Objectives: Neuroendocrine cell hyperplasia of infancy (NEHI) is a children's interstitial and diffuse lung disease of unknown etiology that presents in infancy with characteristic findings of tachypnea, retractions, crackles, and hypoxemia. At the present, the mainstay of treatment is oxygen supplementation to normalize oxygen saturations and decrease work of breathing. There are characteristic pulmonary function, radiographic, and histologic findings, but polysomnography (PSG) data has not been reported. We sought to report PSG data and implications for management and treatment of NEHI patients.
Methods: A retrospective chart review was performed under a Colorado Institutional Review Board approved protocol for which consent was waived. Informatics for Integrating Biology and the Bedside was used to query the electronic medical record at Children's Hospital Colorado for patients with both a diagnosis of NEHI and a PSG. PSG was performed for clinical reasons. Routine sleep quality and respiratory parameters were recorded and analyzed.
Results: Of our 77 patients with NEHI, 14 (19%) children underwent PSG during the study period. Eight children met criteria for OSA and three met criteria for CSA. Ten patients had low oxygen saturations during a study, six had low sleep efficiency, and three had periodic limb movement disorder.
Conclusions: Patients with NEHI may have sleep related breathing disorders that contribute to disrupted sleep, including obstructive and central sleep apnea, hypoxemia, decreased sleep efficiency, and increased periodic limb movement disorder. PSG should be considered as part of NEHI management, as it may lead to recognition of clinically significant sleep-disordered breathing.
Keywords: interstitial lung disease; sleep medicine.
© 2018 Wiley Periodicals, Inc.