RESUMEN
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.
Asunto(s)
Trasplante de Pulmón , Proteolípidos , Surfactantes Pulmonares/deficiencia , Síndrome de Dificultad Respiratoria del Recién Nacido/cirugía , Anticuerpos/sangre , Líquido del Lavado Bronquioalveolar/química , Femenino , Humanos , Terapia de Inmunosupresión/métodos , Lactante , Recién Nacido , Pulmón/patología , Pulmón/fisiopatología , Trasplante de Pulmón/inmunología , Trasplante de Pulmón/patología , Trasplante de Pulmón/fisiología , Complicaciones Posoperatorias/epidemiología , Proteolípidos/análisis , Proteolípidos/inmunología , Surfactantes Pulmonares/análisis , Surfactantes Pulmonares/inmunología , Síndrome de Dificultad Respiratoria del Recién Nacido/inmunología , Síndrome de Dificultad Respiratoria del Recién Nacido/patología , Sobrevivientes , Resultado del TratamientoRESUMEN
OBJECTIVE: To characterize the evaluation and clinical course of children with nonpenetrating injury to the heart. METHODS: We reviewed the medical records for children admitted to St. Louis Children's Hospital between the years 1987 to 1992 with traumatic cardiac injury. Patients with penetrating trauma were excluded; eight children, ages 4 to 13 years, were the study subjects. Chest x-ray studies, electrocardiograms, and serum creatine kinase values were obtained on admission. Two-dimensional echocardiography was performed when indicated by unexplained hemodynamic instability or abnormal radiographic findings. RESULTS: All children with nonpenetrating cardiac trauma were involved in a motor vehicle accident. The principal cardiac diagnoses were ventricular septal defect (1), mitral regurgitation (1), pericardial effusion (2), contusion (3), and arrhythmia (1). Multisystem injury was present in each case, but cardiac injury was not suspected at the time of admission in seven of the eight patients. The hemodynamic status of four children was compromised 12 to 48 hours after admission; echocardiography was diagnostic in each instance, but the electrocardiogram and creatine kinase values were nonspecific. Two patients eventually required cardiac surgery. CONCLUSIONS: Recognition of blunt cardiac trauma in children may be confounded by associated multisystem injury and the delayed onset of clinical manifestations. Echocardiography is a sensitive diagnostic tool for hemodynamically significant disease, and should be performed promptly when patients have unexplained hypotension or diminished peripheral perfusion.