Clinical analysis of 8 cases of familial spontaneous pneumothorax and literatures review / 中华胸心血管外科杂志

ABSTRACT

Objective:To analyze the clinical characteristics and possible causes of familial spontaneous pneumothorax in combination with literatures.Methods:The detailed clinical information of 8 cases of FSP were retrospectively reviewed, and the clinical characteristics of FSP were analyzed combined with literatures.Results:Among 265 FSP patients in 76 families, the ratio of male to female is 1.55∶1, age of onset over 40 years old was 48.30%, 17.11%(13/76)of the cases occurred in the same generation, 67.10%(51/76)in two generations, 14.47%(11/76)in three generations and 1.31%(1/76)in four generations, and no intergenerational phenomenon was found. The proband had the mutation in the FLCN gene. The recurrence rates of observation, thoracentesis, chest tube drainage and operation were 21.62%(8/37), 48.39%(30/62), 38.89%(35/90)and 4.00%(4/100), respectively.Conclusion:Compared with sporadic cases of PSP, the clinical characteristics of FSP are as follows, the incidence of pneumothorax in man is higher than women, but women is significantly higher, and the age of pneumothorax is later, most of them are two generations, which may be related to heredity. And the effect of surgical treatment is the best.