Effect of allergic bronchopulmonary aspergillosis on FEV1in children and adolescents with cystic fibrosis: a European Cystic Fibrosis Society Patient Registry analysis.
To evaluate the effect of allergic bronchopulmonary aspergillosis (ABPA) on FEV1percent predicted in children and adolescents with cystic fibrosis.



Longitudinal data analysis (2008-2010).



Patients participating in the European Cystic Fibrosis Society Patient Registry.



3350 patients aged 6-17 years.



FEV1percent predicted was the main outcome measure (one measurement per year per child). To describe the effect of ABPA (main explanatory variable) on FEV1while controlling for other prognostic factors, a linear mixed effects regression model was applied.



In 2008, the mean (±SD) FEV1percent 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, FEV1in subjects with ABPA on entry to the study was 1.47 percentage points lower than FEV1in patients of similar age without ABPA (p=0.003). There was no FEV1decline 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 FEV1in 2008, followed by high-risk genotype (two severe mutations), female gender, diabetes mellitus, chronicPseudomonas aeruginosainfection and ABPA in descending order of effect size.



In contrast to the common clinical belief of ABPA having a serious impact on lung function, the difference in FEV1between young patients with and without the complication was found to be modest when the effect of other prognostic factors was considered.
Paediatric Pulmonology Unit, First Department of Paediatrics, National and Kapodistrian University of Athens and Aghia Sophia Children's Hospital, Athens, Greece.
Athanasios G
0000-0003-3592-0037
AG
Kaditis
Paediatric Pulmonology Unit, First Department of Paediatrics, National and Kapodistrian University of Athens and Aghia Sophia Children's Hospital, Athens, Greece.|Laboratory of Biomathematics, University of Thessaly School of Medicine, Athens, Greece.
Michael
M
Miligkos
Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy.
Anna
A
Bossi
Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, University of Milan, Milan, Italy.
Carla
C
Colombo
Paediatric Pulmonology Unit and Cystic Fibrosis Unit, 3rd Paediatric Department, Aristotle University of Thessaloniki, Thessaloniki, Greece.
Elpis
E
Hatziagorou
Department of Genetic Epidemiology, Federal State Budgetary Institution 'Research Centre for Medical Genetics', Moscow, Russian Federation.
Nataliya
N
Kashirskaya
Stockholm Cystic Fibrosis Centre Karolinska Institutet, Karolinska University Hospital, Huddinge, Stockholm, Sweden.
Isabelle
I
de Monestrol
Belgian Cystic Fibrosis Registry, Public Health and Surveillance, Scientific Institute of Public Health, Brussels, Belgium.
Muriel
M
Thomas
Respiratory Institute, Schneider Children's Medical Centre of Israel, Sackler School of Medicine, Tel Aviv University, Israel.
Meir
M
Mei-Zahav
First Department of Paediatrics, National and Kapodistrian University of Athens, Athens, Greece.
George
G
Chrousos
Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy.
Anna
A
Zolin
D000293:Adolescent; D017677:Age Distribution; D001229:Aspergillosis, Allergic Bronchopulmonary / Q000150:complications / Q000503:physiopathology*; D015992:Body Mass Index; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications / Q000503:physiopathology*; D005260:Female; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D012042:Registries; D017678:Sex Distribution
allergic bronchopulmonary aspergillosis; cystic fibrosis; lung function