Further research in which both ventilation distribution and chest CT outcomes are obtained in larger groups of infants with CF are required to better define the role of ventilation distribution outcomes for monitoring disease progression and as an outcome for clinical trials. Acknowledgments The AREST CF would like to kinase inhibitor FTY720 thank the contributions of Princess Margaret Hospital for Children, The Telethon Institute for Child Health Research, Royal Children’s Hospital Melbourne and Murdoch Children’s Research Institute. We would also like to thank the participants and their families who contribute to the AREST CF program. We also acknowledge the support of the Cystic Fibrosis Foundation, Australia to the AREST CF program.
The full authorship of this manuscript includes the following members of the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF): Luke Berry, Luke Garratt, John Massie, Lauren Mott, Srinivas Poreddy and Shannon Simpson. The full membership of AREST CF is available at www.arestcf.org. Footnotes Competing Interests: The authors have declared that no competing interests exist. Funding: This study was funded by the Cystic Fibrosis Foundation Therapeutics (SLY04A0, STICK09A0) and the National Health and Medical Research Council (513730). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
Chronic pulmonary infection with Pseudomonas aeruginosa (Pa) is a significant cause of morbidity and mortality in patients with cystic fibrosis (CF).
(1) Effective antibiotic therapy directed against this pathogen is an integral part of the daily treatment regimen for CF patients chronically infected with Pa.(2) Pa infection is primarily localized to the endobronchial space in CF. Therefore, aerosol delivery of antibiotics is an attractive option for treating these infections. Inhaled antibiotics deliver high doses directly to the site of infection, while minimizing systemic exposure and risk of toxicity.(2) At present, the only approved inhaled antibiotic treatments are nebulizer solutions, specifically, tobramycin inhalation solution [TIS (TOBI?; Novartis AG, Switzerland; Bramitob?; Chiesi Farmaceutici S.p.A., Italy)], colistimethate sodium (Colomycin?; Forest Laboratories, Inc, UK; and Promixin? ; Profile Pharma, Ltd, UK; in a few European Countries only), and more recently, aztreonam inhalation solution [AZLI (Cayston?; Gilead Sciences, Inc.
, Forest City, CA, USA)]. Tobramycin Dacomitinib and aztreonam inhalation solutions have demonstrated efficacy and safety in CF patients with chronic Pa infection.(3,4) TIS is recommended by the Cystic Fibrosis Foundation guidelines for treatment of chronic Pa infection in CF patients ��6 years.(5) Despite that, less than 70% of qualified patients in the US CF Patient Registry were prescribed TIS in 2008.