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.

NEHI is associated with substantial respiratory and extra-pulmonary morbidity. Failure to thrive may be associated with greater respiratory morbidity, though further studies are required to define this interaction. Determining the association of these comorbidities and respiratory course in NEHI may enable development of strategies to improve these modifiable factors and potentially pulmonary outcomes.

IntroductionTeaming Up for Asthma Control (TUAC) is a work force development intervention to improve asthma control among children by increasing the competency of school nurses and delivering guideline-based education. We hypothesized that the knowledge and skills of participating school nurses would improve and that this change would positively affect students’ asthma health and reduce health care utilization cost.MethodsAsthma education for school nurses was provided online in a pretest/posttest format or in instructor-led groups. Students with persistent asthma were identified by using a checklist. Expert evaluators obtained student participants’ preassessments/postassessments before and after the 3 asthma checkups by the school nurse, and the assessments were compared. Health care costs were assessed using Medicaid administrative claims data.ResultsA total of 54 school nurses and 178 students in Missouri participated in the TUAC evaluation from 2011 through 2014. Among school nurses who completed the online education (n = 42, 77.8%), knowledge scores significantly increased from pretest (49.1%) to posttest (90.7%, P < .001). Of school nurses who completed assessments on 3 children (n = 34), 91.2% met the ±6% equivalence for 1 or more assessments on forced expiratory volume in 1 second (FEV1) compared with the expert evaluator. At enrollment, 69.7% of students had “not well-controlled” or “very poorly controlled” asthma. Postintervention, FEV1 significantly improved (82.9% to 92.1% predicted), and self-reported impairment and tobacco smoke exposure significantly declined (P < .001). For TUAC students enrolled in Medicaid, there was an average 12-month health care cost difference (−$1,431) compared with controls.ConclusionSchool nurses effectively assessed asthma status, students’ outcomes improved, and health care utilization costs declined. This evaluation contributed to program improvements to further improve health outcomes among students with asthma.

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