Pulmonary neuroendocrine cell system in pediatric and adult lung disease.

In humans lungs affected by naturally occurring pulmonary disease, the pulmonary neuroendocrine cell system, which is normally arranged in a sparse but even distribution throughout the respiratory tract, increases in size. It is likely that the stimulus for this is pulmonary injury and that its purpose is the paracrine regulation of the restoration of pulmonary tissues to their normal state, an hypothesis supported by studies of animal lungs subjected to experimental injury as well as of the development of human and animal lungs in utero. Initially, this increase involves the development of interrupted rows of neuroendocrine cells. In the later stages, however, development of more disorderly intraepithelial aggregates can occur and the small, locally invasive neuroendocrine cell lesions known as tumourlets may occasionally result. Both of these latter structures often contain secretory products not found in the neuroendocrine cells of normal human lungs, probably indicating a derangement of what appears to be a fundamentally physiological response. It is likely that, in some circumstances, this disorderly change may contribute to pulmonary disease as well as being the result of it.