title: Relationship Between Clinical Variables and Health-Related Quality of Life in Young Adult Subjects With Cystic Fibrosis.
abstract: BACKGROUND
  Health-related quality of life (HRQOL) has received much attention in patients with cystic fibrosis (CF). The goal of this study was to evaluate the association between clinical, lung function, sleep quality, and polysomnographic variables with 2 HRQOL questionnaires, the shorter-version World Health Organization Quality of Life (WHOQOL-BREF) and Cystic Fibrosis Quality of Life (CFQOL) questionnaires, in adult subjects with CF.
  
  
  METHODS
  In a cross-sectional study, 51 subjects underwent clinical evaluation and overnight polysomnography and answered WHOQOL-BREF, CFQOL, Pittsburgh Sleep Quality Index, and Epworth Sleepiness Scale questionnaires. In addition, pulmonary function tests, 6-min walk tests, and echocardiography were performed.
  
  
  RESULTS
  For WHOQOL-BREF scores, the sleep quality index was associated with the physical domain; the percent-of-predicted 6-min walk distance (6MWD) and sleepiness scale were associated with the psychological domain; the percent-of-predicted FEV1 and sleep quality index were associated with the social relationship domain; and the sleep quality index was associated with the environment domain. For CFQOL scores, age at diagnosis, clinical score, and sleep quality index were associated with the physical functioning domain; the percent-of-predicted 6MWD and pulmonary arterial systolic pressure were associated with the role domain; sex and sleep quality index were associated with the vitality domain; the apnea-hypopnea index was associated with the emotional functioning domain; sex and body mass index (BMI) were associated with the body image domain; the percent-of-predicted 6MWD and sleep quality index were associated with the health perception domain; age, sex, BMI, and arousal index were associated with the weight domain; age, sex, percent-of-predicted FEV1, percent-of-predicted 6MWD, and pulmonary arterial systolic pressure were associated with the respiratory symptom domain; and the clinical score was associated with the digestive symptom domain.
  
  
  CONCLUSIONS
  The sleep quality index score, 6MWD, sleepiness scale score, and FEV1 were predictors of WHOQOL-BREF scores. Age at diagnosis, clinical score, sleep quality score, 6MWD, sex, apnea-hypopnea index, BMI, current age, arousal index, FEV1, and pulmonary arterial systolic pressure were predictors of CFQOL scores.
authors.affiliation: Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, Rio Grande do Sul, Brazil.
authors.forename: Gabriele C
authors.initials: GC
authors.lastname: Forte
authors.affiliation: Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, Rio Grande do Sul, Brazil.
authors.forename: Gabriela C
authors.initials: GC
authors.lastname: Barni
authors.affiliation: Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, Rio Grande do Sul, Brazil.
authors.forename: Christiano
authors.initials: C
authors.lastname: Perin
authors.affiliation: Hospital de Clínicas de Porto Alegre, Universidade Federal do Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil.
authors.forename: Fernanda C
authors.initials: FC
authors.lastname: Casarotto
authors.affiliation: Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, Rio Grande do Sul, Brazil. Pneumology Department, Hospital de Clínicas de Porto Alegre, Porto Alegre, Rio Grande do Sul, Brazil.
authors.forename: Simone C
authors.initials: SC
authors.lastname: Fagondes
authors.affiliation: Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, Rio Grande do Sul, Brazil. Pneumology Department, Hospital de Clínicas de Porto Alegre, Porto Alegre, Rio Grande do Sul, Brazil. pdalcin@terra.com.br.
authors.forename: Paulo de Tarso Roth
authors.initials: Pde T
authors.lastname: Dalcin
mesh_terms: D000328:Adult; D000367:Age Factors; D001828:Body Image; D015992:Body Mass Index; D001938:Brazil; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000503:physiopathology / Q000523:psychology*; D004452:Echocardiography; D005080:Exercise Test; D005260:Female; D006801:Humans; D008297:Male; D017286:Polysomnography; D011788:Quality of Life; D012129:Respiratory Function Tests; D012720:Severity of Illness Index; D012737:Sex Factors; D012890:Sleep; D011795:Surveys and Questionnaires; D016138:Walking; D055815:Young Adult
keywords: 6-min walk test; cystic fibrosis; quality of life; respiratory function tests; sleep disorders