儿童先天性肝纤维化临床分型及特征-.docVIP

doi: 10.7499/j.issn.1008-8830.2016.04.011 论著·临床研究儿童先天性肝纤维化临床分型及特征 吴欣1　杜霄壤1　丁金芳1　吴孟晋1　罗生强2　冯兴中1 (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 [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(P0.05,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(P0.05;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(P0.05。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。 [中国当代儿科杂志,2016,18(4:335-339] [关键词]　先天性肝纤维化;门静脉高压;儿童 Clinical features of different clinical forms of childhood congenital hepatic fibrosis WU Xin, DU Xiao-Rang, DING Jin-Fang, WU Meng-Jin, LUO Sheng-Qiang, FENG Xing-Zhong. Department of Traditional Chinese Medicine, Beijing Shijitan Hospital Affiliated to Capital Medical University, Beijing 100038, China (Feng X-Z, Email: jhwux2@163.com 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 (P0.05. 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 wit