Frequency and Clinical Manifestations of Patients with Primary Immunodeficiency Disorders in Iran: Update from the Iranian Primary Immunodeficiency Registry

摘要: Primary immunodeficiency disorders (PID) are a heterogeneous group of diseases, characterized by an increased susceptibility to infections. A total 930 patients (573 males and 357 females) registered in Iranian PID Registry (IPIDR) during three decades. Predominantly antibody deficiencies were the most common (38.4%), followed congenital defects phagocyte number and/or function (28.3%), other well-defined syndromes (17.7%), combined T- B-cell immunodeficiencies (11.0%), complement (2.4%), diseases immune dysregulation (2.3%). Common variable was frequent disorder (20.8%), chronic granulomatous disease, ataxia-telangiectasia, btk deficiency, selective IgA T-B-severe immunodeficiency. The frequency less than 50 (<5%). There is increasing trend recognition more recent years. Construction such registry not only important for its epidemiological aspect but also role physician's knowledge about disorders.