Children with cystic fibrosis: who should visit the otorhinolaryngologist?

Martyn G Slieker1, Anne G M Schilder, Cuno S P M Uiterwaal

  • 1Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, The Netherlands.

Insights

A new scoring rule can help identify children with cystic fibrosis (CF) who may have sinonasal polyposis. This rule uses sex, age, rhinorrhea, and lung function to guide referrals to an otorhinolaryngologic physician.

Area of Science:

  • Pediatric Otolaryngology
  • Cystic Fibrosis Research
  • Respiratory Medicine

Background:

  • Sinonasal complications are frequent in pediatric cystic fibrosis (CF) patients.
  • Persistent sinonasal polyposis often necessitates referral to an otorhinolaryngologic (ORL) physician for surgical evaluation.
  • Previous research indicates clinical distinctions between CF patients with and without sinonasal polyps.

Purpose of the Study:

  • To develop a predictive model for sinonasal polyposis in children with CF.
  • To identify clinical characteristics and symptoms indicative of sinonasal polyposis.
  • To aid in selecting pediatric CF patients who would benefit from ORL consultation.

Main Methods:

  • Analysis of data from a database of 140 children with CF undergoing annual multidisciplinary examinations.
  • Evaluation of clinical characteristics and symptoms to predict the presence of sinonasal polyposis.
  • Development and validation of a scoring rule based on identified independent predictors.

Main Results:

  • No single combination of symptoms or characteristics accurately predicted sinonasal polyposis in the entire cohort.
  • In children with a history of nasal symptoms, male sex, age ≥10 years, rhinorrhea, and forced vital capacity (FVC) ≥70% predicted were independent predictors.
  • The developed scoring rule demonstrated an area under the receiver operating characteristic curve of 0.77, with positive and negative predictive values of 0.86 and 0.71, respectively.

Conclusions:

  • A scoring rule incorporating sex, age, rhinorrhea, and FVC can effectively stratify risk for sinonasal polyposis in pediatric CF patients.
  • This rule assists in clinical decision-making regarding the need for ORL referral.
  • Improved identification of at-risk children can lead to timely specialist intervention for sinonasal issues in CF.
Abstract

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