Abstract:

Objective　To investigate the etiology of infant cholestasis，and to evaluate the commonly clinical diagnostic methods in diagnosing infant biliary atresia（BA）. Methods　A total of 142 hospitalized children with infant cholestasis in the pediatric ward of our hospital from January 1，2012 to November 30，2016 were included. Results　Totally 99 cases（69.7%） were comfirmed，and the most common causes were BA，cytomegalovirus infection and citrin protein deficiency. Totally 37 cases（26.1%） were diagnosed as idiopathic infant hepatitis and 6 cases（4.2%） were undiagnosed. Compared with no-BA group，acholie stools predominated in BA group，and the serum γ-GT in BA were significantly higher. Abdominal ultrasonography showed that 81.2% were cholecyst undiscovered or small in size in BA. Hepatobiliary scintigraphy（HBS） showed that 95.0% had no image of gallbladder or radioactive concentration in bowel and 5.0% had delayed image or radioactive conceatration in BA. The difference between the two group；was statistical（P＜0.01）. The?sensitivity，specificity，and? accuracy?of?acholic stools to diagnose BA were?91.3%，92.9%， and?92.2%， γ-GT≥300 U/L were?65.2%，91.8%， 83.3%， abdominal ultrasonography were 68.8%，90.8%， 84.9%， HBS were 95%， 50%， 68.0%， respectively. Conclusion　The common causes of infant cholestasis in BA，idiopathic hepatitis，cytomegalovirus infection，and Citrin protein deficiency. In the infant cholestasis with acholic stools，infracostal liver≥3 cm，serum γ-GT≥300 U/L，cholecyst undiscovered or small in size in abdominal ultrasonography，HBS undiscovered or delayed image of gallbladder or radioactive concentration in bowel，the BA should be suspected.

Key words: infant cholestasis, etiology, clinical manifestation, adjuvant examination

摘要：

目的　分析婴儿胆汁淤积症病因构成，探讨常用临床诊断方法在诊断婴儿胆道闭锁（BA）中价值。方法　选择2012-01-01—2016-11-30福建医科大学附属第一医院儿科住院治疗的142例婴儿胆汁淤积症患儿为研究对象，回顾分析其临床表现、实验室检查、影像学检查及诊断。结果　99例（69.7%）明确诊断，其最常见病因依次为BA、巨细胞病毒感染、Citrin蛋白缺乏；37例（26.1%）诊断为特发性婴儿肝炎，6例（4.2%）病因未明；BA组排陶土样便例数高于非胆道闭锁（NBA）组，血γ-GT明显高于NBA组（P均＜0.01）。以排陶土样便为评判标准诊断BA敏感度、特异度及准确度分别为86.8%、63.4%及69.8%，γ-GT≥300 U/L为评判分界点诊断BA灵敏度、特异度及准确度分别为65.2%、91.8%、83.3%。81.2%BA患儿胆囊窝未探及胆囊声像或胆囊体积小，NBA组仅13.8%（P＜0.01），以胆囊窝未探及胆囊声像或胆囊体积小为评判标准诊断BA敏感度、特异度及准确度分别为68.8%、90.8%及84.9%。95.0%BA患儿未见胆囊显影或肠道没有放射性浓聚，5.0%为延迟胆囊显影或肠道有放射性浓聚，NBA组50.0%未见胆囊显影或肠道没有放射性浓聚，50.0%为延迟胆囊显影或肠道有放射性浓聚，两组间比较差异有统计学意义（P＜0.01）；以放射性核素肝胆显像未见胆囊显影或肠道没有放射性浓聚为评判标准诊断BA敏感度、特异度及准确度分别为95%、50%及68.0%。结论　婴儿胆汁淤积症常见病因为BA、特发性肝炎、巨细胞病毒感染、Citrin蛋白缺乏等；如出现排陶土样便、肝肋下≥3 cm，血γ-GT≥300 U/L，腹部超声胆囊窝未探及胆囊声像或胆囊大小形态异常，核素显像未见胆囊显影或肠道没有放射性浓聚，需高度怀疑婴儿胆道闭锁。

关键词: 婴儿胆汁淤积症, 病因, 临床表现, 辅助检查