Connective Tissue in the Lung: Lessons from the Marfan Syndrome
Authors: REED E. PYERITZ
Journal: Annals of Internal Medicine (1985)

Editorials1 August 1985Connective Tissue in the Lung: Lessons from the Marfan SyndromeREED E. PYERITZ, M.D., Ph.D.REED E. PYERITZ, M.D., Ph.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-103-2-289 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptConnective tissue pervades all organs and participates in vastly different functions depending, in part, on the type and organization of its biochemical components. The pulmonary system is an instructive example. Over 25% of the dry weight of the lung is connective tissue, including both fibrous elements (collagen and elastin) and ground substance (proteoglycans). Attempts to understand fully the physiologic role of connective tissue in the lung have confronted the theoretic and practical obstacles that have confounded work with other organs. The biochemical components are complex molecules, and their individual characterization is incomplete (1). Moreover, the fibrous and proteoglycan components are...References1. PIEZ K and REDDI A eds. Extracellular Matrix Biochemistry. New York: Elsevier; 1984. Google Scholar2. TRELSTAD R ed. The Role of Extracellular Matrix in Development. New York: Alan R. Liss; 1984. Google Scholar3. HANCE A and CRYSTAL R. The connective tissue of lung. Am Rev Respir Dis. 1975;112:657-711. MedlineGoogle Scholar4. PROCKOP D and KIVIRIKKO K. Heritable diseases of collagen. N Engl J Med. 1984;311:376-86. CrossrefMedlineGoogle Scholar5. PYERITZ R and MCKUSICK V. Basic defect(s) in the Marfan syndrome [Editorial]. N Engl J Med. 1981;305:1011-2. CrossrefMedlineGoogle Scholar6. TSIPOURAS P, BORRESEN A, DICKSON L, BERG K, PROCKOP D, and RAMIREZ F. Molecular heterogeneity in the mild autosomal dominant forms of osteogenesis imperfecta. Am J Hum Genet. 1984;36:1172-9. MedlineGoogle Scholar7. PYERITZ R and MCKUSICK V. The Marfan syndrome: diagnosis and managment. N Engl J Med. 1979;300:772-7. CrossrefMedlineGoogle Scholar8. HALL J, PYERITZ R, DUDGEON D, and HALLER J. Pneumothorax in the Marfan syndrome: prevalence and therapy. Ann Thorac Surg. 1984;37:500-4. CrossrefMedlineGoogle Scholar9. WOOD J, BELLAMY D, CHILD A, and CITRON K. Pulmonary disease in patients with Marfan syndrome. Thorax. 1984;39:780-4. CrossrefMedlineGoogle Scholar10. VERGHESE C. Anaesthesia in Marfan's syndrome. Anaesthesia. 1984;39:917-22. CrossrefMedlineGoogle Scholar11. PYERITZ R, GOTT V, and MCDONALD G. Surgical repair of the Marfan aorta: technique, indications and complications. Johns Hopkins Med J. 1982;151:71-82. MedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAuthors: REED E. PYERITZ, M.D., Ph.D.Affiliations: The Johns Hopkins University School of Medicine Baltimore, Maryland PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byStandardization and validation of a novel and simple method to assess lumbar dural sac sizeSafety and cardiovascular behavior during pulmonary function in patients with Marfan syndromePulmonary Involvement in Miscellaneous Connective Tissue DiseasesA catalogue of multiple congenital anomaly syndromesMarfan syndrome: a diagnostic dilemmaCystic lung in Marfan's syndrome.Pleiotropy revisited: Molecular explanations of a classic conceptMarfan syndrome: A reviewLens Subluxation and Tall StaturePulmonary Function in the Marfan SyndromeScandinavian Journal of Thoracic and Cardiovascular Surgery Supplementum 37 1 August 1985Volume 103, Issue 2Page: 289-290KeywordsConnective tissueLungsMarfan syndrome ePublished: 1 December 2008 Issue Published: 1 August 1985 PDF downloadLoading ...