Artículos de revistas
Brazilian report on primary immunodeficiencies in children: 166 cases studied over a follow-up time of 15 years
Registro en:
Journal Of Clinical Immunology. Plenum Publ Corp, v. 17, n. 4, n. 340, n. 345, 1997.
0271-9142
WOS:A1997XN54200009
10.1023/A:1027335000994
Autor
Grumach, AS
Duarte, AJS
BellinatiPires, R
Pastorino, AC
Jacob, CMA
Diogo, CL
CondinoNeto, A
Kirschfink, M
CarneiroSampaio, MMS
Institución
Resumen
One hundred sixty-six cases of primary immunodeficiency diseases (PID) (95 males, 71 females), diagnosed according to WHO criteria, have been registered at the Children's Hospital, University of Sao Paulo, Brazil. The following frequencies were found: predominantly humoral defects, 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 deficiencies, 6% (it = 10). IgA deficiency was the most frequent disorder (n = 60), followed by transient hypogammaglobulinemia (n = 14), chronic granulomatous 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 infections were the cause of death in 12.7%. Allergic symptoms were observed in 41%, mainly in IEA-deficient, hypogammaglobulinemic. or hyper-IEE patients, and autoimmune disorders in 5%, predominantly in IgA and complement deficiencies. Five patients suffered from BCG dissemination: two of them died. This is the first Brazilian report on PID over an observation time of 15 years. 17 4 340 345