title: Factors influencing quality of life and disease severity in Hungarian children and young adults with cystic fibrosis.
abstract: BACKGROUND
  The aim of our study was to evaluate factors affecting cystic fibrosis (CF) patients' health-related quality of life (HRQoL) and to assess the level of agreement on HRQol between children and their parents.
  
  
  METHODS
  Fifty-nine patients (mean age: 14.03 ± 4.81 years) from 5 Hungarian CF centres completed the survey. HRQoL was measured using The Cystic Fibrosis Questionnaire-Revised (CFQ-R). Parents were asked to fill out a questionnaire about their smoking habits, educational level and history of chronic illness. Disease severity was assessed using the physician-reported Shwachman-Kulczycki (SK) score system. Spirometry, Body Mass Index (BMI) percentile (pc), hospitalisation and Pseudomonas aeruginosa (PA) infection were examined as physiologic parameters of CF, and the impact of these factors on HRQoL was assessed. A multivariate regression analysis was performed to identify the most important factors affecting HRQoL. The level of significance was set to 0.05.
  
  
  RESULTS
  Passive smoking and parental educational level and chronic diseases status did not have a significant impact on the patients' HRQoL (p > 0.05). Significantly lower SK scores and spirometry values were found in low BMI pc patients (p < 0.001), in hospitalised (p < 0.01) and in PA-infected patients (p < 0.01), than in the adequate-weight, non-hospitalised and PA culture-negative subgroup. Lower CFQ-R scores were detected in hospitalised patients than in non-hospitalised patients in their Physical functioning domain. PA-infected patients had HRQoL scores that were significantly worse in the Body image (p < 0.01) and Respiratory symptoms (p < 0.05) domains than the PA culture-negative patients. Patients with a low BMI pc (<25th BMI pc) had significantly lower scores in the Eating, Body image and Treatment burden domains, than the adequate-weight patients (>25th BMI pc) (p < 0.01). A strong child-parent agreement was found in the Physical functioning domain (r = 0.77, p < 0.01).
  
  
  CONCLUSIONS
  Passive smoking, parental educational level and chronic diseases of parents do not affect the HRQoL of CF patients. In contrast, hospitalisation, PA infection and malnutrition have a significant and negative impact on patients' HRQoL and the clinical severity of the disease. Parents and children were consistent in their scoring of symptoms and behaviours that were observable.
authors.affiliation: Department of Pharmacy Administration, Semmelweis University, Hogyes Endre u, 7-9, 1092 Budapest, Hungary. rekabodnar@ymail.com.
authors.forename: Reka
authors.initials: R
authors.lastname: Bodnar
authors.forename: Laszlo
authors.initials: L
authors.lastname: Kadar
authors.forename: Klara
authors.initials: K
authors.lastname: Holics
authors.forename: Rita
authors.initials: R
authors.lastname: Ujhelyi
authors.forename: Lajos
authors.initials: L
authors.lastname: Kovacs
authors.forename: Katalin
authors.initials: K
authors.lastname: Bolbas
authors.forename: Gyongyi
authors.initials: G
authors.lastname: Szekely
authors.forename: Kalman
authors.initials: K
authors.lastname: Gyurkovits
authors.forename: Eniko
authors.initials: E
authors.lastname: Solyom
authors.forename: Agnes
authors.initials: A
authors.lastname: Meszaros
mesh_terms: D000293:Adolescent; D000328:Adult; D015992:Body Mass Index; D002648:Child; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000523:psychology*; D005260:Female; D005500:Follow-Up Studies; D005541:Forced Expiratory Volume; D006304:Health Status; D006305:Health Status Indicators; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D008297:Male; D009752:Nutritional Status; D011446:Prospective Studies; D011788:Quality of Life; D012720:Severity of Illness Index; D013147:Spirometry; D011795:Surveys and Questionnaires; D055815:Young Adult