Open Access
CC BY-NC-ND 4.0 · Laryngorhinootologie 2022; 101(S 02): S243-S244
DOI: 10.1055/s-0042-1746942
Poster
Pediatric ENT

Effect of CFTR modulation therapy on chronic rhinosinusitis in cystic fibrosis – a case report

Authors

  • Daniela Heine

    1   Universitätsklinikum Augsburg, HNO Augsburg

  • Eric Treutlein

    1   Universitätsklinikum Augsburg, HNO Augsburg

  • Johannes Zenk

    1   Universitätsklinikum Augsburg, HNO Augsburg
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    Introduction 

    Cystic fibrosis (CF) is one of the most common genetic diseases in the Caucasian population. A mutation in the CFTR gene results in dysfunction of ion channels on epithelial cells of various organs, with chronic sinusitis as a common ENT comorbidity. CFTR modulation therapy can improve the performance of affected Cl<sup>-</sup> channels. Potentiators can activate an existing CFTR channel and correctors can support the synthesis of the CFTR channel. The triple therapy Trikafta combines the correctors Tezacaftor and Elexacaftor and the potentiator Ivacaftor. The drug is approved for cystic fibrosis (CF) therapy in Europe since August 2020.

    Methods 

    A case report of a 15-year-old female patient with CF after three sinus surgeries for recurrent polyposis nasi. The interventions where not able to control the nasal symptoms. The patient started triple therapy with Trikafta. The course under medication was observed.

    Results 

    With Trikafta therapy, an absence of recurrent nasal polyps and nasal symptoms was achieved. The follow-up period is 12 months. The improvement CFTR channel function led to a significant improvement of the nasal condition.

    Conclusion 

    Trikafta, a combination drug approved for CF therapy in Europe in 2020, showed very good efficacy on the existing nasal symptomatology in this case. If appropriate, Trikafta may be a powerful alternative to sinus surgery in patients suffering from CF.


     

    Conflict of Interest

    The author declares that there is no conflict of interest.

    Publikationsverlauf

    Artikel online veröffentlicht:
    24. Mai 2022

    © 2022. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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