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 fibrosis

摘要:

目的 比较不同临床分型先天性肝纤维化(CHF)患儿的临床特征,总结儿童CHF 的特点.方法 2002 年1 月至2015 年6 月期间确诊为CHF 的60 例患儿中,门脉高压型26 例,胆管炎型3 例,混合型30 例,隐匿型1 例.采集26 例门脉高压型和30 例混合型患儿的性别、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研究.结果 发热、黄疸和肝肿大在混合型患儿中的发生率高于门脉高压型患儿(P<0.05),CHF 患儿脾肿大和肝硬化发生率高,但在两型患儿中比较差异无统计学意义(P>0.05);混合型患儿凝血酶原活动度、白细胞计数、血小板计数、血小板平均体积、丙氨酸转氨酶、天冬氨酸转氨酶、碱性磷酸酶、ν谷氨酰转肽酶、亮氨酸氨基肽酶和总胆汁酸水平高于门脉高压型患儿,国际标准化比值和白蛋白水平低于门脉高压型患儿(P<0.05).结论 儿童CHF 中以门脉高压型和混合型常见,门脉高压型和混合型均以肝硬化、肝脾肿大等门脉高压表现明显,但混合型常伴肝损伤.

Abstract:

Objective To compare the clinical features of children with different clinical forms of congenital hepatic fibrosis (CHF), and provides a description of the characteristics of childhood CHF. Methods Sixty 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. Results Fever, 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 significant 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). Conclusions PH 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.