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。
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.
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
Key words
cholestasis /
cytomegalovirus /
biliary atresia /
citrin deficiency
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