Cross-cultural adaptation and pilot psychometric validation of a quality-of-life questionnaire for adults with Primary Ciliary Dyskinesia in Spain.

Autores de IIS La Fe

• María Del Lidón Carretero Vilarroig

  Autor

• Teresa Jaijo Sanchís

  Autor

• Miguel Armengot Carceller

  Autor

Participantes ajenos a IIS La Fe

• Lucas JS
• Behan L
• Dasí F
• Sánchez-Thevenet P
• Reula A

Grupos

• 

  Biomedicina Molecular, Celular y Genómica

Abstract

BACKGROUND: The Quality-of-Life Primary Ciliary Dyskinesia (QOL-PCD) instrument is the only health-related QOL instrument specific to measuring patient-reported outcomes in PCD patients. The adaptation and validation of the Spanish version of this questionnaire is a crucial step in formulating effective healthcare strategies that will improve the quality of life of these patients within the Spanish context. In this regard, the objective of the study was to cross-culturally adapt and pilot-validate a Spanish version for patients with PCD. METHODS: A total of 21 Spanish patients with PCD participated in this study. The QOL-PCD was translated and cross-culturally adapted following BESTCILIA Work Package 4 and ISPOR recommendations, respectively. Pilot psychometric validation was explored through internal consistency (Cronbach's a) and construct validity studies. Stability and convergent validity were studied in eight patients. QoL analyses were performed using Pearson correlations and a Welch t-test. RESULTS: The Spanish QOL-PCD questionnaire included 40 items evaluating the ten original scales. All participants demonstrated a clear understanding of the questionnaire. All scales shown moderate to good internal consistency. Test-retest reliability proved particularly strong for Physical Functioning, Vitality, Emotional Functioning, Role and Social Functioning. SF-36 and SNOT-22 comparisons demonstrated moderate to high correlations. As hypothesized, a positive correlation was found between lower respiratory symptoms and history of lung transplantation. CONCLUSION: The Spanish QOL-PCD questionnaire has robust psychometric properties and is therefore a validated tool for assessing quality of life (QoL) in Spanish adults with primary ciliary dyskinesia (PCD). This work will improve patient care and inform future PCD research.

Copyright © 2026 The Authors. Published by Elsevier Ltd.. All rights reserved.

Keywords

• Primary ciliary dyskinesia; Psychometric analysis; Quality of life; Rare diseases; Validation study