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J Cyst Fibros. 2016 Oct 11. pii: S1569-1993(16)30611-7. doi: 10.1016/j.jcf.2016.09.009. [Epub ahead of print]

Year to year change in FEV1 in patients with cystic fibrosis and different mutation classes.

Author information

  • 1Department of Pediatrics, University of Leuven, Belgium; Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy. Electronic address: christiane.deboeck@uz.kuleuven.ac.be.
  • 2Department of Pediatrics, University of Leuven, Belgium; Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy.

Abstract

In patients with cystic fibrosis, most treatments addressing the underlying basic defect are mutation or mutation class specific. These treatments are disease modifying if they lower the year to year change in lung function. We therefore calculated the current loss of lung function, measured by year to year change in forced expired volume in 1s in 11,417 patients included in the European Cystic Fibrosis Society Patient Registry. Whereas patients with at least one mutation of class IV or V have on average a lower year to year change, we did not find a difference between patients with a stop codon mutation, homozygous for F508del or at least one class III mutation. These data are useful background information to discuss the impact of different disease modifying treatments.

KEYWORDS:

Age distribution; Cystic fibrosis; Lung function; Mutation class

PMID:
27745801
DOI:
10.1016/j.jcf.2016.09.009
[PubMed - as supplied by publisher]
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