TY - JOUR
T1 - Long-term outcomes after infant lung transplantation for surfactant protein B deficiency related to other causes of respiratory failure
AU - Palomar, Lisanne M.
AU - Nogee, Lawrence M.
AU - Sweet, Stuart C.
AU - Huddleston, Charles B.
AU - Cole, F. Sessions
AU - Hamvas, Aaron
PY - 2006/10
Y1 - 2006/10
N2 - 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 (∼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.
AB - 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 (∼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.
UR - http://www.scopus.com/inward/record.url?scp=33749149259&partnerID=8YFLogxK
U2 - 10.1016/j.jpeds.2006.06.004
DO - 10.1016/j.jpeds.2006.06.004
M3 - Article
C2 - 17011330
AN - SCOPUS:33749149259
SN - 0022-3476
VL - 149
SP - 548
EP - 553
JO - Journal of Pediatrics
JF - Journal of Pediatrics
IS - 4
ER -