Ursodeoxycholic acid for treatment of cholestasis in children on long-term total parenteral nutrition: A pilot study

Ursodeoxycholic acid for treatment of cholestasis in children on long-term total parenteral nutrition: A pilot study
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DOI:
10.1053/gast.1996.v111.pm8780577
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发表时间:
1996-09-01
期刊:
影响因子:
29.4
通讯作者:
Guarino, A
Guarino, A
中科院分区:
医学1区
文献类型:
--
作者:
Spagnuolo, MI;Iorio, R;Guarino, A

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背景和目的:胆汁淤积性肝病 (CLD) 是全肠外营养 (TPN) 的常见且有时致命的并发症,可能需要停用 TPN。本试验研究的目的是评估熊去氧胆酸 (UDCA) 作为 TPN 相关 CLD 的治疗作用。方法:7 名因顽固性腹泻综合征而接受长期 TPN 的儿童(4 名男孩和 3 名女孩)出现胆汁淤积并接受 UDCA 治疗。通过监测 CLD 的临床和生化标志物来评估治疗效果,包括 γ-谷氨酰转肽酶 (GGT)、碱性磷酸酶 (ALE)、结合胆红素和丙氨酸氨基转移酶 (ALT)。结果:在所有儿童中,UDCA 与 CLD 体征的消失以及 4-8 周内胆汁淤积生化标志物的正常化相关。 3例在接受TPN期间暂时停用UDCA的儿童中观察到GGT、ALP和ALT血清水平反弹升高,然而,在重新开始UDCA后,所有病例的胆汁淤积标志物均恢复正常,并在恢复完全经口喂养后撤回UDCA;胆汁淤积未复发。六个孩子完全康复。一名儿童因缺乏血管通路而死亡。结论 UDCA 似乎是治疗儿童 TPN 相关胆汁淤积的有效方法。
Background & Aims: Cholestatic liver disease (CLD) is a frequent and sometimes fatal complication of total parenteral nutrition (TPN) that may require withdrawal of TPN, The aim of this pilot study was to evaluate ursodeoxycholic acid (UDCA) as treatment of TPN-associated CLD. Methods: Seven children (4 boys and 3 girls) undergoing long-term TPN because of intractable diarrhea syndrome developed cholestasis and were treated with UDCA. Treatment efficacy was evaluated by monitoring clinical and biochemical markers of CLD, including gamma-glutamyl transpeptidase (GGT), alkaline phosphatase (ALE), conjugated bilirubin, and alanine aminotransferase (ALT). Results: In all children, UDCA was associated with the disappearance of signs of CLD and with normalization of biochemical markers of cholestasis within 4-8 weeks. A rebound increase of GGT, ALP, and ALT serum levels was observed in 3 children in whom UDCA was temporarily discontinued while they were still undergoing TPN, However, after reinstitution of UDCA, markers of cholestasis normalized in all cases, UDCA was withdrawn on reinstitution of full oral feeding; there was no relapse of cholestasis. Six children fully recovered. One child died because of the lack of vascular access. Conclusions UDCA appears to be an effective treatment for TPN-related cholestasis in children.