小儿原发性免疫缺陷病的临床病理分析

本文10例共有特点是发病早，年龄均小于1岁；均以感染起病，病原菌种类有病毒、霉菌与细菌等。首先是临床疑诊有免疫缺陷病，5例生前免疫指标低下。死后，均经病理证实。10例中联合免疫缺陷6例；胸腺发育不良2例；B细胞免疫缺陷合并继发性胸腺萎缩退化2例。

Analysis of the Clinic and Pathology of Primary Immunodeficiency Disease in Children

This paper reports 10 cases of primary itnmunodeficiency disease. Their conunon characters are illness-attack early because the onset of age all younger than one year old. The symptoms of ten patients are infection due to virus, fungus, or bacteria.FirsL, clinical suspected diagnosis made by clinical symptoms and signs, then, immunic data showed lower than normal, finaly, all diagnosed by pathology. There are sever combined immunodeficiency disease 6, congenital thymus aplasia 2, and B cell immunodeficiency with secondary thymus atrophia-degeneration 2 in 10 cases.