Akademik Veri Yönetim Sistemi
Chest, 2026 (SCI-Expanded, Scopus)
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 of 378]) from 73 centers across 17 countries were enrolled, with a median age at diagnosis of 9 months (interquartile range [IQR], 6-13 months). Therapeutic interventions included oxygen supplementation (75.9% [287 of 378]); inhaled bronchodilators, inhaled glucocorticoids, or both (62.4% [236 of 378]); systemic glucocorticoids (37.0% [140 of 378]); and nutritional support (33.8% [128 of 378]). Of the children who received oxygen therapy, 53.6% (154 of 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 < .05). Longitudinal data were available for 48.9% of patients (185 of 378) with a median follow-up of 19 months (IQR, 16-57 months). The proportion of symptomatic children declined over time, with the most marked improvement observed at 4 years of age. Resolution of imaging and pulmonary function abnormalities also was reported; however, a subset of patients continued to demonstrate persistent hypoxemia, crackles, and exercise intolerance, as well as abnormal imaging and pulmonary function test findings into adolescence. Interpretation: Our results show that significant differences in treatment strategies for PTI/NEHI were observed across European countries, highlighting the need for evidence-based guidelines. Although long-term prognosis generally is favorable, residual symptoms remain in some patients, warranting continued follow-up.