Abstract
ABSTRACTObjective: To verify the association between quality of life, functional capacity and clinical and nutritional status in children and adolescents with cystic fibrosis (CF).Methods: Cross-sectional study, including patients from eight to 18 years old with CF. Quality of life, functional capacity, nutritional status and clinical status were evaluated with the Cystic Fibrosis Questionnaire; the 6-minute walk test (6MWT) and manual gripping force (MGF); the height percentiles for age and body mass index for age and respiratory function test, respectively. Pearson and Spearman correlation tests and logistic regression were used to analyze the data.Results: A total of 45 patients, 13.4±0.5 years old, 60% female, 60% colonized by Pseudomonas aeruginosa and 57.8% with at least one F508del mutation participated in the study. When assessing the perception of quality of life, the weight domain reached the lowest values, and the digestive domain, the highest. In the pulmonary function test, the forced expiratory volume of the first second was 77.3±3.3% and the 6MWT and MGF presented values within the normal range. There was an association between quality of life and functional capacity, nutritional status and clinical status of CF patients.Conclusions: The study participants had good clinical conditions and satisfactory values of functional capacity and quality of life. The findings reinforce that the assessment of quality of life may be important for clinical practice in the management of treatment.
Highlights
Cystic fibrosis (CF) is a genetic, autosomal, recessive disease, more common in Caucasians, which manifests itself in many patients in the first years of life.[1,2] The disease is characterized by a dysfunction of the cystic fibrosis transmenbrane conductance regulator (CFTR), responsible for regulating the transport of sodium, chlorine and water through epithelial membranes.[2,3] The prevalence of CF varies around the world: from one in 1,400 inhabitants in Ireland to one in 3,500 in the United States.[4]
A cross-sectional study was carried out as a result of the first stage of evaluations of the cohort of CF patients followed by a reference center located in Rio de Janeiro State, which features a multidisciplinary team composed of doctors, physiotherapists, nutritionists, nurses, psychologists and social workers, which is responsible for the care of approximately 165 CF patients in quarterly consultations segmented by the type of bacterial colonization presented at the time of the consultation, that is, scheduled in groups according to the bacteria colonized in sputum
All children and adolescents aged between eight and 18 years old and with a CF diagnosis confirmed by the presence of two mutations in the CFTR gene, as agreed by the Cystic Fibrosis Foundantion,[14] who attended the scheduled multidisciplinary consultation were invited to participate in the study
Summary
Cystic fibrosis (CF) is a genetic, autosomal, recessive disease, more common in Caucasians, which manifests itself in many patients in the first years of life.[1,2] The disease is characterized by a dysfunction of the cystic fibrosis transmenbrane conductance regulator (CFTR), responsible for regulating the transport of sodium, chlorine and water through epithelial membranes.[2,3] The prevalence of CF varies around the world: from one in 1,400 inhabitants in Ireland to one in 3,500 in the United States.[4]. It is considered as an important indicator for assessing the QoL of patients with CF
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