原发性免疫缺陷病临床发病情况回顾  

Overview of clinical occurrence of primary immunodeficiency disorders in children

作　　者：赵惠君[1] 陈同辛[1] 郝轶群[1] 周云芳[1] 应大明[1] 

机构地区：[1]上海交通大学医学院附属新华医院上海市儿科医学研究所免疫/肿瘤研究室,200092

出　　处：《中华儿科杂志》2006年第6期403-406,共4页Chinese Journal of Pediatrics

摘　　要：目的了解原发性免疫缺陷病的临床发病情况,加强临床医师对该类疾病的认识,促进今后防治工作的开展。方法回顾性分析1974年1月至2003年12月我院93例原发性免疫缺陷病患儿的临床资料。结果93例原发性免疫缺陷病患儿中,以抗体缺陷为主者占39.8%(37/93);以联合免疫缺陷者占22.6%(21/93);以T淋巴细胞缺陷为主者占14.0%(13/93);其他明确的免疫缺陷综合征者占12.9%(12/93);吞噬功能缺陷者占9.7%(9/93);补体缺陷者占1.1%(1/93)。从年度诊断的原发性免疫缺陷病病例数来看有逐年增多的趋势,1996年以后诊断例数呈现大幅度上升,共诊断50例。死亡病例共计16例,以联合免疫缺陷病为主。男∶女=3∶1。结论我国各类原发性免疫缺陷病均有发病,并且随着诊断技术水平的不断提高,临床诊断的病例日益增多,已成为临床,特别是儿内科的一组重要的疾病,应引起全国临床医师的注意。Objective More than one hundred primary immunodeficiency disorders have been discovered so far. But the incidence of these disorders in our country is still not clear, so we analyzed the clinical data of 93 children with primary immunodeficiency disorders seen in our hospital in recent 30 years to understand the occurrence of primary immunodeficiency disorders in children, to promote the clinicians to become familiar with these disorders, to improve the nationwide registry system and to establish the basis for the treatment and prevention in future. Methods To analyze the constituent ratio of the 93 children with primary immunodeficiency disorders seen in our hospital from 1974 to 2003, diagnostic and classification criteria were set by taking the proposal by International Union of Immunological Societies （IUIS）PID classification committee in 2003 into account. All the data were analyzed retrospectively. Results In the 93 children with primary immunodeficiency disorders, antibody deficiencies were the most frequent （39. 8% ） finding, followed by combined immunodeficiency, combined T- and B-cell disorders （22.6%）, and T lymphocytic deficiencies alone （ 14. 0% ）. Immunodeficiency with other major defects accounted for 12. 9%, phagocytic disorders 9. 7%, and complement deficiencies 1.1%. Thus, there seemed to be a tendency that the incidence increased with time. The incidence of these disorders has increased significantly as shown by 50 diagnosed cases in children with these disorders since 1996. Sixteen children died, with the highest mortality occurred with combined immunodeficiency. Seven children developed bronchiectasis. Two children suffered from persistent diarrhea while one of the two was complicated with persistent intestinal fistula. One child developed juvenile rheumatoid arthritis, another one with granulocytopenia and iridocyclitis, and the other with allergic purpura. The boys： girls ratio for all disorders was 3： 1. The age of onset ranged from 10 days to 37 years of age. Conclusio

关 键 词：免疫缺陷综合征 回顾性研究 

分 类 号：R593.2[医药卫生—内科学]