Background Asthma and obstructive sleep apnea (OSA) in children share multiple epidemiological risk factors and the prevalence of snoring is higher in asthmatic children, suggesting that the latter may be at increased risk for OSA. Since both asthma and OSA are inflammatory disorders, we hypothesized that polysomnographically-demonstrated OSA would be more frequent among poorly controlled asthmatics (PCA), and that treatment of OSA, if present, would ameliorate the frequency of acute asthmatic exacerbations (AAE). Methods Children with PCA were referred for an overnight sleep study, and adenotonsillectomy (T&A) was performed if OSA was present. Frequency of asthma symptoms and exacerbations were compared. Results 92 PCA children, ages 3-10 years, with a mean frequency of AAE of 3.4±0.4/year were prospectively referred for a sleep study. OSA (i.e., AHI>5/hrTST) was present in 58 patients (63.0%; OR: 40.9, 12.9-144.1, p<0.000001 compared to the prevalence of OSA in a non-asthmatic population). Information at 1-year follow-up was available for 35 PCA children after tonsillectomy and adenoidectomy (T&A). The annual frequency of AAE, rescue inhaled use, and asthma symptoms in this sub-group decreased compared to no changes in the group without OSA. Conclusions The prevalence of OSA is markedly increased among PCA children and treatment of OSA appears to be associated with substantial improvements in the severity of the underlying asthmatic condition.

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Rationale: Neuroendocrine cell hyperplasia of infancy (NEHI) is a diffuse lung disease that presents in infancy and improves during childhood. Long-term outcomes have not previously been described. In one familial cohort, we have reported that NEHI is associated with a heterozygous variant of NKX2.1/TTF1. Objectives: Our objective was to determine whether pulmonary abnormalities persist in adults with NEHI, to aid in elucidating the natural history of this disease.Methods: Four adult relatives with heterozygous NKX2.1 mutation and with clinical histories compatible with NEHI enrolled in a prospective study that included questionnaires, pulmonary function tests, and chest computed tomography scans. Measurements and Main Results:Mild radiologic abnormalities including mosaicism were seen in all four cases. Three individuals had obstruction on pulmonary function tests, two had marked air trapping, and three had symptomatic impairments with exercise intolerance.Conclusions: Although clinical improvement occurs over time, NEHI may result in lifelong pulmonary abnormalities in some cases. Further studies are required to better describe the natural history of this disease and would be facilitated by additional delineation of genetic mechanisms to enable improved case identification.