Rhinosinusitis in cystic fibrosis: not a simple story

Int J Pediatr Otorhinolaryngol. 2008 May;72(5):619-24. doi: 10.1016/j.ijporl.2008.01.010. Epub 2008 Feb 21.

Abstract

Objective: Chronic inflammation of sinuses and nasal mucosa is found in 74-100% patients suffering from cystic fibrosis, whereas nasal polyps in 6-44% patients. The aim of this paper is to assess rhinosinusitis types taking into account the forms of cystic fibrosis and the kind of CFTR gene mutation.

Material and methods: The author presents material of 126 cystic fibrosis patients, 90 with typical clinical features and 36 with atypical phenotype. Genetic tests were carried out to determine the genotype of CFTR gene. The sample was divided into four groups according to the genotype effect on the chloride canal function. Cytological examination of nasal mucosa was carried out in all the patients.

Results: In 71.5% of patients with cystic fibrosis, infectious chronic non-specific rhinosinusitis was found. Other types of rhinosinusitis--acute infectious, chronic allergic and non-allergic with eosinophilia were found in 21.4% of patients, whereas in 7.1% of patients no clinical symptoms of rhinosinusitis were found. Nasal polyps were found in 23 (18.3%) patients with cystic fibrosis: in 21 patients with a typical form and in 2 patients with an atypical form. Nasal polyps were more frequent in groups with the genotype consisting of both "strong" mutations than in the group with unknown or "mild" mutations.

Conclusion: Rhinosinusitis in cystic fibrosis patients is not homogenous pathology. Infectious chronic non-specific rhinosinusitis is found the most frequently, but other forms of rhinosinusitis appear quite often and they require proper treatment.

MeSH terms

  • Acute Disease
  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Chronic Disease
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / genetics
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Humans
  • Hypersensitivity / complications
  • Hypersensitivity / diagnosis
  • Infant
  • Infections / complications
  • Infections / diagnosis
  • Mutation
  • Nasal Polyps / complications
  • Rhinitis / complications*
  • Rhinitis / diagnosis
  • Sinusitis / complications*
  • Sinusitis / diagnosis

Substances

  • Cystic Fibrosis Transmembrane Conductance Regulator