title: Sinonasal manifestations of cystic fibrosis: a correlation between genotype and phenotype?
abstract: BACKGROUND
  Patients with Cystic Fibrosis are prone to develop sinonasal disease. Studies in genotype-phenotype correlations for sinonasal disease are scarce and inconclusive.
  
  
  METHODS
  In this observational study several aspects of sinonasal disease were investigated in 104 adult patients with CF. In each patient a disease specific quality of life questionnaire (RSOM-31), nasal endoscopy and a CT scan of the paranasal sinuses were performed. Patients were divided into two groups, class I-III mutations and class IV-V mutations, based on their CFTR mutations.
  
  
  RESULTS
  The prevalence of rhinosinusitis in adult patients with CF was 63% and the prevalence of nasal polyps 25%. Patients with class I-III mutations had significantly smaller frontal sinuses, sphenoid sinuses, more opacification in the sinonasal area and more often osteitis/neoosteogenesis of the maxillary sinus wall compared to patients with class IV and V mutations.
  
  
  CONCLUSIONS
  These data suggest more severe sinonasal disease in patients with class I-III mutations compared to patients with class IV-V mutations.
authors.affiliation: Department of Pulmonology, Haga Teaching Hospital, Leyweg 275 2545 CH The Hague, The Netherlands. Electronic address: m.berkhout@hagaziekenhuis.nl.
authors.forename: M C
authors.initials: MC
authors.lastname: Berkhout
authors.affiliation: Department of Radiology, Haga Teaching Hospital, Leyweg 275 2545 CH, The Hague, The Netherlands.
authors.forename: C J
authors.initials: CJ
authors.lastname: van Rooden
authors.affiliation: Department of Otorhinolaryngology, Haga Teaching Hospital, Leyweg 275 2545 CH, The Hague, The Netherlands.
authors.forename: E
authors.initials: E
authors.lastname: Rijntjes
authors.affiliation: Department of Otorhinolaryngology, Academic Medical Centre Amsterdam, Meibergdreef 9 1105 AZ Amsterdam, The Netherlands.
authors.forename: W J
authors.initials: WJ
authors.lastname: Fokkens
authors.affiliation: Department of Pulmonology, Haga Teaching Hospital, Leyweg 275 2545 CH The Hague, The Netherlands.
authors.forename: L H
authors.initials: LH
authors.lastname: el Bouazzaoui
authors.affiliation: Department of Pulmonology, Haga Teaching Hospital, Leyweg 275 2545 CH The Hague, The Netherlands.
authors.forename: H G M
authors.initials: HG
authors.lastname: Heijerman
mesh_terms: D000328:Adult; D003550:Cystic Fibrosis / Q000150:complications* / Q000235:genetics / Q000378:metabolism; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D004247:DNA / Q000235:genetics; D004252:DNA Mutational Analysis; D004724:Endoscopy; D005260:Female; D006801:Humans; D008297:Male; D009154:Mutation; D010254:Paranasal Sinus Diseases / Q000175:diagnosis / Q000209:etiology* / Q000235:genetics; D010641:Phenotype; D011788:Quality of Life; D011795:Surveys and Questionnaires; D014057:Tomography, X-Ray Computed
chemical_list: D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D004247:DNA
keywords: Cystic fibrosis; Genotype; Nasal polyps; Rhinosinusitis