熊去氧胆酸可改善囊性纤维化婴儿的胆汁淤积。
Ursodeoxycholic acid improves cholestasis in infants with cystic fibrosis.
作者信息
Scher H, Bishop W P, McCray P B
机构信息
Department of Pediatrics, College of Medicine, University of Iowa, Iowa City 52242, USA.
出版信息
Ann Pharmacother. 1997 Sep;31(9):1003-5. doi: 10.1177/106002809703100909.
OBJECTIVE
To describe two infants with cholestatic jaundice treated with ursodeoxycholic acid (UDCA).
CASE SUMMARY
Two infants with cystic fibrosis (CF)-associated hepatobiliary disease, manifesting as cholestatic jaundice and elevated liver enzymes within the first 6 weeks of life, had improved biochemical indices of liver function following treatment with UDCA 20-40 mg/kg/d.
DISCUSSION
To our knowledge, this is the first report of UDCA treatment in infants with CF-associated cholestatic jaundice. Infants and children require treatment with increased doses of UDCA to compensate for reduced intestinal absorption of bile acid and immaturity of the enterohepatic circulation.
CONCLUSIONS
UDCA appears to be a cost-effective treatment for CF-associated hepatobiliary disease in infants and children.
目的
描述两名接受熊去氧胆酸（UDCA）治疗的胆汁淤积性黄疸婴儿。
病例摘要
两名患有囊性纤维化（CF）相关肝胆疾病的婴儿，在出生后6周内表现为胆汁淤积性黄疸和肝功能酶升高，在接受20 - 40 mg/kg/d的UDCA治疗后，肝功能生化指标有所改善。
讨论
据我们所知，这是首例关于UDCA治疗CF相关胆汁淤积性黄疸婴儿的报告。婴儿和儿童需要增加UDCA剂量进行治疗，以补偿胆汁酸肠道吸收减少和肠肝循环不成熟的情况。
结论
UDCA似乎是治疗婴儿和儿童CF相关肝胆疾病的一种经济有效的疗法。
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