Abstract
It well-known that the spontaneous pheumothorax (SP) and apical bulls are diagnostic criteria of a Marfan syndrome. However the Marfan syndrome is a rare pathology, and the SP at tall and thin young men meets very often. For the purpose search of features of a Marfan syndrome has been surveyed 270 patients with a primary SP. Among patients men (n=248) prevailed, middle age of patients has made 24 years.
We have analysed major and minor criterions of the Marfan syndrome in the skeletal system, ocular system, cardiovascular system, skin and integument and dura. Sceletal major criterion met most often. Anterior chest deformity have been diagnosed for 14 patients, at 33 patients isnted reduced upper-segment to lower-segment ratio or arm span to height ratio more 1.05, at 62 patients were wrist and thumb signs. Scoliosis or spondylolisthesis have been taped at 45 patients. Medial displacement of the medial malleolus, causing pes planus were observed at 28 patients. Sceletal minor criteria and their combinations have been diagnosed for 112 patients. Dilatation of the ascending aorta and other are not diagnosed in one case. However such minor criteria as mitral valve prolapse has been diagnosed for 125 patients. Skin and integument criterion have been diagnosed for 38 patients. Ocular and dura criterion met seldom. The combination of criteria has not allowed to diagnose Marfan syndrome at one patient, however allows to speak about presence Marfanoid phenotype at the patients with a SP.The finding of a strong association of SP with Marfanoid phenotype suggests that in many patients may be a manifestation of a systemic abnormality of connective tissue.
- © 2011 ERS