I. Create a clinical sample bank of neonates with lung disease to test hypotheses regarding
the pathogenesis of bronchopulmonary dysplasia (BPD).
II. Determine whether a developmental deficiency of surfactant protein B (SP-B) contributes
to the occurrence of respiratory distress and BPD in these patients.
III. Study metabolic abnormalities associated with inherited deficiency of SP-B in these
IV. Determine whether plasma nitrotyrosine levels, a marker of peroxynitrite mediated
oxidant stress, are elevated in premature infants who develop BPD.
V. Measure the temporal changes in critical components of the inflammatory process (cell
composition, inducible nitric oxide synthase, hyaluronan (HA), receptor for HA mediated
mobility, and selected cytokines) in bronchoalveolar lavage, blood, and urine samples
obtained from these patients, and to correlate these changes with their clinical course.
VI. Examine changes in the insulin-like growth factor axis that occur in the lungs of
infants with respiratory distress syndrome (RDS) and BPD.
VII. Determine the relationship between degradation of elastin and the clinical course of
VIII. Determine whether the normal fall in plasma endothelin-1 concentrations after birth
are delayed in infants with RDS and BPD.
Bronchoalveolar lavage and urine samples are obtained from patients on day of life 0, 1, 3,
7, 14, 21, and 28, and every 2 weeks thereafter until the infant is extubated. Serial blood
samples are obtained from patients on day of life 0 (cord blood if possible), 1, 3, 7, 14,
and 28, and prior to hospital discharge. Infants who require supplemental oxygen beyond 28
days of life will have 3 additional blood samples obtained at 6, 8, and 12 weeks of life.
Those infants with established bronchopulmonary dysplasia who are admitted to the hospital
at over 4 weeks of age have plasma samples obtained at the time of admission, and every 2
weeks thereafter for a maximum total of 5 samples.
Premature infants with gestational age of less than 33 weeks requiring mechanical
Term or near term infants, at least 33 weeks gestation, with severe respiratory distress,
requiring mechanical ventilation with an FiO2 greater than 0.5 and mean airway pressure
greater than 10
Infants over 4 weeks old with established bronchopulmonary dysplasia requiring mechanical