Brazilian report on primary immunodeficiencies in children: 166 cases studied over a follow-up time of 15 years

Brazilian report on primary immunodeficiencies in children: 166 cases studied over a follow-up time of 15 years

Autor Grumach, A. S. Google Scholar
Duarte, AJS Google Scholar
BellinatiPires, R. Google Scholar
Pastorino, A. C. Google Scholar
Jacob, CMA Google Scholar
Diogo, C. L. Google Scholar
CondinoNeto, A. Google Scholar
Kirschfink, M. Google Scholar
CarneiroSampaio, MMS Google Scholar
Instituição Universidade Federal de São Paulo (UNIFESP)
Universidade Estadual de Campinas (UNICAMP)
Universidade de São Paulo (USP)
Resumo 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 São 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.
Assunto primary immunodeficiency
immunologic diseases
Idioma Inglês
Data 1997-07-01
Publicado em Journal of Clinical Immunology. New York: Plenum Publ Corp, v. 17, n. 4, p. 340-345, 1997.
ISSN 0271-9142 (Sherpa/Romeo, fator de impacto)
Editor Plenum Publ Corp
Extensão 340-345
Direito de acesso Acesso restrito
Tipo Artigo
Web of Science WOS:A1997XN54200009

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