PT  - JOURNAL ARTICLE
AU  - Turner, J A
AU  - Stanley, N N
TI  - Fragile lung in the Marfan syndrome.
AID  - 10.1136/thx.31.6.771
DP  - 1976 Dec 01
TA  - Thorax
PG  - 771--775
VI  - 31
IP  - 6
4099  - http://thorax.bmj.com/content/31/6/771.short
4100  - http://thorax.bmj.com/content/31/6/771.full
SO  - Thorax1976 Dec 01; 31
AB  - 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.