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- Title
Neuroendocrine Cell Hyperplasia of Infancy. Clinical Score and Comorbidities.
- Authors
Liptzin, Deborah R.; Pickett, Kaci; Brinton, John T.; Agarwal, Amit; Fishman, Martha P.; Casey, Alicia; Towe, Christopher T.; Taylor, Jane B.; Kurland, Geoffrey; Hagood, James S.; Wambach, Jennifer; Srivastava, Ruma; Al-Saleh, Hani; Dell, Sharon D.; Young, Lisa R.; Deterding, Robin R.
- Abstract
Rationale: Neuroendocrine cell hyperplasia of infancy (NEHI) is an important form of children's interstitial and diffuse lung disease for which the diagnostic strategy has evolved. The prevalence of comorbidities in NEHI that may influence treatment has not been previously assessed.Objectives: To evaluate a previously unpublished NEHI clinical score for assistance in diagnosis of NEHI and to assess comorbidities in NEHI.Methods: We performed a retrospective chart review of 199 deidentified patients with NEHI from 11 centers. Data were collected in a centralized Research Electronic Data Capture registry and we performed descriptive statistics.Results: The majority of patients with NEHI were male (66%). The sensitivity of the NEHI Clinical Score was 87% (95% confidence interval [CI], 0.82-0.91) for all patients from included centers and 93% (95% CI, 0.86-0.97) for those with complete scores (e.g., no missing data). Findings were similar when we limited the population to the 75 patients diagnosed by lung biopsy (87%; 95% CI, 0.77-0.93). Of those patients evaluated for comorbidities, 51% had gastroesophageal reflux, 35% had aspiration or were at risk for aspiration, and 17% had evidence of immune system abnormalities.Conclusions: The NEHI Clinical Score is a sensitive tool for clinically evaluating NEHI; however, its specificity has not yet been addressed. Clinicians should consider evaluating patients with NEHI for comorbidities, including gastroesophageal reflux, aspiration, and immune system abnormalities, because these can contribute to the child's clinical picture and may influence clinical course and treatment.
- Subjects
UNITED States; NEUROENDOCRINE cells; HYPERPLASIA; INTERSTITIAL lung diseases in children; COMORBIDITY; GASTROESOPHAGEAL reflux; RESEARCH; RESEARCH methodology; INTERSTITIAL lung diseases; RETROSPECTIVE studies; MEDICAL cooperation; EVALUATION research; SEVERITY of illness index; COMPARATIVE studies; CELLS; RESEARCH funding; COMPUTED tomography
- Publication
Annals of the American Thoracic Society, 2020, Vol 17, Issue 6, p724
- ISSN
2329-6933
- Publication type
journal article
- DOI
10.1513/AnnalsATS.201908-617OC