马凡综合征中的脆弱肺脏。
Fragile lung in the Marfan syndrome.
作者信息
Turner J A, Stanley N N
出版信息
Thorax. 1976 Dec;31(6):771-5. doi: 10.1136/thx.31.6.771.
Abstract
Two cases of the Marfan syndrome presented with spontaneous pneumothorax. Both had chest radiographs showing bilateral bullae in the upper lung zones and pulmonary function tests consistent with mild emphysema. There were dereases in forced expiratory flow rates at low lung volumes, carbon monoxide transfer factor, and lung elastic recoil. It is suggested that pneumothorax and bullous emphysema in this syndrome are caused by a weakness in the pulmonary connective tissue framework.
摘要
两例马方综合征患者出现自发性气胸。两人的胸部X光片均显示上肺区有双侧肺大疱，肺功能测试结果与轻度肺气肿相符。低肺容量时的用力呼气流速、一氧化碳弥散量和肺弹性回缩力均降低。提示该综合征中的气胸和大疱性肺气肿是由肺结缔组织框架薄弱所致。
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