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目的:总结新生儿胆汁淤积病例临床特征和病因,并分析胃肠外营养相关性胆汁淤积症(PNAC)和非PNAC患儿的差异。方法:对2013年11月至2016年11月苏州大学附属儿童医院住院的72例新生儿胆汁淤积病例进行回顾性分析。将所有患儿分为胃肠外营养相关性胆汁淤积症(PNAC)组39例,非PNAC组33例,观察两组喂养、静脉营养使用、感染史、窒息史等胆汁淤积相关因素,以及发病时间、肝脏受损情况、总胆红素和结合胆红素水平及相关实验室指标等,并分析两组引起淤胆的病因和预后差异。结果:PNAC组禁食、开奶、使用胃肠外营养的时间均明显长于非PNAC组(均P<0.01),有感染史、窒息、机械通气比例均高于非PNAC组(均P<0.01);NEC的发病率两组无统计学意义,非PNAC组发生淤胆、出现肝损伤的时间却明显早于PNAC组(均P<0.01)。两组好转率无统计学意义。淤胆病因中PNAC合并感染38例、非PNAC合并感染23例,胆道闭锁2例、先天多发畸形或染色体异常3例、遗传代谢性疾病3例、其他原因7例。结论:新生儿胆汁淤积病因繁多,PNAC主要见于早产儿,相对非PNAC患儿而言,其禁食,使用静脉营养时间更长,感染发生率更高,而非PNAC病因更为复杂。对明确淤胆的患儿应尽早病因诊断,并长期随访。
Objective: To summarize the clinical features and etiology of neonatal cholestasis and to analyze the differences between parenteral nutrition-associated cholestasis (PNAC) and non-PNAC children. Methods: A retrospective analysis of 72 cases of neonatal cholestasis hospitalized at Children’s Hospital Affiliated to Soochow University from November 2013 to November 2016 was conducted. All patients were divided into parenteral nutrition-related cholestasis (PNAC) group of 39 cases, non-PNAC group of 33 cases, the two groups fed, intravenous nutrition, infection history, history of choking and other cholestasis related factors, Time, liver damage, total bilirubin and bilirubin levels and related laboratory indicators, and analysis of the two groups caused by cholera etiology and prognosis differences. Results: The time of fasting, opening of milk and using parenteral nutrition in PNAC group were significantly longer than that of non-PNAC group (all P <0.01). The incidence of infection, asphyxia and mechanical ventilation were higher in PNAC group than in non-PNAC group ). The incidence of NEC was not statistically significant in both groups. The incidence of cholestasis in non-PNAC group was significantly earlier than that in PNAC group (all P <0.01). The improvement rate of two groups was not statistically significant. Cholera etiology PNAC infection in 38 cases, non-PNAC infection in 23 cases, biliary atresia in 2 cases, congenital multiple births or chromosomal abnormalities in 3 cases, 3 cases of genetic metabolic disease, and other causes in 7 cases. CONCLUSIONS: There are many causes of neonatal cholestasis. PNAC is mainly found in premature infants. Compared with non-PNAC infants, fasting, intravenous nutrition and infection rates are higher than those without PNAC, and the causes of non-PNAC are more complicated. Clearly cholestatic children should be diagnosed as soon as possible, and long-term follow-up.