Prevalence of Primary Immunodeficiency Diseases and Related Risk FactorsAmong Children Attending a Hospital in Ahvaz, Iran

Primary immunodeficiency (PI) diseases are those occur because of a primarydisorder in the immune system.

This retrospective study was conducted on 70 children with PI diseases referred tothe allergy and immunology department of Abuzar Children Hospital in Ahvaz, Iran from 2015 to 2018.

The most common PI diseases in the majority of male children (56.92%) were severe combinedimmunodeficiency disease (21.42%) and chronic granulomatous disease (8.57%). Parents in most ofpatients (83%) were blood relatives. About 66.67% of all death cases (9.23%) was due to severe combinedimmunodeficiency disease; hypogammaglobulinemia and Griscelli syndrome accounted for about16.67% of deaths. The most common manifestations were pneumonia, fever, diarrhea, and sepsis.Based on the family history, 33 children had allergies, 9 had malignancy, one had autoimmune disease,and 39 had mothers with a history of abortion. Most of children were under treatment with intravenousimmunoglobulin and prophylactic antibiotics, (99.88% and 88.99%, respectively), while 2.81% were underinterferon-gamma therapy and transplantation, and 1.40% were receiving corticosteroids and granulocytecolony-stimulating factor therapy.

The severe combined immunodeficiency disease, chronic granulomatous disease, and Brutondisease are the most common PI diseases in children living in Ahvaz.