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Early detection of lung function decrements in children and adolescents with cystic fibrosis using new reference values

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Summary

Interpretation of lung function values in children with cystic fibrosis (CF) depends on the applied reference values. We hypothesize that differences between the new global lung function initiative (GLI) values and the formerly used Zapletal et al. values produce significantly different clinical results. We analyzed 3719 lung function measurements of 108 children and adolescents (n = 54 male; aged 6–18 years) with CF treated between September 1991 and July 2009. Data were analyzed in milliliters (ml) and % predicted (pred.) and interpreted using Zapletal and GLI reference values. Applying GLI compared to Zapletal resulted in significantly lower mean forced expiratory volume in 1s (FEV1)% pred. values: Zapletal 86.6% (SD 20.6), GLI 79.9% (SD 20.3) and 32% (n = 497/1543) were misclassified as normal when using Zapletal. Despite showing no overall differences in FEV1 and forced vital capacity (FVC) between concomitant Pseudomonas detection (PA+) in n = 938 and Pseudomonas negative (PA-) (n = 2781) using either reference PA+ resulted in lower FEV1 and FVC values with increasing age; however, measurement of small airway obstruction with forced expiratory flow at 75% of FVC (FEF75) values – available for Zapletal –showed significant differences. Reassurance regarding lung function when using old reference values may occur with potential clinical significance. Discrepancies in lung function interpretation underline the importance of using uniform and best available reference values.

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Acknowledgements

The authors would like to thank the “CF HILFE Wien Niederösterreich und Burgenland” for their support of the study.

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Authors and Affiliations

  1. Department of Pediatrics and Adolescent Medicine, Wilhelminenspital Vienna, Montleartstrasse 37, 1160, Vienna, Austria

    Angela Zacharasiewicz & Thomas Frischer

  2. Department of Pediatrics, Medical University Hospital Vienna, Vienna, Austria

    Sabine Renner, Flora Haderer, Eleonore Dehlink, Zsolt Szepfalusi & Thomas Frischer

  3. Department of Biomedical Imaging and Image Guided Therapy, Medical University Vienna, Vienna, Austria

    Michael Weber

  4. Department of Pediatrics, The Royal Brompton Hospital, Sydney Street, SW3 6NP, London, UK

    Eleonore Dehlink

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  1. Angela Zacharasiewicz
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  2. Sabine Renner
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  3. Flora Haderer
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  4. Michael Weber
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  5. Zsolt Szepfalusi
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  6. Thomas Frischer
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Correspondence to Angela Zacharasiewicz.

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Conflict of interest

A. Zacharasiewicz, S. Renner, F. Haderer, M. Weber, E. Dehlink, Zsolt Szepfalusi, and T. Frischer declare that they have no competing interests.

Ethical standards

The ethics committee of the department of pediatrics of the Medical University of Vienna approved the collection and analysis of lung function data of all CF patients of the center in retrospect; hence, all CF lung function data were available and included into the study.

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Zacharasiewicz, A., Renner, S., Haderer, F. et al. Early detection of lung function decrements in children and adolescents with cystic fibrosis using new reference values. Wien Klin Wochenschr 129, 533–539 (2017). https://doi.org/10.1007/s00508-017-1184-0

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  • DOI: https://doi.org/10.1007/s00508-017-1184-0

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