- 期刊
Familial Spontaneous Pneumothorax-Report of Seven Cases in Two Families
家族性自發性氣胸
摘要
自發性氣胸常發生於瘦高的年輕人，尤其男性居多，一般是因為肺尖氣皰破裂而產生氣胸。但家族性自發性氣胸則不多見，世界上報告的病例只有59個家族，共135例。其產生機轉不明，但可能和遺傳有關。高醫胸腔外科自1981年來經歷了2個家族，7個病例，總共發了13次的自發性氣胸。因為顯然地有家族性發病的傾向，引起我們的注。1980年Sharpe等人提出HLA haplotype A2B40及α1-antitrypsin血中濃度。結果在所有病例，並未發現HLA A2B40的haplo-type，也沒有α1-antitrypsin缺乏的情形。因此我們認為HLA haplotype A2B40, α1-antitrypsin和家族性自發性氣胸並無特殊關係。特提出報告。
關鍵字
無資料並列摘要
Spontaneous pneumothorax usually occurs sporadically in healthy young adults, who tend to be tall and thin. The usual cause is rupture of small subpleu-ral bullae found in the apex of the upper lobe. Familial spontaneous pneumothorax is a rare condition. With a review of the English literature from 1921 to 1991, only 59 families with 135 cases have been reported. A genetic background has been suggested, but the actual mode of inheritance is still unknown. Relationships between the HLA haplotype A2B40, the alpha –antitrypsin phenotype and the occurrence of spontaneous pneu-mothorax have been proposed, but are still controversial. We present 7 patients in 2 families with 13 episodes of spontaneous pneu-mothorax. Neither congenital defect, alpha –antitrypsin deficiency nor the de-scribed A2B40 HLA haplotype could be found in our patients. We propose that there are no such factors existed in these patients. Further study is required to determine if any genetic marker is linked to familial spontaneous pneumothorax.
延伸閱讀
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