Lung transplantation for treatment of infants with surfactant protein B deficiency

被引:115
作者
Hamvas, A
Nogee, LM
Mallory, GB
Spray, TL
Huddleston, CB
August, A
Dehner, LP
deMello, DE
Moxley, M
Nelson, R
Cole, FS
Colten, HR
机构
[1] WASHINGTON UNIV, SCH MED, DEPT SURG, ST LOUIS, MO 63110 USA
[2] WASHINGTON UNIV, SCH MED, EDWARD MALLINCKRODT DEPT PEDIAT, ST LOUIS, MO 63110 USA
[3] ST LOUIS UNIV, SCH MED, DEPT PATHOL, ST LOUIS, MO 63103 USA
[4] ST LOUIS UNIV, SCH MED, EA DOISY DEPT BIOCHEM & MOL BIOL, ST LOUIS, MO 63103 USA
[5] JOHNS HOPKINS UNIV, SCH MED, DEPT PEDIAT, BALTIMORE, MD 21205 USA
[6] UNIV S FLORIDA, SCH MED, DEPT PEDIAT, TAMPA, FL 33620 USA
[7] WASHINGTON UNIV, SCH MED, DEPT PATHOL, ST LOUIS, MO 63110 USA
关键词
D O I
10.1016/S0022-3476(97)70348-2
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Objective: To evaluate lung transplantation for treatment of surfactant protein B (SP-B) deficiency. Study design: We compared surfactant composition and function from pretransplantation and posttransplantation samples of bronchoalveolar lavage fluid, somatic and lung growth, neurodevelopmental progress, pulmonary function, and pulmonary immunohistology in 3 infants with SP-B deficiency who underwent bilateral lung transplantation at 2 months of age and 3 infants who underwent lung transplantation for other reasons. Results: Two years after transplantation, the 2 surviving infants with SP-B deficiency exhibited comparable somatic growth and cognitive development to the comparison infants. All infants had delays in gross motor development that improved with time. Both groups have exhibited normal gas exchange, lung growth, and pulmonary function. The SP-B-deficient infants have also exhibited normal SP-B expression and pulmonary surfactant function after lung transplantation. In two SP-B-deficient infants antibody to SP-B developed. No pathologic consequences of this antibody were identified. Conclusions: Apart from the development of anti-SP-B antibody, the outcomes for SP-B-deficient infants after lung transplantation are similar to those of infants who undergo lung transplantation for other reasons. Lung transplantation offers a successful interim therapy until gene replacement for this disease is available.
引用
收藏
页码:231 / 239
页数:9
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