Surfactant protein B deficiency: Antenatal diagnosis and prospective treatment with surfactant replacement

Aaron Hamvas, F. Sessions Cole, Daphne E. deMello, Michael Moxley, Jeffrey A. Whitsett, Harvey R. Colten, Lawrence Nogee

Research output: Contribution to journalArticle

Abstract

An infant with a family history of congenital alveolar proteinosis associated with surfactant protein B (SP-B) deficiency was identified when SP-B was not detected in amniotic fluid obtained at 37, 38, and 40 weeks of gestation. Surfactant replacement with commercially available preparations that contained SP-B was begun soon after delivery. Progressive respiratory failure developed despite continued surfactant replacement, corticosteroid therapy, and extracorporeal membrane oxygenation. The infant died at 54 days of age while awaiting lung transplantation. Surfactant extracted from amniotic fluid, bronchoalveolar lavage fluid, and lung tissue had no phosphatidylglycerol; surface tension was 24 dynes/cm (normal,

Original languageEnglish (US)
Pages (from-to)356-361
Number of pages6
JournalJournal of Pediatrics
Volume125
Issue number3
DOIs
Publication statusPublished - 1994

    Fingerprint

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this