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Risk stratification model to detect early pulmonary disease in infants with cystic fibrosis diagnosed by newborn screening
Author(s) -
Britton Lacrecia J.,
Oates Gabriela R.,
Oster Robert A.,
Self Staci T.,
Troxler Robert B.,
Hoover Wynton C.,
Gutierrez Hector H.,
Harris William T.
Publication year - 2016
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.23536
Subject(s) - medicine , cystic fibrosis , newborn screening , pulmonary function testing , retrospective cohort study , pediatrics , vital capacity , socioeconomic status , lung , lung function , population , diffusing capacity , environmental health
Summary Objective The clinical benefit of newborn screening (NBS) for cystic fibrosis (CF) has been primarily nutritional, with less overt respiratory impact. Identification of risk factors for infant CF lung disease could facilitate targeted interventions to improve pulmonary outcomes. Methods This retrospective study evaluated socioeconomic information, clinical data, and results from routine infant pulmonary function testing (iPFT) of infants diagnosed with CF through NBS (N = 43) at a single CF center over a 4‐year period (2008–2012). A five‐item composite clinical score was developed and combined with socioeconomic indicators to facilitate identification of CF infants at increased risk of early‐onset respiratory impairment. Results Paternal education was positively associated with lung function ( P = 0.02). Clinical score <7 (on a scale of 0–10) predicted diminished pulmonary measure ( P < 0.005). Retrospective risk stratification by clinical score and paternal education identified CF infants at low, intermediate, or high risk of pulmonary disease. Forced expiratory volume (FEV 0.5 %, mean ± SD) averaged 115 ± 19% in the low‐risk group, 97 ± 17% in the intermediate‐risk group, and 90 ± 8% in the high‐risk group ( P < 0.005). Results were similar for mid‐expiratory flows (FEF 25–75 %). Multiple regression analysis confirmed the predictive value of this risk stratification model of CF infant pulmonary health. Conclusion We combined socioeconomic and clinical data to risk‐stratify CF infants for early‐onset lung disease as quantified by iPFT. Our model showed significant differences in infant pulmonary function across risk groups. The developed tool offers an easily available, inexpensive, and non‐invasive way to assess risk of respiratory decline in CF infants and identify those meriting targeted therapeutic attention. Pediatr Pulmonol. 2016;51:1168–1176. © 2016 Wiley Periodicals, Inc.