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Original article
Evolution of pulmonary inflammation and nutritional status in infants and young children with cystic fibrosis
  1. Sarath C Ranganathan1,2,3,4,
  2. Faith Parsons5,
  3. Catherine Gangell5,
  4. Siobhan Brennan5,
  5. Stephen M Stick5,6,7,
  6. Peter D Sly5,6,
  7. on behalf of the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF)
  1. 1Department of Respiratory Medicine, Royal Children's Hospital Melbourne, Melbourne, Australia
  2. 2Infection, Immunity & Environment Theme, Murdoch Children's Research Institute, Melbourne, Australia
  3. 3Department of Paediatrics, University of Melbourne, Melbourne, Australia
  4. 4Brighton and Sussex Medical School, University of Sussex, Brighton, UK
  5. 5Clinical Sciences, Telethon Institute for Child Health Research, Centre for Child Health Research, University of Western Australia, Perth, Western Australia, Australia
  6. 6Department of Respiratory Medicine, Princess Margaret Hospital, Perth, Western Australia, Australia
  7. 7School of Paediatric and Child Health, University of Western Australia, Perth, Western Australia, Australia
  1. Correspondence to Professor Sarath C Ranganathan, Department of Respiratory Medicine, Royal Children's Hospital Melbourne, Flemington Road, Parkville 3052, Melbourne, Australia; sarath.ranganathan{at}


Introduction Improved nutrition is the major proven benefit of newborn screening programmes for cystic fibrosis (CF) and is associated with better clinical outcomes. It was hypothesised that early pulmonary inflammation and infection in infants with CF is associated with worse nutrition.

Methods Weight, height and pulmonary inflammation and infection in bronchoalveolar lavage (BAL) were assessed shortly after diagnosis in infants with CF and again at 1, 2 and 3 years of age. Body mass index (BMI) was expressed as z-scores. Inflammatory cells and cytokines (interleukin 1β (IL-1β), IL-6, IL-8 and tumour necrosis factor α (TNFα)), free neutrophil elastase activity and myeloperoxidase were measured in BAL. Mixed effects modelling was used to assess longitudinal associations between pulmonary inflammation, pulmonary infection (Staphylococcus aureus and Pseudomonas aeruginosa) and BMI z-score after adjusting for potential confounding factors.

Results Forty-two infants were studied (16 (38%) male; 39 (93%) pancreatic insufficient); 36 were diagnosed by newborn screening (at median age 4 weeks) and six by early clinical diagnosis (meconium ileus). Thirty-one (74%) received antistaphylococcal antibiotics. More than two-thirds were asymptomatic at each assessment. Mean BMI z-scores were −1.5 at diagnosis and 0.5, −0.2 and −0.1 at 1, 2 and 3 years, respectively. Neutrophil elastase and infection with S aureus were associated with lower BMI, whereas age (p=0.01) and antistaphylococcal antibiotics (p=0.013) were associated with increased BMI. On average, each log10 increase in free neutrophil elastase activity was associated with a 0.43 (95% CI 0.06 to 0.79) reduction in BMI z-score.

Discussion Early nutritional status is associated with the underlying pulmonary pathophysiology in CF, and better understanding of these relationships is required. Studies are required to assess whether interventions can decrease pulmonary inflammation and improve nutrition. Early surveillance will enable such targeted interventions with the aim of improving these important clinical outcomes.

  • Bronchoscopy
  • cystic fibrosis
  • paediatric lung disaese
  • respiratory infection

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Supplementary materials

  • Web Only Data thx.2010.139493

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  • Web Only Data thx.2010.139493

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  • Funding This study has been funded by grants from the US Cystic Fibrosis Foundation (SLY04A0 and STICK09A0), the National Health and Medical Research Council (3211912, #458513) and the Murdoch Children's Research Institute, Australia.

  • Competing interests None.

  • Ethics approval This study was conducted with the approval of the Princess Margaret Hospital Research Ethics Committee, Perth.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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