Survival and Predictors of Death Among Primary Immunodeficient Patients: A Registry-Based Study
Survival and Predictors of Death Among Primary Immunodeficient Patients: A Registry-Based Study
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DOI：
10.1007/s10875-011-9636-1
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发表时间：
2012-06-01
影响因子：
9.1
通讯作者：
Moussa, Mohamed A. A.
中科院分区：
文献类型：
--
作者：
Al-Herz, Waleed;Moussa, Mohamed A. A.
方法收集2004年1月至2011年7月科威特国家原发免疫缺陷疾病登记处收集的176例患者资料，男98例，女78例。结果报告病例分布为T、B细胞免疫缺陷(30.1%)，以抗体免疫缺陷为主(19.9%)，其他明确免疫缺陷(25%)，免疫紊乱疾病(14.8%)，先天性吞噬细胞缺陷(14.8%)，功能或两者兼而有之(6.25%)和补充不足(4.0%)。在总共619.1个危险病人年中，48名病人死亡(死亡率为每千人年77.53%)。研究队列的总存活率为72.7%(男性为72.4%，女性为73.1%)。最常见的死亡原因是败血症(46%)，其次是肺炎(29%)。患者在出现症状后存活2年、4年和6年的概率分别为76%、73%和69%。被发现可以预测死亡的变量是父母的血缘关系、败血症、腺病毒和巨细胞病毒感染、不能茁壮成长、PID类别和发病年龄。
Purposes The aims of this study were to investigate survival among patients with primary immunodeficiency disorders (PID) in Kuwait and to determine whether certain variables were associated with increased risk of death.Methods The data of 176 patients (98 males and 78 females) were extracted from the Kuwait National Primary Immunodeficiency Disorders Registry and the observation period was from January 2004 to July 2011.Results The distribution of the reported patients was combined T- and B-cell immunodeficiencies (30.1%), predominantly antibody immunodeficiency (19.9%), other well-defined immunodeficiencies (25%), diseases of immune dysregulation (14.8%), congenital defects of phagocyte number, function or both (6.25%), and complement deficiencies (4.0%). In a total of 619.1 patient-years at risk, 48 patients died (mortality incidence rate 77.53 per 1,000 person-years). The overall survival in the studied cohort was 72.7% (72.4% for males and 73.1% for females). The most common cause of death was sepsis (46%) followed by pneumonia (29%). The probabilities that a patient survived 2, 4, and 6 years after onset of symptoms were 76%, 73%, and 69%, respectively. The variables that were found to be predictors for death are parental consanguinity, sepsis, adenovirus and CMV infections, failure to thrive, PID category, and onset age