Prevalence of Primary Immunodeficiency Diseases in Kerman, Southeast of Iran

Introduction Primary Immunodeficiency Diseases (PID) are rare and heterogeneous congenital diseases leading to increased unusual susceptibility to developing infections and causing some malignancies and autoimmune diseases. This study was conducted to evaluate characteristics of these diseases in patients attending the clinic of immunodeficiency diseases in Kerman. Materials and Methods In a case series study from 2003 to 2014 in the clinic of immunodeficiency diseases in Afzalipour Hospital in Kerman, 32 patients with primary immunodeficiency disease were included. Data was analyzed by statistical software SPSS-19. The level of significance was considered P<0.05 in all cases. Results The mean age at the onset of symptoms was 4.45 +/- 4.35 years. About 60% of patients had phagocytic disorders. Antibody deficiencies were seen in approximately 20% of patients. X-linked agammaglobulinemia was reported in about 6.5% of patients. About 75% of parents were relatives. Developing pneumonia was observed as the most common infection in about 70% followed by adenitis which existed in 47% of cases. Conclusion This study revealed that intra-family marriages can be a risk factor for the development of PID in children. So it is essential to recommend genetic counseling before marriage and pregnancy in addition to improving awareness of families with known disease.