S94 CYSTIC FIBROSIS NEWBORN SCREENING: THE CASE FOR INVASIVE SURVEILLANCE AFTER DIAGNOSIS

P. Stafler, J. C. Davies, I. M. Balfour-Lynn, M. Rosenthal, A. Bush. Royal Brompton Hospital, London, UK

Introduction and Objectives Cystic fibrosis (CF) newborn screening (NBS) raised the issue of how to manage asymptomatic infants. There is evidence of early functional, structural and pathological changes, and even bronchiectasis in babies with CF diagnosed by NBS. We have previously shown a high prevalence of unsuspected lower airway infection in newly diagnosed, unscreened children with CF. The aim of this study was to determine the yield of bronchoalveolar lavage (BAL) microbiology/cytology and 24-hour pH monitoring in a group of NBS-diagnosed infants.

Methods Infants with CF diagnosed by NBS between July 2007 and July 2009 underwent surveillance fibre-optic bronchoscopy (FOB), BAL and 24-hour dual-probe pH monitoring. All the invasive procedures were performed under the same general anaesthetic.

Results Full data sets were available for 19 infants, median age 3 months (47–193 days) at the time of FOB. In 6 out of 19 (32%) at least one bacterial organism was identified on BAL. None of the patients was symptomatic at the time of FOB. Cough swabs taken at various time points (8–77 days) prior to FOB were all negative. Organisms identified were Haemophilus influenzae (n = 3), Staphylococcus aureus (n = 2), methicillin-resistant S aureus (MRSA) (n = 1), Pseudomonas aeruginosa (n = 1) and Moraxella catarrhalis (n = 1). All led to a change in antibiotics. Five of 16 (31%) had neutrophilia (median 11.4%, range 0.3–73%). Nine of 17 (53%) had an abnormal pH study with a pH index ⩾12. Only 2 of those 9 patients (22%) had a clinical diagnosis of gastro-oesophageal reflux prior to pH study.

Conclusions The high yield of microbiology, cytology and pH probe investigations in infants subjected to NBS justifies invasive surveillance FOB. Longitudinal studies to determine if early aggressive treatment results in improved outcome are awaited.

S95 EFFECT OF EXERCISE ON LARGE ARTERY HAEMODYNAMICS IN CYSTIC FIBROSIS

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