Spontaneous Pneumothorax and Pulmonary Disease in the Marfan Syndrome

Case Studies1 June 1965Spontaneous Pneumothorax and Pulmonary Disease in the Marfan SyndromeReport of Two Cases and Review of the LiteratureEDWARD M. DWYER JR., M.D., FRANK TRONCALE, M.D.EDWARD M. DWYER JR., M.D.Search for more papers by this author, FRANK TRONCALE, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-62-6-1285 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptThe Marfan syndrome is a rare but well-defined hereditary disorder of connective tissue (1). Prominent features of the syndrome include abnormalities of the skeletal, cardiovascular, and ocular systems that are manifested as either a congenital anomaly (microphakia, coarctation of the aorta) or a degenerative lesion of elastic tissue (aortic dissection, ectopic lens, hernia). Lesser known areas of involvement are renal (2, 3), dermatologic (4), and pulmonary. The spectrum of pulmonary lesions in the Marfan syndrome includes congenital malformations, cystic disease, emphysema, spontaneous pneumothorax, and susceptibility to respiratory infections. In spite of several recent reports (5-7), the etiology, pathogenesis, and pathology...References1. MCKUSICK VA: Heritable Disorders of Connective Tissue, 2nd ed. C. V. Mosby & Co., St. Louis, 1960. Google Scholar2. LOUGHRIDGE LW: Renal abnormalities in the Marfan syndrome. Quart. J. Med. 28: 531, 1959. MedlineGoogle Scholar3. BOOTHLOUGHRIDGETURNER CCLWMD: Arachnodactyly with congenital lesions of the urinary tract. Brit. Med. J. 2: 80, 1957. CrossrefMedlineGoogle Scholar4. HABER H: Miescher's elastoma (elastoma intrapapillare perforans verruciforme). Brit. J. Derm. 71: 85, 1959. CrossrefMedlineGoogle Scholar5. FULEIHANSUHSHEPARD FJSKRH: Some aspects of pulmonary function in the Marfan syndrome. Bull. Hopkins Hosp. 113: 320, 1963. MedlineGoogle Scholar6. 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Mal. Coeur 51: 1105, 1958. MedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAuthors: EDWARD M. DWYERJR., M.D.; FRANK TRONCALE, M.D.Affiliations: New York, New YorkFrom the Department of Medicine, St. Luke's Hospital, New York, N. Y.Requests for reprints should be addressed to Edward M. Dwyer, Jr., M.D., Cardiovascular Laboratory, Presbyterian Hospital, 622 W. 168th Street, New York, N. Y. 10032. 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A proposito de una observacionCor Pulmonale Secondary to Severe Kyphoscoliosis in Marfan's SyndromeRecurrent spontaneous pneumothorax in the high-risk patientRECURRENT PNEUMOTHORAX IN A PATIENT WITH THE MARFAN SYNDROMEMISDIRECTED MONEY-RAISINGANTITRYPSIN DEFICIENCY IN LUNG DISEASE 1 June 1965Volume 62, Issue 6Page: 1285-1292KeywordsEmphysemaEtiologyHerniaHospital medicineLesionsMarfan syndromePathogenesisPulmonary diseasesUpper respiratory tract infectionsVentilators ePublished: 1 December 2008 Issue Published: 1 June 1965 PDF downloadLoading ...