OBJECTIVE: To determine if the outcomes of lung transplantation for infants with surfactant protein-B (SP-B) deficiency are unique. STUDY DESIGN: 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). RESULTS: 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. CONCLUSIONS: 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.
OBJECTIVE: To determine if the outcomes of lung transplantation for infants with surfactant protein-B (SP-B) deficiency are unique. STUDY DESIGN: 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). RESULTS: 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. CONCLUSIONS: 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.
Authors: Daniela M Tomazela; Bruce W Patterson; Elizabeth Hanson; Kimberly L Spence; Tiffany B Kanion; David H Salinger; Paolo Vicini; Hugh Barret; Hillary B Heins; F Sessions Cole; Aaron Hamvas; Michael J MacCoss Journal: Anal Chem Date: 2010-03-15 Impact factor: 6.986
Authors: Whitney B Eldridge; Qunyuan Zhang; Albert Faro; Stuart C Sweet; Pirooz Eghtesady; Aaron Hamvas; F Sessions Cole; Jennifer A Wambach Journal: J Pediatr Date: 2017-02-16 Impact factor: 4.406
Authors: Geoffrey Kurland; Robin R Deterding; James S Hagood; Lisa R Young; Alan S Brody; Robert G Castile; Sharon Dell; Leland L Fan; Aaron Hamvas; Bettina C Hilman; Claire Langston; Lawrence M Nogee; Gregory J Redding Journal: Am J Respir Crit Care Med Date: 2013-08-01 Impact factor: 21.405