婴儿胆汁淤积症的鉴别诊断思路

朱启镕; 王建设

婴儿胆汁淤积症的鉴别诊断思路

复旦大学附属儿科医院

详细信息

中图分类号: R725.7
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出版历程
- 收稿日期: 2011-03-29
- 出版日期: 2011-07-20

Differential diagnosis of infantile cholestasis

摘要

摘要: 胆汁淤积是婴儿肝脏疾病最常见的临床表现。病因在很大程度上决定其预后,有些病因需要医生做出快速的诊断及采取恰当的治疗。胆道闭锁、败血症、尿路感染、甲状腺功能低下、Citrin缺陷病、酪氨酸血症及先天性胆汁酸合成缺陷都要及早诊断,因为对这些疾病及时适当的治疗可显著的改善预后。
- 胆汁淤积 /
- 黄疸 /
- 胆道闭锁
Abstract: Cholestasis is the most common manifestation of liver diseases in infancy.The clinical outcome is mostly dependent on the etiology, some of them require prompt identification and then proper management.Biliary atresia, sepsis, urinary tract infection, hypothyroidism, citrin deficiency, tyrosemia and congenital bile acid synthetic defects need highest priority, because proper management could improve the outcome significantly.
- cholestasis /
- jaundice /
- biliary atresia

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参考文献(10)

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[7]Fu HY, Zhang SR, Yu H, et al.Most common SLC25A13 mutationin 400 Chinese infants with intrahepatic cholestasis[J].World JGastroenterol, 2010, 16 (18) :2278-2282.

[8]方玲娟, 王建设.先天性胆汁酸合成障碍及胆汁淤积性肝病[J].临床肝胆病杂志, 2010, 26 (6) :585-588.

[9]Gonzales E, Gerhardt MF, Fabre M, et al.Oral cholic acid forhereditary defects of primary bile acid synthesis:a safe andeffective long-term therapy[J].Gastroenterology, 2009, 137 (4) :1310-1320.

[10]Pierre G, Setchell K, Blyth J, et al.Prospective treatment ofcerebrotendinous xanthomatosis with cholic acid therapy[J].JInherit Metab Dis.2008 Dec 27.[Epub ahead of print]

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