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P102 Recovery Of Baseline Lung Function After A Pulmonary Exacerbation In Children With Primary Ciliary Dyskinesia (pcd)
  1. M Sunther,
  2. S Carr,
  3. C Hogg,
  4. A Bush
  1. Royal Brompton Hospital, London, UK


Rationale Spirometry in children with cystic fibrosis (CF) frequently fails to return to baseline after treatment for a pulmonary exacerbation [Am J Respir Crit Care Med 2010; 182: 627–32]. It is unclear however how often lung function returns to previous baseline levels after treatment of a pulmonary exacerbation with intravenous antibiotics in children with PCD.

Objectives To determine in children with PCD: (1) the proportion treated for a pulmonary exacerbation who recover to baseline FEV1 within 3 months and at 12 months and (2) to try to identify factors which are associated with failure to recover spirometry.

Methods Cohort study using the PCD database for children at the Royal Brompton Hospital from 2003 to 2013. We selected the first clinically diagnosed pulmonary exacerbation treated with intravenous antibiotics. The best FEV1 in the 3 months after treatment and at 12 months was compared to the best FEV1 in the 12 months before treatment (baseline). Recovery to baseline was defined as any FEV1 after treatment that was greater than or equal to 90% of the baseline FEV1.

Results Of the 30 children treated for pulmonary exacerbations, 77% recovered to baseline lung function within 3 months and 73% at 12 months. There were no significant differences between the responders and non-responders in terms of age, sex, ethnicity, BMI, baseline FEV1, persistent sputum infection or use of antibiotic prophylaxis or mucolytic agent (Table).

Conclusions Similar to findings in CF, around 25% PCD patients fail to recover to baseline lung function after treatment of a pulmonary exacerbation with intravenous antibiotics. Better treatment strategies are needed, and the results also suggest that prevention of exacerbations would be a useful end-point in clinical trials.

Abstract P102 Table 1

Characteristics of patient cohort

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