Neuroendocrine cell hyperplasia of infancy (NEHI) is a rare interstitial lung disease of childhood that typically presents in the first year of life.
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Terminology
Neuroendocrine cell hyperplasia of infancy was previously reported as persistent tachypnea of infancy until the disease was found to be associated with an increased number of neuroendocrine cells on histopathology of lung biopsies.
Epidemiology
NEHI typically occurs in the first year of life. Its current prevalence and incidence are not known but may be underreported as it is commonly misdiagnosed with other types of lung diseases. Etiology is unknown, but genetic factors are likely involved.
Clinical presentation
Infants typically present in respiratory distress with tachypnea, chest wall retractions, hypoxia, crepitations on auscultation of the chest, and failure to thrive.
Pathology
The gold standard for diagnosis of neuroendocrine cell hyperplasia of infancy is demonstrating an increase in neuroendocrine cells with an otherwise normal lung architecture on tissue diagnosis. Standard staining will demonstrate essentially normal lung histology but shows an increase in Bombesin-positive cells with specific staining (indicating increased neuroendocrine cells). Neuroendocrine cell prominence appears to be related with disease severity.
Radiographic features
Reports from current research and case series suggests that neuroendocrine cell hyperplasia of infancy may have consistent radiographic features.
Plain radiographs
Chest radiographs are non-specific but commonly show hyperinflation.
CT
The most common features are ground glass opacification (GGO), most commonly affecting the right middle lobe and lingula, followed by a mosaic pattern of air trapping often involving multiple lobes. Other findings are less specific and include consolidation, hyperinflation, linear and reticular opacities, and bronchial wall thickening.
Treatment and prognosis
Treatment is generally supportive with no current consensus, but consists of:
- supplemental oxygen if hypoxic
- adequate nutrition
- steroids
- treatment of overlapping infections
- immunization
Long term prognosis of neuroendocrine cell hyperplasia of infancy is generally good with most patients improving over time, however some patients may develop persistent lung disease with airway obstruction and recurrent respiratory infections.
Differential diagnosis
- infective lung diseases
- cystic fibrosis
- other childhood interstitial lung diseases
- pulmonary hypoplasia
- chronic neonatal lung disease of prematurity
- surfactant dysfunction disorders
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