Severe perinatal Marfan syndrome

The cardiovascular manifestations of the Marfan syndrome in older children and adults have been well described. Clinical, radiographic, and echocardiographic data regarding three patients with severe perinatal Marfan syndrome are described. Two of these patients had the syndrome at birth and died in...

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Bibliographic Details
Published in:Pediatrics (Evanston) 1989-07, Vol.84 (1), p.83-89
Main Authors: GROSS, D. M, ROBINSON, L. K, SMITH, L. T, GLASS, N, ROSENBERG, H, DUVIC, M
Format: Article
Language:English
Subjects:
Acute Disease
Biological and medical sciences
Biopsy
Cardiovascular Diseases - diagnosis
Cardiovascular Diseases - etiology
Cardiovascular Diseases - pathology
Echocardiography
Humans
Infant
Infant, Newborn
Lung - ultrastructure
Marfan Syndrome - complications
Marfan Syndrome - diagnosis
Marfan Syndrome - pathology
Medical sciences
Microscopy, Electron
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
Skin - ultrastructure
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Summary:The cardiovascular manifestations of the Marfan syndrome in older children and adults have been well described. Clinical, radiographic, and echocardiographic data regarding three patients with severe perinatal Marfan syndrome are described. Two of these patients had the syndrome at birth and died in infancy. The syndrome was diagnosed in the third patient at 6 months of age and the child is still alive at 3 years of age. The possible relationship among the Marfan syndrome, Ehlers-Danlos syndrome, and osteogenesis imperfecta is considered. Patients with Marfan syndrome and severe cardiorespiratory problems early in life tend to have a limited life expectancy.
ISSN:0031-4005
1098-4275
DOI:10.1542/peds.84.1.83