婴儿胆汁淤积症1360例病因及临床转归分析

The etiology and clinical outcome of 1360 infantile cholestasis cases

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。

Objective To analyse the etiology and clinical outcome of infantile cholestasis. Method The clinical data were collected of 3weeks years old to 3months 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. ResultMain cause of 1 360 cases with cholestasis were anatomic anomalies（316cases,23.23%）,infection（540cases,39.70%）,inborn errors of metabolism（145cases,10.66%）,unexplained（350cases,25.74%）,others（9cases,0.66%）.The three most common reason were cytomegalovirus infection（485cases,35.66%）,biliary atresia（301cases,22.13%）and Citrin deficiency（96cases,7.06%）.1 193cases（87.72%）were improved and discharged,166cases（12.20%）were gived up treatment,and 1case（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.