儿童先天性肝纤维化的临床特点

Clinical features of congenital hepatic fibrosis in children

目的探讨儿童先天性肝纤维化(CHF)的临床特点。方法选取2002年1月至2015年6月首次确诊的111例CHF患者为研究对象,根据确诊时年龄分为儿童组60例和成年组51例,采集两组的性别、年龄、临床表现、体征、实验室检查、影像特点等临床资料进行回顾性对比分析。结果儿童组和成年组的脾肿大和肝硬化发生率均高,差异无统计学意义(P>0.05);儿童组乏力、黄疸、肝脾肿大的发生率高于成年组,成年组则多见合并肾囊肿和肝囊肿,差异均有统计学意义(P<0.05);儿童组的丙氨酸转氨酶(ALT)、天冬氨酸转氨酶(AST)、碱性磷酸酶(ALP)、亮氨酸氨基肽酶(LAP)、总胆汁酸(TBA)水平以及AST异常率高于成年组,差异有统计学意义(P<0.05)。结论 CHF患者常见肝硬化和脾肿大。儿童期确诊CHF者肝脾肿大更明显,ALT、AST和ALP、LAP、TBA升高及异常更多,肾囊肿和肝囊肿相对少见。

Objective To explore the clinical features of congenital hepatic fibrosis (CHF) in children.MethodsA total of 111 patients diagnosed with CHF during January 2002 to June 2015 were included and divided into children group and adult group according to the age at diagnosis. The clinical data including gender, age, clinical symptoms, signs, laboratory examinations, and imaging characteristics were retrospectively analyzed and compared between two groups.ResultsThere were high incidences of splenomegaly and liver cirrhosis in both children and adult groups, and no difference was found between two groups (P>?0.05). The fatigue, jaundice, and hepatosplenomegaly were more common in children group while the renal cyst and liver cyst were more common in adult group, and the differences were statistically signiifcant (P all?<?0.05). Compared with adult group, the abnormal rate of ALT, AST, ALP, LAP, TBA, and AST were signiifcantly higher in children group (P all?<?0.05). ConclusionCHF patients often have hepatocirrhosis and splenomegaly. CHF patients diagnosed in childhood have more sever hepatosplenomegaly, are more in high levels of ALT, AST, ALP, LAP, and TBA, and less in renal cyst and hepatic cyst.