Health-Related Quality of Life in Primary Immune Deficient Patients

The primary immunodeficiency (pi) disorders are abnormalities in development andmaturation of the immune system. individuals with pi disease may experience frequentinfections, which limit their abilities to exhibit physical and psychological well-beingsecondary to their illness. in this survey we compared health-related quality of life of primaryimmune deficient patients with healthy children.the case-control study was designed for patients with pi disease who were referred tochildren medical center in 2004-2005. demographic information was taken and pediatricquality of life (pedqol) questionnaire were filled for 50 pi patients and 100 healthychildren.the mean age in pi patients was 12.62± 3.65 (range from 8 to 18) years and in thecontrol group was 11.04± 3.3 years. in pi patients 68% were male and 32% female .mostpatients with pi disease had a diagnosis of common variable immunodeficiency (54%) or xlinkedagammaglobulinemia (24%). patients with pi disease had great limitations in physicalfunctioning and psychological well-being (p<0.001 and p<0.001 respectively) compared withchildren without a chronic health condition. patients had lower pedqol scores in all agegroups compared with normal sample (p<0.001). long duration of disease significantlycorrelated with low psychological score. (r = -3.23. p= 0.03)children with pi disease experience poorer health related quality of life than healthychildren, indicating more attention should be paid to early diagnosis and treatment of pidisease, as well as more attention to their social limitation. pi patients may needpsychological consultation for better coping with their illness.