Deficiency of Pulmonary Surfactant Protein B in Congenital Alveolar Proteinosis

Lawrence M. Nogee, Daphne E. Demello, Louis P. Dehner, Harvey R. Colten

Research output: Contribution to journalArticle

500 Scopus citations

Abstract

Congenital pulmonary alveolar proteinosis is an uncommon cause of respiratory failure in full-term newborns14. Although its histopathological appearance is similar to that of the alveolar proteinosis observed in older children and adults,5 the congenital form of the illness follows a different clinical course. All reported infants with congenital alveolar proteinosis have died within the first year of life despite maximal medical therapy. The incidence and cause of congenital alveolar proteinosis are unknown. Familial cases have been reported, and it has been speculated that the cause is an inborn error of surfactant metabolism4. In this report we describe…

Original languageEnglish
Pages (from-to)406-410
Number of pages5
JournalNew England Journal of Medicine
Volume328
Issue number6
DOIs
StatePublished - Feb 11 1993

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