Objective -The aim of this paper is to describe the health related quality of life (HRQoL) in an unselected hospital-based cohort of patients with Cystic fibrosis (CF), and to explore the association between HRQoL and psychological status. Study design -115 patients with CF (47 children, 26 adolescents and 42 adults) attending the Bambino Gesù Pediatric Hospital in Rome, Italy, were recruited. HRQoL was measured through the Cystic Fibrosis Questionnaire (CFQ), child and adult versions. The

more »

... Self Report Form and the General Health Questionnaire were used to screen for psychological distress in patients aged below and above 18 respectively. Multivariable logistic regression analysis was performed to explore the relationship between CFQ scores and psychological distress, adjusting for socio-demographic and clinical variables. Results -HRQoL was high, except for Social Limitations, Energy/Wellbeing and Treatment Constraints. Twenty-eight patients (24.4%) scored positive for psychological distress. HRQoL scores below median for Symptoms, Body Image and Social Limitations, and unemployment were significantly associated with the presence of psychological distress, while age, screening-based diagnosis, gender and medical factors were not. Conclusions -Screening for psychological distress should be part of routine management of patients with CF. Opportunities for age-appropriate socialization, work and leisure time should be allowed and encouraged.