Abstract
To determine if the outcomes of lung transplantation for infants with surfactant protein-B (SP-B) deficiency are unique. From a prospective analysis to identify infants with genetic causes of surfactant deficiency, we identified 33 SP-B-deficient infants from 1993 to 2005, and, among those undergoing lung transplantation (n = 13), compared their survival, pulmonary function, and developmental progress with infants who underwent transplantation at <1 year of age for parenchymal lung disease (n="13)" or pulmonary vascular disease (n="11)." five-year survival rates ( approximately 50%, p=".3)" and causes of death were similar for all three groups once the infants underwent transplantation. however, significant pretransplantation mortality decreased 5-year survival from listing to approximately 30% (p=".17)." pulmonary function, development of bronchiolitis obliterans, and school readiness were similar among the three groups. we detected anti sp-b antibody in serum of 3 of 7 sp-b-deficient infants and none of 7 sp-b-sufficient infants but could not identify any associated adverse outcomes. long-term outcomes after infant lung transplantation for sp-b-deficient infants are similar to those of infants transplanted for other indications. these outcomes are important considerations in deciding to pursue lung transplantation for infants with disorders of alveolar homeostasis.>
