Relationship between functioning, clinical severity, and respiratory status in children and adolescents with cystic fibrosis: a cross-sectional study

Authors

  • Luciana Santos Carvalho Programa de Pós-Graduação Stricto Sensu em Ciências da Reabilitação e Desempenho Físico-Funcional, Faculdade de Fisioterapia, Universidade Federal de Juiz de Fora, Juiz de Fora, Minas Gerais, Brasil. https://orcid.org/0000-0002-1535-9265
  • Rafaela Ramos Anacleto da Silva Programa de Pós-Graduação Stricto Sensu em Ciências da Reabilitação e Desempenho Físico-Funcional, Faculdade de Fisioterapia, Universidade Federal de Juiz de Fora, Juiz de Fora, Minas Gerais, Brasil. https://orcid.org/0009-0003-5615-0980
  • Mário Flávio Cardoso de Lima Hospital Universitário da Universidade Federal de Juiz de Fora, Empresa Brasileira de Serviços Hospitalares, Juiz de Fora, Minas Gerais, Brasil. https://orcid.org/0000-0002-5735-4411
  • Marta Cristina Duarte Faculdade de Medicina, Universidade Federal de Juiz de Fora, Juiz de Fora, Minas Gerais, Brasil. https://orcid.org/0000-0002-6139-9380
  • Paula Silva de Carvalho Chagas Programa de Pós-Graduação Stricto Sensu em Ciências da Reabilitação e Desempenho Físico-Funcional, Faculdade de Fisioterapia, Universidade Federal de Juiz de Fora, Juiz de Fora, Minas Gerais, Brasil. https://orcid.org/0000-0002-4312-0403
  • Carla Malaguti Programa de Pós-Graduação Stricto Sensu em Ciências da Reabilitação e Desempenho Físico-Funcional, Faculdade de Fisioterapia, Universidade Federal de Juiz de Fora, Juiz de Fora, Minas Gerais, Brasil.

Keywords:

Cystic fibrosis;, Functioning;, PEDI-CAT;, Functional assessment;, Children.

Abstract

Introduction: Cystic fibrosis (CF) is a chronic genetic disease with multisystemic impact, primarily respiratory, which can compromise functioning in children and adolescents. The Pediatric Evaluation of Disability Inventory – Computer Adaptive Test (PEDI-CAT) is a functional tool based on the International Classification of Functioning, Disability and Health (ICF), with potential to assess multiple domains of functioning. Objective: To evaluate the relationship between functioning, clinical severity, and respiratory impairment in children and adolescents with CF. Material and Methods: A cross-sectional observational study was conducted with 26 participants aged 6 to 21 years with a confirmed diagnosis of CF. Anthropometric and spirometry characteristics (forced expiratory volume – FEV1), functional capacity (Six-Minute Walk Test – 6MWT), Shwachman-Kulczycki score, and functioning (PEDI-CAT) were assessed. Correlations were analyzed using Pearson’s coefficient, and group comparisons by t-test. Results: All PEDI-CAT domains showed a positive and moderate correlation with FEV1 (r = 0.46–0.57; p < 0.05), and the mobility domain was positively and moderately correlated with clinical severity (r = 0.48; p = 0.01). No significant correlations were observed between PEDI-CAT domains and the 6MWT. Children with greater respiratory obstruction presented higher scores in the responsibility domain of the PEDI-CAT (p < 0.05).  Conclusion: Functioning, as measured by the PEDI-CAT, is related to clinical and respiratory severity in CF. The instrument proved to be sensitive in discriminating functional differences, particularly in the responsibility domain

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Published

2026-03-23

How to Cite

1.
Carvalho LS, Silva RRA da, Lima MFC de, Duarte MC, Chagas PS de C, Malaguti C. Relationship between functioning, clinical severity, and respiratory status in children and adolescents with cystic fibrosis: a cross-sectional study. HU Rev [Internet]. 2026Mar.23 [cited 2026Mar.29];51:1-9. Available from: https://periodicos.ufjf.br/index.php/hurevista/article/view/51373

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Vol. 51 (2025)

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Artigos Originais

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Copyright (c) 2026 Luciana Santos Carvalho, Rafaela Ramos Anacleto da Silva, Mário Flávio Cardoso de Lima, Marta Cristina Duarte, Paula Silva de Carvalho Chagas, Carla Malaguti

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