家族性自发性气胸临床特点分析

Clinical features analysis of familial spontaneous pneumothorax

目的 总结分析家族性自发性气胸（eamilial spontaneous pneumothorax,FSP）病例的临床特点.方法 回顾2001年4月至2013年3月诊治的21个家系,65例家族性自发性气胸患者的临床资料,对比既往文献,分析家族性自发性气胸的临床特点.结果 单一家系中平均患病3.1例（2～6）;同代患病者19.0％,两代患病者61.9％,三代患病者19.0％;患者男女比1.4∶1,气胸初次发病平均年龄（36.2±12.2）岁,体质量指数（BMI）男24.0±2.6,女22.6±3.0,双侧气胸者32.7％;患者经非手术治疗的复发率为50.0％.结论 与散发自发性气胸相比,家族性自发性气胸的临床特点有：男女发病率更接近,气胸初发年龄偏大,瘦高体型者较少,双侧气胸者更多,合并多发肺大疱者多,经非手术治疗的复发率更高.

Objective To summarize and analyze the clinical features of familial spontaneous pneumothorax.Methods During April 2001 to March 2013,the clinical data of 65 familial spontaneous pneumothorax from 21 families were retrospected and summarized.Contrasting with previous literature,the clinical features of familial spontaneous pneumothorax were analyzed.Results Patients in one family vary from 2 to 6,average 3.1.Families in which patients distribute in one generation,two generations and three generations were 19.0％,61.9％ and 19.0％ respectively.Male/female ratio of patients was.Mean onset age was 36.2 ± 12.2 ; Body mass index （BMI） of male and female patients were 24.0 ± 2.6 and 22.6 ± 3.0.32.7％ patients suffered bilateral pneumothorax.The recurrence rate after non-operative treatment was 50.0％.Conclusion Compared with sporadic spontaneous pneumothorax,Familial spontaneous pneumothorax has the following features：The incidence in man and woman was more similar; The onset age was older; Lanky body is less common; More were bilateral pneumothorax; Multiple pulmonary bullae are more common; Patients with non-surgical treatment have a higher recurrence rate.