Management and Long-Term Outcomes of Persistent Tachypnea of Infancy/Neuroendocrine Cell Hyperplasia of Infancy: A European Multicenter Retrospective Study.

Background Persistent tachypnea of infancy (PTI), also known as neuroendocrine cell hyperplasia of infancy (NEHI), represents one of the most common childhood interstitial lung diseases. Despite its frequency, standardized management protocol is lacking, and long-term outcome data remain limited.Research Question What treatment is used for patients with PTI/NEHI, how does clinical management vary across European countries, and what are the long-term outcomes in affected patients?Study Design And Methods This was a European, multicenter, retrospective, observational study. Clinical characteristics, therapeutic interventions, and long-term follow-up data were collected and analyzed. Treatment strategies were compared among countries that contributed at least 10 patients.Results A total of 378 children (63.5% male, 240/378) from 73 centers across 17 countries were enrolled, with a median age at diagnosis of 9 months (IQR, 6-13 months). Therapeutic interventions included oxygen supplementation (75.9%, 287/378), inhaled bronchodilators and/or inhaled glucocorticoids (62.4%, 236/378), systemic glucocorticoids (37.0%, 140/378), and nutritional support (33.8%, 128/378). Of the children who received oxygen therapy, 53.6% (154/287) were reported to have been weaned off, with a median age at weaning of 24 months (IQR, 16-36 months). Marked variability in treatment practices was observed across participating countries (p