Clinical features of congenital hepatic ifbrosis in children
10.3969/j.issn.1000-3606.2016.06.011
- VernacularTitle:儿童先天性肝纤维化的临床特点
- Author:
Xin WU
;
Xiaorang DU
;
Jinfang DING
;
Mengjin WU
;
Shengqiang LUO
;
Xingzhong FENG
- Publication Type:Journal Article
- Keywords:
congenital hepatic ifbrosis;
Caroli disease;
ductal plate malformation
- From:
Journal of Clinical Pediatrics
2016;34(6):444-448
- CountryChina
- Language:Chinese
-
Abstract:
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.