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Forskolin-induced Swelling in Intestinal Organoids: An In Vitro Assay for Assessing Drug Response in Cystic Fibrosis Patients
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Voriconazole therapy in children with cystic fibrosis

Tom Hilliard1, Siân Edwards, Roger Buchdahl

  • 1Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, UK. t.hilliard@imperial.ac.uk

Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society
|October 26, 2005
PubMed

Insights

Voriconazole shows promise for treating allergic bronchopulmonary aspergillosis (ABPA) in children with cystic fibrosis (CF). It may be an effective alternative to steroids, especially in monotherapy, though not all patients showed improvement.

Area of Science:

  • Medical Mycology
  • Pediatric Pulmonology
  • Pharmacology

Background:

  • Voriconazole is an antifungal with demonstrated efficacy, particularly in immunocompromised patients.
  • Cystic Fibrosis (CF) patients often face complications from fungal infections, notably Aspergillus fumigatus.

Purpose of the Study:

  • To evaluate the clinical experience of using voriconazole in pediatric patients with cystic fibrosis.
  • To assess voriconazole's efficacy and safety in treating allergic bronchopulmonary aspergillosis (ABPA) and Aspergillus colonization in CF.

Main Methods:

  • A retrospective case note review was conducted.
  • The study included 21 children (aged 5-16 years) with CF treated with voriconazole over 18 months.

Main Results:

  • Voriconazole monotherapy in 2 children with ABPA led to sustained clinical and serological improvements without steroids.
  • Combination therapy in 11 children with ABPA also showed significant improvements in pulmonary function and serology.
  • 8 children with recurrent Aspergillus fumigatus isolates but without ABPA did not improve; adverse effects occurred in 33% of patients.

Conclusions:

  • Voriconazole may serve as a valuable adjunctive therapy for ABPA in cystic fibrosis patients.
  • Voriconazole monotherapy presents a potential alternative treatment when oral corticosteroids are contraindicated or unsuitable.
Abstract

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