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Effect of allergic bronchopulmonary aspergillosis on FEV1 in children and adolescents with cystic fibrosis: a European Cystic Fibrosis Society Patient Registry analysis
  1. Athanasios G Kaditis1,
  2. Michael Miligkos1,2,
  3. Anna Bossi3,
  4. Carla Colombo4,
  5. Elpis Hatziagorou5,
  6. Nataliya Kashirskaya6,
  7. Isabelle de Monestrol7,
  8. Muriel Thomas8,
  9. Meir Mei-Zahav9,
  10. George Chrousos10,
  11. Anna Zolin3
  1. 1 Paediatric Pulmonology Unit, First Department of Paediatrics, National and Kapodistrian University of Athens and Aghia Sophia Children's Hospital, Athens, Greece
  2. 2 Laboratory of Biomathematics, University of Thessaly School of Medicine, Athens, Greece
  3. 3 Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy
  4. 4 Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, University of Milan, Milan, Italy
  5. 5 Paediatric Pulmonology Unit and Cystic Fibrosis Unit, 3rd Paediatric Department, Aristotle University of Thessaloniki, Thessaloniki, Greece
  6. 6 Department of Genetic Epidemiology, Federal State Budgetary Institution 'Research Centre for Medical Genetics', Moscow, Russian Federation
  7. 7 Stockholm Cystic Fibrosis Centre Karolinska Institutet, Karolinska University Hospital, Huddinge, Stockholm, Sweden
  8. 8 Belgian Cystic Fibrosis Registry, Public Health and Surveillance, Scientific Institute of Public Health, Brussels, Belgium
  9. 9 Respiratory Institute, Schneider Children's Medical Centre of Israel, Sackler School of Medicine, Tel Aviv University, Israel
  10. 10 First Department of Paediatrics, National and Kapodistrian University of Athens, Athens, Greece
  1. Correspondence to Dr Athanasios G Kaditis, First Department of Paediatrics, National and Kapodistrian University of Athens School of Medicine and Aghia Sophia Children's Hospital, Thivon and Papadiamantopoulou St, Athens 115.27, Greece; kaditia{at}hotmail.com

Abstract

Objective To evaluate the effect of allergic bronchopulmonary aspergillosis (ABPA) on FEV1 percent predicted in children and adolescents with cystic fibrosis.

Design Longitudinal data analysis (2008–2010).

Setting Patients participating in the European Cystic Fibrosis Society Patient Registry.

Participants 3350 patients aged 6–17 years.

Main outcome measure FEV1percent predicted was the main outcome measure (one measurement per year per child). To describe the effect of ABPA (main explanatory variable) on FEV1 while controlling for other prognostic factors, a linear mixed effects regression model was applied.

Results In 2008, the mean (±SD) FEV1 percent predicted was 78.6 (±20.6) in patients with ABPA (n=346) and 88 (±19.8) in those without ABPA (n=2806). After considering other variables, FEV1 in subjects with ABPA on entry to the study was 1.47 percentage points lower than FEV1 in patients of similar age without ABPA (p=0.003). There was no FEV1 decline associated with ABPA over the subsequent study years as the interaction of ABPA with age was not significant (p>0.05). For patients aged 11.82 years (population mean age), poor body mass index had the greatest impact on FEV1 in 2008, followed by high-risk genotype (two severe mutations), female gender, diabetes mellitus, chronic Pseudomonas aeruginosa infection and ABPA in descending order of effect size.

Conclusions In contrast to the common clinical belief of ABPA having a serious impact on lung function, the difference in FEV1 between young patients with and without the complication was found to be modest when the effect of other prognostic factors was considered.

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Footnotes

  • Contributors AGK has full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. AGK has prepared and submitted the initial research proposal, contributed to data analysis, prepared the first and the final versions of the manuscript, and approved the version to be submitted and published. MM, GC and AZ have contributed to study design and data analysis, revised the manuscript critically for important intellectual content, and approved the version to be submitted and published. AB, CC, EH, NK, IM, MT and MM-Z have contributed to acquisition of data, revised the manuscript critically for important intellectual content, and approved the version to be submitted and published.

  • Competing interests None declared.

  • Ethics approval Scientific and Steering committees of the European Cystic Fibrosis Society Patient Registry.

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

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