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Recovery of baseline lung function after pulmonary exacerbation in children with primary ciliary dyskinesia
Author(s) -
Sunther Meera,
Bush Andrew,
Hogg Claire,
McCann Lauren,
Carr Siobhán B.
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.23479
Subject(s) - medicine , primary ciliary dyskinesia , exacerbation , spirometry , pulmonary function testing , clinical endpoint , vital capacity , cystic fibrosis , lung , anesthesia , clinical trial , asthma , lung function , bronchiectasis , diffusing capacity
Summary Rationale Spirometry in children with cystic fibrosis (CF) frequently fails to return to baseline after treatment for a pulmonary exacerbation. It is unclear whether the same is true for children with primary ciliary dyskinesia (PCD). Objectives To determine in children with PCD treated with intravenous antibiotics for a pulmonary exacerbation: (1) the proportion who recover to baseline forced expiratory volume at 1 sec (FEV 1 ) within 3 months after treatment and (2) to try to identify factors which are associated with failure to regain pre‐exacerbation FEV 1 . Methods Cohort study using the PCD database for children at the Royal Brompton Hospital, 2003–2013. We selected the first pulmonary exacerbation treated with intravenous antibiotics. The best FEV 1 within 3 months after treatment was compared to the best FEV 1 in the 12 months before treatment (baseline). Recovery to baseline was defined as any FEV 1 after treatment that was greater than or equal to 90% of the baseline FEV 1 . Results 32/150 children (21%) had at least one pulmonary exacerbation. 23/30 (77%) regained baseline spirometry within 3 months of treatment. There was no difference between responders and non‐responders in any baseline characteristics. Conclusions Around 25% of children with PCD fail to recover to baseline lung function within 3 months following treatment for a pulmonary exacerbation, similar to CF. Better treatment strategies are needed, and the results also suggest that prevention of exacerbations would be a useful end‐point in clinical trials. Pediatr Pulmonol. 2016;51:1362–1366. © 2016 Wiley Periodicals, Inc.