Neuroendocrine hyperplasia of infancy (NEHI) vs. diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH): What is the difference?

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Introduction: Neuroendocrine Hyperplasia of Infancy (NEHI) is a rare disease that belongs to the group of interstitial lung disease in children; characterised by hypoxemia, tachypnoea, and respiratory distress, often misdiagnosed as reactive airway disease or bronchiolitis. The diagnosis relies on histologic evaluation. We describe the clinical presentation and paraclinical characteristics of paediatric patients with NEHI.

Aim(s): A cross-sectional study and retrospective review of the electronic medical records, imaging, and polysomnographic study of children with NEHI.

Materials and methods: Ten children with NEHI were identified from 2013 to 2023, with females slightly more prevalent. The average age at consultation was 9.3 months (range 5 - 20). Key clinical features included low saturations, tachypnoea, and half-showing retractions. Chest CT scans revealed ground-glass opacities and bronchial thickening. Polysomnograms displayed basal desaturation and central apnoeas, averaging 73.8% for nadir oxygen saturation. Treatment emphasised inhaled steroids, leukotriene inhibitors, and azithromycin.

Conference:

Presenting Author:

Authors: González Devia D, Restrepo Gualteros S, Ortiz Pérez M, Fernández Hernández B,

Keywords: Neuroendocrine Hyperplasia of Infancy, Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia, child,