ABSTRACT

BACKGROUND/AIMS

Primary antibody deficiencies (PADs) are the most common group of primary immunodeficiency disorders that present with a wide range of clinical features. We conducted this study to evaluate the frequency and characteristics of patients with PADs.

MATERIAL and METHODS

The medical records of 160 children (M:F 99:61, mean age 42.39±28.44 months) were retrospectively reviewed from the Near East University Hospital database. Children who had PADs were then compared according to their demographic, clinical, and laboratory findings.

RESULTS

A total of 97 children were diagnosed with PADs (49 THGI, 31 IgGD, 1 SIgAD, 8 CVID, 6 IgMD, and 2 PHGI)*. Patients with THGI had the youngest age at the time of diagnosis (27±16.40 months), and those with CVID had the oldest age at the time of diagnosis (81.62±49.9 months old) compared with children in other PAD groups. In the HGG group, 63.9% of children presented with both recurrent wheezing/ cough and infections, 22.7% had only recurrent infection, 13.4% of them had only recurrent wheezing/cough, and 71.7% of the children were atopic. At the end of the 2-year follow-up period, 8 patients (5 THGI and 3 IgGSD) out of 70 with HGG recovered.

CONCLUSION

PADs generally present with recurrent infections and/or recurrent bronchoconstriction unresponsive to standard asthma treatment. Evaluation of the immune system is important to increase the quality of life of these patients and also decrease the healthcare costs.