Furuya K N, Roberts E A, Canny G J, Phillips M J
Division of Gastroenterology, Hospital for Sick Children, Toronto, Ontario, Canada.
J Pediatr Gastroenterol Nutr. 1991 Jan;12(1):127-30. doi: 10.1097/00005176-199101000-00023.
A male infant presenting with neonatal hepatitis syndrome, characterized by conjugated hyperbilirubinemia and very mild liver function test abnormalities, at 2 weeks of age was found to have no excretion of radioisotope into the intestinal tract on hepatobiliary scan. Liver biopsy revealed severe interlobular bile duct paucity. Other features of Alagille's syndrome were not present; other conditions frequently associated with interlobular bile duct paucity were also excluded. Subsequently, the infant was found to have cystic fibrosis. Cystic fibrosis is thus another disease that may be associated with paucity of interlobular bile ducts presenting as neonatal hepatitis syndrome, and this represents a different pathogenesis of cholestatic jaundice in neonates with cystic fibrosis besides those previously recognized.
一名2周大的男婴表现为新生儿肝炎综合征，其特征为结合胆红素血症和非常轻微的肝功能检查异常，在肝胆扫描中发现无放射性同位素排入肠道。肝活检显示严重的小叶间胆管稀少。阿拉吉耶综合征的其他特征不存在；其他常与小叶间胆管稀少相关的疾病也被排除。随后，发现该婴儿患有囊性纤维化。因此，囊性纤维化是另一种可能与表现为新生儿肝炎综合征的小叶间胆管稀少相关的疾病，这代表了囊性纤维化新生儿胆汁淤积性黄疸的一种不同于先前认识的发病机制。
