Abstract
A premature infant with arachnodactyly and congenital cyanotic heart disease had severe pulmonary emphysema, a little-known manifestation of Marfan syndrome. The possible etiologies of emphysema in this syndrome are discussed.
References
McKusick VA (1972) The Marfan syndrome. In: McKusick VA (ed) Heritable disorders of connective tissue, 4th edn. CV Mosby St. Louis 61–223
Oswald N, Parkinson T (1949) Honeycomb lungs. Quart J Med 18: 1
Lipton RA, Greenwald RA, Seriff NS (1971) Pneumothorax and bilateral honeycombed lung in Marfan syndrome. Am Rev Respir Dis 104: 924
Dwyer EM, Troncale F (1965) Spontaneous pneumothorax and pulmonary disease in the Marfan syndrome. Ann Intern Med 62: 1285
Bolande RP, Tucker AS (1964) Pulmonary emphysema and other cardiorespiratory lesions as part of the Marfan abiotrophy. Pediatrics 33: 356
Wood JR, Bellamy D, Child AH, Citron KM (1984) Pulmonary disease in patients with Marfan syndrome. Thorax 39: 780
Phornphutkul C, Rosenthal A, Nadas AS (1973) Cardiac manifestations of Marfan's syndrome in infancy and childhood. Circulation 47: 587
Heldrich F Jr, Wright CE (1967) Marfan's syndrome: Diagnosis in the neonate. Am J Dis Child 116: 419
Rados A (1942) Marfan's syndrome: (arachnodactyly coupled with dislocation of the lens). Arch Ophthalmol (Chicago) 27: 477
Nieman N, Rauber G, Marchal M, Vidailhet M, Fall M (1968) Maladie de Marfan chez un nouveau-né avec atteintes polyviscerales. Ann Pediatr 15: 619
Reye DK, Bale PM (1973) Elastic tissue in pulmonary emphysemia in Marfan syndrome. Arch Pathol 96: 427
Sayers CP, Goltz RW, Mottag J (1975) Pulmonary elastic tissue in generalized elastolysis (cutis laxa) and Marfan's syndrome. A light and electron microscopic study. J Invest Dermatol 65: 451
Boucek RJ, Noble NL, Gunja-Smith Z, Butter WT (1981) The Marfan syndrome: a deficiency in chemically stable collagen cross-links. New Engl J Med 305: 988
Prockap DJ, Kivirikko KI (1984) Heritable diseases of collagen. New Engl J Med 311: 376
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Day, D.L., Burke, B.A. Pulmonary emphysema in a neonate with Marfan syndrome. Pediatr Radiol 16, 518–521 (1986). https://doi.org/10.1007/BF02387973
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DOI: https://doi.org/10.1007/BF02387973