Congenital > Neuroendocrine hyperplasia of infancy

Neuroendocrine Hyperplasia of Infancy

Clinical:

In neuroendocrine hyperplasia of infancy, neuroendocrine cells are more numerous than normal cells throughout the airway epithelium [1]. NEHI insidiously appears in the first year of life and typically manifests with tachypnea, retractions, hypoxemia, and failure to thrive [1,2].

X-ray:

The CXR may be normal or may reveal hyperinflation with variable increased perihilar opacity [2].

HRCT appears to be the most reliable non-invasive imaging test [2]. Multi-lobar ground-glass opacity predominantly involving the right middle lobe and lingula, as well as a mosaic pattern of air-trapping are typical findings on HRCT with a reported sensitivity of 78-83 % and specificity of 100% [1,2]. However, the diagnosis cannot be completely excluded with CT as up to 22% of cases may not be identified [2].

REFERENCES:

(1) Radiographics 2017; Semple TR, et al. Interstitial lung disease in children made easier...well, almost. 37: 1679-1703

(2) Ital J Pediatr 2016; Caimmi S, et al. Neuroendocrine cell hyperplasia of infancy: an unusual cause of hypoxemia in children. 42: 84

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