ABSTRACT
The primary immunodeficiency [PI] disorders are abnormalities in development and maturation of the immune system. Individuals with PI disease may experience frequent infections, which limit their abilities to exhibit physical and psychological well-being secondary to their illness. In this survey we compared health-related quality of life of primary immune deficient patients with healthy children. The case-control study was designed for patients with PI disease who were referred to Children Medical Center in 2004-2005. Demographic information was taken and Pediatric Quality Of Life [PEDQOL] questionnaire were filled for 50 PI patients and 100 healthy children. The mean age in PI patients was 12.62 +/- 3.65 [range from 8 to 18] years and in the control group was 11.04 +/- 3.3 years. In PI patients 68% were male and 32% female .Most patients with PI disease had a diagnosis of common variable immunodeficiency [54%] or X-linked agammaglobulinemia [24%]. Patients with PI disease had great limitations in physical functioning and psychological well-being [p<0.001 and p<0.001 respectively] compared with children without a chronic health condition. Patients had lower PEDQOL scores in all age groups compared with normal sample [p<0.001]. Long duration of disease significantly correlated with low psychological score. [r=-3.23. P=0.03] Children with PI disease experience poorer health related quality of life than healthy children, indicating more attention should be paid to early diagnosis and treatment of PI disease, as well as more attention to their social limitation. PI patients may need psychological consultation for better coping with their illness