Article
Pathophysiology and treatment of surfactant protein-B deficiency.
Biology of the neonate - 1 Jan 1995
Hamvas A, Nogee L M, deMello D E, Cole F S
Abstract excerpt
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...
Topics
- Genetic Therapy
- Humans
- Infant, Newborn
- Infant, Newborn, Diseases
- Lung Transplantation
- Mutation
- Proteolipids
- Pulmonary Surfactants
- Respiratory Insufficiency