Lung transplantation for treatment of infants with surfactant protein B deficiency☆,☆☆,★,★★
Section snippets
PATIENTS
The clinical presentations of three unrelated term female infants were similar to previous reports: respiratory distress with the need for mechanical ventilation at less than 24 hours of age (SP-B–deficient infants 1, 2, and 3, Table I)4, 5, 6, 11.
Empty Cell SP-B–deficient infants Comparison infants Empty Cell Infant 1 Infant 2 Infant 3 C1 * C2† C3† Age at transplantation (wk) 10 (first), 26 (second) 9 9 35 13 12 Duration of mechanical ventilation after transplantation (wk) 5, 6 1 5 2 3 3 Duration of
Pulmonary function testing and neurodevelopmental progress
To evaluate lung compliance and small airway patency, functional residual capacity, specific respiratory system compliance, and flow at FRC were measured with the Sensormedics model 2600 Pulmonary Cart (Sensormedics, Yorba Linda, Calif.). 16 Lung volume was estimated from serial standard posteroanterior chest radiographs as previously described. 17, 18 Neurodevelopmental progress was assessed with the Bayley Scales of Infant Development, 19 and gross motor function was assessed with the Peabody
Somatic growth, neurodevelopmental progress, lung growth, and pulmonary function testing
At follow-up at least 10 months after operation, two comparison infants and one infant with SP-B deficiency were at or above the 5th percentile for weight, whereas the other two infants were well below the 5th percentile for weight. All five infants were at or below the 10th percentile for length. Head circumference growth was progressing at or above the 5th percentile for all infants (Table II).
Empty Cell SP-B–deficient infants Comparison
DISCUSSION
Infants with SP-B deficiency can be successfully treated with lung transplantation. The outcomes at 2 years of age of two such infants suggest that somatic and lung growth and cognitive development are maintained despite severe respiratory failure in the pretransplantation period, pulmonary denervation, and immunosuppression. Motor and auditory development may be adversely affected, however, by long-term use of neuromuscular blockade in the pretransplantation interval or by chronic
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Cited by (0)
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From the Edward Mallinckrodt Department of Pediatrics, and the Departments of Surgery and Pathology, Washington University School of Medicine and St. Louis Children's Hospital, and the Department of Pathology and the E.A. Doisy Department of Biochemistry and Molecular Biology, St. Louis University School of Medicine, St. Louis, Missouri; the Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland; and the Department of Pediatrics, University of South Florida School of Medicine, Tampa, Florida.
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Supported in part by the American Lung Association of Eastern Missouri (A.H.), the American Lung Association (D.E.dM.), National Institute of Health grant No. HL-34748 (D.E.dM.), National Institutes of Health grant No. HL-37591 (H.R.C.), National Institutes of Health grant No. 1P20NS3256801 (F.S.C.), March of Dimes Basil O'Connor Award (L.M.N.), and March of Dimes (A.H., F.S.C.).
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Reprint requests: Aaron Hamvas, MD St. Louis Children's Hospital, Division of Newborn Medicine, One Children's Place, St. Louis, MO 63110.
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