中国儿童保健杂志 ›› 2016, Vol. 24 ›› Issue (11): 1209-1212.DOI: 10.11852/zgetbjzz2016-24-11-27

• ·经验交流· • 上一篇    下一篇

婴儿胆汁淤积症1 360例病因及临床转归分析

刘雨1,吴运芹2,李贵南2,庄严2,黄佳1,高喜容2,李双杰3   

  1. 1 南华大学儿科学院,湖南 衡阳 421000;
    2 湖南省儿童医院新生儿科,
    湖南 长沙 410000;
    3 湖南省儿童医院 肝病中心, 湖南 长沙 410000
  • 收稿日期:2016-03-23 发布日期:2016-11-01 出版日期:2016-11-01
  • 通讯作者: 高喜容,E-mail:gaoxirong@126.com;李双杰,E-mail:lesjie62@vip.sina.com
  • 作者简介:刘雨(1989-),女,研究生,主要研究方向为儿科学(新生儿方向)。

The etiology and clinical outcome of 1 360 infantile cholestasis cases.

LIU Yu1,WU Yun-qin2,LI Gui-nan2,ZHUANG Yan2,HUANG Jia1,GAO Xi-rong2,LI Shuang-jie3.   

  1. 1 University of South China,Hengyang,Hunan 421000,China;
    2 Department of Neonatal,Hunan Children's Hospital,
    Changsha,Hunan 410000,China;
    3 Liver Center of Hunan Children's Hospital,Changsha,Hunan 410000,China
    Corresponding author:GAO Xi-rong,E-mail:gaoxirong@126.com;LI Shuang-jie,E-mail:lesjie62@vip.sina.com
  • Received:2016-03-23 Online:2016-11-01 Published:2016-11-01
  • Contact: GAO Xi-rong,E-mail:gaoxirong@126.com;LI Shuang-jie,E-mail:lesjie62@vip.sina.com

摘要: 目的 分析婴儿胆汁淤积症的病因及临床转归,以助于临床早期诊断和治疗。方法 总结2011年1月-2015年9月本院收治的足月出生、生后3周~3月发生胆汁淤积症患儿的临床资料,分析其病因及临床特征,追踪其临床转归。结果 1 360例婴儿胆汁淤积症主要病因为:解剖或结构异常(316例,23.23%),感染性(540例,39.70%),遗传代谢性(145例,10.66%),不明原因(350例,25.74%),其他(9例,0.66%)。其中最常见的疾病为巨细胞病毒感染(485例,35.66%)、胆道闭锁(301例,22.13%)、希特林蛋白缺乏症 (96例,7.06%)。住院期间1 360例患儿中1 193例(87.72%)好转出院,166例(12.20%)明确诊断后放弃治疗,1例(0.07%)死亡。166例放弃治疗患儿中104例为胆道闭锁,13例外院手术现存活、12例家属情绪激动未告知预后、19例死亡(1例手术患儿)、60例失访。1例胰腺肿瘤死亡,希特林蛋白缺乏症、不明原因胆汁淤积门诊随访预后可。结论 婴儿胆汁淤积症病因复杂,巨细胞病毒、胆道闭锁、希特林蛋白缺乏症发病率高,早期病因诊断有利于改善其临床转归。

关键词: 胆汁淤积症, 巨细胞病毒, 胆道闭锁, 希特林蛋白缺乏症

Abstract: Objective To analyse the etiology and clinical outcome of infantile cholestasis. Method The clinical data were collected of 3 weeks years old to 3 months years old infants with cholestasis in our hospital from January 2011 to September 2015.The causes and clinical features of the disease were analyzed,and to track the clinical prognosis. Result Main cause of 1 360 cases with cholestasis were anatomic anomalies (316 cases,23.23%),infection (540 cases,39.70%),inborn errors of metabolism(145 cases,10.66%),unexplained (350 cases,25.74%),others (9 cases,0.66%).The three most common reason were cytomegalovirus infection (485 cases,35.66%),biliary atresia (301 cases,22.13%) and Citrin deficiency (96 cases,7.06%).1 193 cases (87.72%) were improved and discharged,166 cases (12.20%)were gived up treatment,and 1 case (0.07%) were dead. Conclusion The etiology of infantile cholestasis is complicated.Incidence of biliary atresia,cytomegalovirus,citrin deficiency in cholestatic jaundice is high,early etiology diagnosis is beneficial to improve the clinical outcome.

Key words: cholestasis, cytomegalovirus, biliary atresia, citrin deficiency

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