Work

Deficiency of Pulmonary Surfactant Protein B in Congenital Alveolar Proteinosis

Year: 1993

Type: article

Abstract: Congenital pulmonary alveolar proteinosis is an uncommon cause of respiratory failure in full-term newborns1–4. 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 . . .

Source: New England Journal of Medicine

DOI: 10.1056/nejm199302113280606

Authors: Lawrence M. Nogee, Daphne E. deMello, Louis P. Dehner, Harvey R. Colten

Institution: Washington University in St. Louis

Language: English

Topic: Neonatal Respiratory Health Research

Subfield: Pulmonary and Respiratory Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: bronze

Funder: National Heart, Lung, and Blood Institute