BRAZILIAN REPORT ON PRIMARY IMMUNODEFICIENCIES IN CHILDREN - 166 CASES STUDIED OVER A FOLLOW-UP TIME OF 15 YEARS

One hundred sixty-six cases of primary immunodeficiency diseases (PID) (95 males, 71 females), diagnosed according to WHO criteria, have bee n registered at the Children's Hospital, University of Sao Paulo, Braz il. The following frequencies were found: predominantly humoral defect s, 60.8% (n = 101); T cell defects, 4.9% (n = 8): combined ID, 9.6% (n = 16); phagocyte disorders, 18.7% (n = 31); and complement deficienci es, 6% (it = 10). IgA deficiency was the most frequent disorder (n = 6 0), followed by transient hypogammaglobulinemia (n = 14), chronic gran ulomatous disease (ii = 14), and X-linked agammaglobulinemia (n = 9). In comparison to other (national) reports, we observed higher relative frequencies of phagocyte and complement deficiencies. Recurrent infec tions were the cause of death in 12.7%. Allergic symptoms were observe d in 41%, mainly in IEA-deficient, hypogammaglobulinemic. or hyper-IEE patients, and autoimmune disorders in 5%, predominantly in IgA and co mplement deficiencies. Five patients suffered from BCG dissemination: two of them died. This is the first Brazilian report on PID over an ob servation time of 15 years.