Congenital pulmonary alveolar proteinosis is an uncommon causeof respiratory failure in full-term newborns1,2,3,4. Althoughits histopathological appearance is similar to that of the alveolarproteinosis observed in older children and adults,5 the congenitalform of the illness follows a different clinical course. Allreported infants with congenital alveolar proteinosis have diedwithin the first year of life despite maximal medical therapy.The incidence and cause of congenital alveolar proteinosis areunknown. Familial cases have been reported, and it has beenspeculated that the cause is an inborn error of surfactant metabolism4.
From the Department of Pediatrics, Division of Allergy and Pulmonary Medicine (L.M.N., H.R.C.), and the Department of Pathology (L.P.D.), Washington University School of Medicine; and the Department of Pathology, Cardinal Glennon Children's Hospital (D.E.D.) -- both in St. Louis.
Address reprint requests to Dr. Nogee at the Division of Neonatology, CMSC 210, Johns Hopkins Children's Center, 600 N. Wolfe St., Baltimore, MD 21287-3200.
References
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