Abstract Case Report A 6-month-old, fully vaccinated female presented to the emergency department with tachypnea and increased work of breathing and was found to be hypoxemic with oxygen saturation of 88% and respiratory rate of 80 breaths/minute. Physical exam was remarkable for persistent nasal flaring, end-expiratory grunting, inter- and subcostal retractions, and mildly diminished breath sounds bilaterally. History was notable for poor growth (1st percentile for weight), intermittent tachypnea, and a brief neonatal intensive care stay at birth for respiratory distress despite an uncomplicated term delivery at 38 weeks gestation. On presentation, she was treated with albuterol and ipratropium without change in respiratory status. Respiratory pathogen panel was positive for rhino/enterovirus and chest radiograph demonstrated hyperinflation and symmetric perihilar opacities bilaterally. She was placed on low-flow nasal cannula of 0.5 L/min and was admitted for presumed viral bronchiolitis. During hospitalization, work-up revealed aspiration with thin liquids on videofluoroscopic swallow study. Echocardiogram showed a structurally normal heart with normal biventricular function and no evidence of pulmonary hypertension. Despite several weeks of hospitalization, she was unable to wean from supplemental oxygen, exceeding the expected course for viral bronchiolitis. Chest computed tomography (CT) demonstrated bilateral patchy consolidations and ground-glass opacities. Pediatric pulmonology was consulted, and a presumptive diagnosis of neuroendocrine hyperplasia of infancy (NEHI) was made. She was discharged on home oxygen therapy with plans to follow up with pediatric pulmonology for a possible lung biopsy and airway evaluation. At nearly 2 years of age, the patient continues to require continuous supplemental oxygen; however, her growth has improved (30th percentile for weight), and developmental milestones are appropriate. Discussion NEHI is a rare pediatric interstitial lung disease characterized by an abnormal proliferation of pulmonary neuroendocrine cells in the distal airways, resulting in persistent tachypnea, hypoxemia, and retractions. NEHI typically presents during early infancy and may lead to prolonged oxygen requirements into early childhood. This case emphasizes the importance of considering NEHI in infants with persistent tachypnea, failure to thrive, and inability to wean oxygen. Characteristic chest CT findings, including ground-glass opacities, can aid in diagnosis. The etiology of NEHI remains largely unknown, and no therapies have been proven to reduce oxygen dependence. Early recognition can guide appropriate evaluation and long-term management. This abstract is funded by: None
Naron et al. (Fri,) studied this question.