Neuroendocrine cell hyperplasia of infancy

What is neuroendocrine cell hyperplasia of infancy (NEHI), and how does it present clinically and radiographically?

NEHI is a poorly understood entity with an increased number of neuroendocrine cells seen in the lung tissue at biopsy. Patients often present with persistent tachypnea and hypoxia. Radiographs are often nonspecific but may show hyperinflation with perihilar opacities. CT scans often show characteristic ground glass opacities in the right middle lobe and lingula or in a paraspinal distribution. Identifying these patterns and suggesting the diagnosis may eliminate the need for biopsy. Mosaic attenuation of the lungs due to air trapping can also be seen. The disease is usually self-limited and does not respond to corticosteroids.


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