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Pathophysiology and treatment of surfactant protein-B deficiency.

Authors
  • Hamvas, A
  • Nogee, L M
  • deMello, D E
  • Cole, F S
Type
Published Article
Journal
Biology of the neonate
Publication Date
Jan 01, 1995
Volume
67 Suppl 1
Pages
18–31
Identifiers
PMID: 7647155
Source
Medline
License
Unknown

Abstract

Surfactant protein B (SP-B) deficiency is an inherited disease of full-term newborn infants which leads to lethal respiratory failure within the first year of life. Genetic analysis of affected infants has permitted identification of a mutation in the SP-B gene found in several unrelated kindreds which disrupts pulmonary surfactant composition and function. Lung transplantation has resulted in reconstitution of pulmonary surfactant function and long-term survival. SP-B deficiency represents the first opportunity to link physiologic characteristics of respiratory failure in infancy with specific molecular and cellular defects. This linkage will facilitate development of novel strategies for the treatment of neonatal respiratory diseases.

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