Fragile lung in the Marfan syndrome. Issue 6 (December 1976)

• Record Type: Journal Article
• Authors: Turner, J A; Stanley, N N
• Is Part Of: Thorax. Volume 31:Issue 6(1976)
• Page Start: 771
• Page End: 775
• Publication Date: 1976-12
• Subjects: Chest -- Diseases -- Periodicals; Thorax; Chest -- Diseases; Periodicals; Periodicals; 617.54
• DOI: 10.1136/thx.31.6.771
• Languages: English
• ISSNs: 0040-6376

Abstract

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.