months were classified as having alpha-1-antitrypsin deficiency (17 patients), scanty interlobular bile ducts (16 patients), or "neonatal hepatitis" (70 patients). Twenty-two gradually developed chronic liver disease and the remaining 81 recovered within a few months. Prognosis was found to be poor for infants with alpha-1-antitrypsin deficiency, scanty interlobular bile ducts, and familial "idiopathic" hepatitis. Patients who developed cirrhosis often presented with severe and persistent neonatal cholestasis, mimicking extrahepatic biliary atresia and leading to laparotomy. Thus, a high-risk group of infants-defined by aetiology, family history, and degree of cholestasis-can be recognised in the first months of life.
摘要
103例在3个月前开始出现长期胆汁淤积的婴儿被分类为患有α-1-抗胰蛋白酶缺乏症（17例）、小叶间胆管稀少（16例）或“新生儿肝炎”（70例）。22例逐渐发展为慢性肝病，其余81例在几个月内康复。发现α-1-抗胰蛋白酶缺乏症、小叶间胆管稀少和家族性“特发性”肝炎的婴儿预后较差。发展为肝硬化的患者常表现为严重且持续的新生儿胆汁淤积，类似肝外胆道闭锁并导致剖腹手术。因此，在生命的最初几个月就可以识别出一组由病因、家族史和胆汁淤积程度定义的高危婴儿。
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