Mallouh A A, Asha M I
Department of Pediatrics, Dhahran Health Center, Saudi Arabia.
Pediatr Infect Dis J. 1988 Oct;7(10):689-92. doi: 10.1097/00006454-198810000-00004.
Differentiating acute cholestatic jaundice resulting from hepatic vasoocclusive crises and hepatitis in children with sickle cell disease can be difficult. Both conditions result in hyperbilirubinemia, mainly of the conjugated type, and in elevation of serum transaminases. Five children with sickle cell disease, acute severe cholestatic jaundice and negative serology for hepatitis A and B presented in good general condition, with modest elevation of serum transaminases, and had an early uneventful recovery. Five children with sickle cell disease and serologically proved hepatitis A infection were sicker, exhibited a similar elevation of bilirubin concentration with marked elevation of the serum transaminases and recovered more slowly. The clinical course and outcome of hepatitis A in children with sickle cell disease was similar to that of hepatitis A in normal children. Unlike early reports acute cholestatic jaundice in our patients with sickle cell disease, whether caused by hepatitis or by hepatic vasoocclusive crises, was found to be benign with an uneventful recovery.
区分镰状细胞病患儿因肝血管闭塞性危机和肝炎导致的急性胆汁淤积性黄疸可能具有挑战性。这两种情况都会导致高胆红素血症，主要是结合型高胆红素血症，并伴有血清转氨酶升高。五名患有镰状细胞病、急性重度胆汁淤积性黄疸且甲型和乙型肝炎血清学检查呈阴性的患儿，一般状况良好，血清转氨酶轻度升高，且早期恢复顺利。五名患有镰状细胞病且血清学证实感染甲型肝炎的患儿病情较重，胆红素浓度升高情况相似，血清转氨酶显著升高，恢复较慢。镰状细胞病患儿甲型肝炎的临床病程和结局与正常儿童甲型肝炎相似。与早期报告不同，我们发现镰状细胞病患者的急性胆汁淤积性黄疸，无论由肝炎还是肝血管闭塞性危机引起，均为良性，恢复顺利。
