CFDB - Cystic Fibrosis DataBase

  1. Home
  2. Database
Search (?)
at least one term
all terms
Advanced search
Other Reviews - Other Review

Providing Mobile Patient Access to Their Electronic Secondary Care Patient Record in Adults With Cystic Fibrosis: Results of a Prospective, Parallel, Randomized Open-Pilot Quantitative Study.

  • Year: 2025
  • Date:
  • Author: Chadwick HK
Source

JMIR Form Res. 2025 Dec 25;9:e69747. doi: 10.2196/69747.

Study design (if review, criteria of inclusion for studies)

Systematic review

Participants

Studies reporting nasal endoscopic outcomes using validated scoring systems before and after CFTR modulator therapy were included.

Interventions

CFTR modulator therapy

Outcome measures

Rhinosinusitis and nasal polyposis. Endoscopic scores, including the Modified Lund-Kennedy scale

Main results

Out of 232 identified records, 10 studies met inclusion criteria, representing populations from six countries. Most studies assessed triple therapy (Elexacaftor/Tezacaftor/Ivacaftor). Endoscopic scores, including the Modified Lund-Kennedy scale, showed significant reductions in nasal polyps, mucosal edema, and discharge. Pediatric and adult groups benefited alike, with triple therapy proving more effective than dual or monotherapy. Secondary outcomes included improved pulmonary function, weight gain, and olfactory recovery.

Authors' conclusions

This review demonstrates that CFTR modulators provide significant benefits for sinonasal disease in CF, reinforcing their role as a comprehensive therapeutic approach addressing both upper airway and systemic disease burdens.

Keywords: pharmacological_intervention; Steroids; Nasal Polyps; Respiratory Tract Diseases; Adult; Aged; CFTR Modulators; Genetic Predisposition to Disease; placebo; VX-770; VX-661; ivacaftor; Aminophenols; tezacaftor; VX-445; elexacaftor; Trikafta; kaftrio;