Two years follow up of the patients who recieved IVIG treatment due to primary immune deficiency

Abstract

INTRODUCTION: Primary immune deficiency are observed frequently in countries such as Turkey where relative marriage rates are high. In this study, the demographic and clinical properties of our patients have been presented who have received IVIG treatment at our center due to primary immune deficiency. METHODS: The patients who received IVIG treatment at the Inonu University Faculty of Medicine Turgut Ozal Medical Center Pediatric Allergy and Immunology Clinic during 2013-2015 with primary immune deficiency diagnosis were examined retrospectively. TRESULTS: 16 patients who underwent IVIG treatment due to primary immune deficiency were included in the study. 9 %56 of the patients were girls. The mean age of the patients was 9 1-17 years , whereas the mean age of diagnosis was 3 7 months – 8 years . 9 56% of the patients had a history of relative marriage, whereas 5 31% had a history of primary immune deficiency in the family. 4 25% of the 16 patients who received IVIG treatment were diagnosed with AT, 4 25% were diagnosed with unclassified hypogammaglobulinemia, 2 12% were diagnosed with AKIY, 2 12% were diagnosed with XLA and one patient was diagnosed respectively with Ig G sub group deficiency, CVID, Hyper Ig M syndrome, Protein kinase C deficiency. The most frequently recurring complaints of the patients were infections. Whereas the most frequently affected system was the lower respiratory system. Dermatitis findings accompanied the infections in 2 12% patients. There was a significant decrease in the infection prevalence in all patients following the IVIG treatment. One patient was exitus during the follow up. DISCUSSION AND CONCLUSION: Primary immune deficiency should be considered for the pre-diagnosis of patients who complain of frequent infections and who have a history of relative marriage and family history. Complications that might arise due especially to infections can be prevented with an early diagnosis