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Pathogenesis and Management of Nasal Polyposis in Cystic Fibrosis

  • Sinusitis (ML Kowalski, Section Editor)
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Abstract

Beginning in preschool age, during their lives, up to 50% of cystic fibrosis (CF) patients experience obstructing nasal polyposis (NP), which is rare in non-CF children. Pathogenetic factors of NP in general and especially in CF are still obscure. However, defective epithelial ion transport from mucosal glands plays a central role in CF, and viscous secretions impair mucociliary clearance, promoting chronic pathogen colonization and neutrophil-dominated chronic inflammation. Presently, CF-NP is not curable but can be clinically stabilized, though the large variety of proposed treatment modalities indicates a lack of standardization and of evidence of treatment efficacy. When conservative measures are exhausted, surgical intervention combining individually adapted endoscopic sinus surgery and supportive conservative treatment is performed. Topical steroids, approved as the gold standard for non-CF NP, may be beneficial, but they are discussed to be less effective in neutrophilic inflammation, and CF-specific antimicrobial and mucolytic therapy, as is true of all treatment modalities, urgently requires evaluation by controlled clinical trials within interdisciplinary networks.

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Acknowledgment

The authors thank Franziska Doht and Dr. Ruth Michl for their support in preparation of the manuscript.

Disclosure

Dr. Mainz has served on advisory boards for Grunenthal, Gilead, and Pharmaxis; has had travel expenses covered by Novartis, Pari, Roche, Infectopharm, and MSD; and has conducted clinical trials on behalf of Luxemburgian and Germany CF Foundations and Pari Germany, Roche, Infectopharm, and Forest.

Dr. Koitschev reported no potential conflicts of interest relevant to this article.

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  1. Cystic Fibrosis Center, Department of Paediatrics, Paediatric Pulmonology, Jena University Hospital, Kochstrasse 2, 07740, Jena, Germany

    Jochen G. Mainz

  2. Division of Paediatric Otorhinolaryngology and Otology, Department of Otorhinolaryngology, Klinikum Stuttgart, Stuttgart, Germany

    Assen Koitschev

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Mainz, J.G., Koitschev, A. Pathogenesis and Management of Nasal Polyposis in Cystic Fibrosis. Curr Allergy Asthma Rep 12, 163–174 (2012). https://doi.org/10.1007/s11882-012-0250-y

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