中国当代儿科杂志2016，Vol.18Issue(4)：335-339,5.DOI:10.7499/j.issn.1008-8830.2016.04.011
儿童先天性肝纤维化临床分型及特征
Clinical features of different clinical forms of childhood congenital hepatic ifbrosis
吴欣 1杜霄壤 1丁金芳 1吴孟晋 1罗生强 2冯兴中1
作者信息
- 1. 首都医科大学附属北京世纪坛医院中医科，北京 100038
- 2. 解放军第302医院中西医结合肝病中心，北京 100039
- 折叠
摘要
Abstract
ObjectiveTo compare the clinical features of children with different clinical forms of congenital hepatic ifbrosis (CHF), and provides a description of the characteristics of childhood CHF.MethodsSixty children with CHF between January 2002 and June 2015 were enrolled, including 26 children with portal hypertensive CHF (PH CHF), 3 children with cholangitic CHF, 30 children with combined portal hypertensive and cholangitic CHF (mixed CHF), and 1 child with latent forms of CHF. The medical data of 26 children with PH CHF and 30 children with mixed CHF, including gender, age, clinical manifestations, physical signs, laboratory tests and imaging characteristics, were retrospectively studied.ResultsFever, jaundice and hepatomegaly were more frequently noted in children with mixed CHF than in those with PH CHF (P<0.05). Splenomegaly and liver cirrhosis occurred more often in children with CHF, but there was no signiifcant difference in the incidences of splenomegaly and liver cirrhosis between the children with PH CHF and mixed CHF. The plasma prothrombin activity, white blood cell counts, platelet counts, mean platelet volume, serum levels of alanine transaminase, aspartate transaminase, alkaline phosphatase, γ-glutamyl transferase, leucine aminopeptidase, and total bile acids in children with mixed CHF were higher than in those with PH CHF (P<0.05). The decreased international normalized ratio and lower serum albumin levels were more frequently observed in children with mixed CHF than in those with PH CHF (P<0.05).ConclusionsPH and mixed CHF are common forms in childhood CHF. The children with the two forms of PH usually manifest portal hypertension such as cirrhosis and hepatosplenomegaly. The liver damage may be common in children with mixed CHF.关键词
先天性肝纤维化/门静脉高压/儿童Key words
Congenital hepatic ifbrosis/Portal hypertension/Child引用本文复制引用
吴欣,杜霄壤,丁金芳,吴孟晋,罗生强,冯兴中..儿童先天性肝纤维化临床分型及特征[J].中国当代儿科杂志,2016,18(4):335-339,5.
