Gancz Daniela W, Cunha Maristela T, Leone Claudio, Rodrigues Joaquim C, Adde Fabíola V
Unidade de Pneumologia Pediatrica, Instituto da Crianca, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, SP, BR.
Servico de Fisioterapia, Instituto da Crianca, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, BR.
Clinics (Sao Paulo). 2018 Oct 18;73:e427. doi: 10.6061/clinics/2017/e427.
The current study sought to evaluate the quality of life of young patients with cystic fibrosis and correlate these results with the clinical parameters indicative of disease severity.
This cross-sectional study applied the validated Portuguese version of a cystic fibrosis specific quality of life questionnaire to clinically stable patients aged 14 to 21 years old. The correlations between the questionnaire domain scores and forced expiratory volume in one second (FEV1) values, the Shwachman-Kulczycki score, and body mass index were assessed, and correlations were considered as significant when p<0.05.
A total of 31 patients (11 females; 16.4±2.3 years old) were evaluated, and the median scores on the questionnaire domains ranged from 66.7 to 100. A significant correlation was found between body mass index and the weight (r=0.43, p=0.016) and the eating questionnaire domains (r=0.44, p=0.013); between FEV1 and the physical (r=0.53, p=0.002) and treatment burden (r=0.41, p=0.023) domains; and between the Shwachman-Kulczycki score and the physical (r=0.39, p=0.03), health (r=0.41, p=0.023), and role (r=0.37, p=0.041) domains. A significant difference was found amongst patients with FEV1 values above or below 60% of the predicted value with regard to the role and health domains. No differences in the scores were found according to gender.
The current cystic fibrosis specific quality of life questionnaire scores exhibited wide variability across all domains; however, they indicated a relatively satisfactory quality of life amongst the patients studied. Certain domains exhibited significant correlations with clinical parameters; thus, this instrument has consistent associations with clinical outcomes.
本研究旨在评估年轻囊性纤维化患者的生活质量，并将这些结果与表明疾病严重程度的临床参数相关联。
这项横断面研究对14至21岁临床稳定的患者应用了经过验证的葡萄牙语版囊性纤维化特异性生活质量问卷。评估了问卷领域得分与一秒用力呼气量（FEV1）值、施瓦赫曼 - 库尔奇茨基评分和体重指数之间的相关性，当p<0.05时，相关性被认为具有显著性。
共评估了31名患者（11名女性；年龄16.4±2.3岁），问卷领域的中位数得分在66.7至100之间。发现体重指数与体重（r = 0.43，p = 0.016）和饮食问卷领域（r = 0.44，p = 0.013）之间存在显著相关性；FEV1与身体（r = 0.53，p = 0.002）和治疗负担（r = 0.41，p = 0.023）领域之间存在显著相关性；施瓦赫曼 - 库尔奇茨基评分与身体（r = 0.39，p = 0.03）、健康（r = 0.41，p = 0.023）和角色（r = 0.37，p = 0.041）领域之间存在显著相关性。在FEV1值高于或低于预测值60%的患者中，在角色和健康领域发现了显著差异。根据性别未发现得分差异。
目前的囊性纤维化特异性生活质量问卷得分在所有领域都表现出很大的变异性；然而，它们表明在所研究的患者中生活质量相对令人满意。某些领域与临床参数表现出显著相关性；因此，该工具与临床结果具有一致的关联。
