Bronchoscopy in cystic fibrosis infants diagnosed by newborn screening

Patrick Stafler (1) , Jane Davies (1) , Ian M Balfour-Lynn (1) , Mark Rosenthal (1) , Andrew Bush (1)

1 Respiratory Paediatrics

Background There is evidence of early functional and structural changes in babies with cystic fibrosis (CF) diagnosed on newborn screening (NBS). The aim of the present study was to determine the yield of bronchoalveolar lavage (BAL) microbiology and cytology, and 24 hour pH monitoring in a group of CF infants diagnosed on NBS. Methods Infants referred to a tertiary paediatric respiratory centre between July 2007 and November 2009 underwent surveillance fibre-optic bronchoscopy (FOB), BAL, and insertion of a 24 hour dual pH probe monitoring under a single general anaesthetic. Results We studied 33 infants, median age of 100 days (47 to 215 days) at the time of FOB. In 9 of 33 (27%) bacterial organisms were identified. 7 of the 9 patients (78%) were asymptomatic and only 1 had had a positive cough swab prior to FOB. Neutrophilia was identified in 18/27 (67%) cases with a median of 11% (6% to 73%). 13/31 (42%) had an abnormal pH study with a pH index greater than 12%. Conclusions The high yield of microbiology, cytology and pH probe investigations in NBS infants justifies invasive surveillance. Longitudinal studies to determine if early aggressive treatment results in improved outcome are awaited.

Mots clés: Medicine

Patrick Stafler, Jane Davies, Ian M Balfour-Lynn, Mark Rosenthal, Andrew Bush. Bronchoscopy in cystic fibrosis infants diagnosed by newborn screening. Pediatric Pulmonology, 2011, 46 (7), pp.696. ⟨10.1002/ppul.21434⟩. ⟨hal-00618191⟩