HYPERPLASIA OF PULMONARY NEUROENDOCRINE CELLS IN A CASE OF CHILDHOOD PULMONARY-EMPHYSEMA

Pulmonary emphysema is very uncommon in children in the first decade o f life. The few cases documented in the literature were all due to alp ha(1)-antitrypsin deficiency. We present the case of a 6-year-old whit e boy with chronic cough and dyspnea on exertion. Lung biopsy showed p anacinar type emphysema with patent airways and diffuse hyperplasia of pulmonary neuroendocrine cells revealed after immunostaining for bomb esin, a peptide produced by these cells. We speculate that idiopathic diffuse hyperplasia of bombesin-producing pulmonary neuroendocrine cel ls may contribute to the pathogenesis of unusual COPD in childhood.