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

Authors
  • Grumach, A S
  • Duarte, A J
  • Bellinati-Pires, R
  • Pastorino, A C
  • Jacob, C M
  • Diogo, C L
  • Condino-Neto, A
  • Kirschfink, M
  • Carneiro-Sampaio, M M
Type
Published Article
Journal
Journal of Clinical Immunology
Publisher
Springer Nature
Publication Date
Jul 01, 1997
Volume
17
Issue
4
Pages
340–345
Identifiers
PMID: 9258773
Source
Medline
Keywords
License
Unknown

Abstract

Over a 15-year observation period (1981-96), 166 cases of primary immunodeficiency disease (PID) were registered at the Department of Pediatrics, University of Sao Paulo, Brazil. PID was diagnosed according to World Health Organization criteria and only children with well-established deficiencies were included. The following frequencies were noted by PID classification: predominantly humoral defects (60.8%), T cell defects (4.9%), combined immunodeficiency (9.6%), phagocyte disorders (18.7%), and complement deficiencies (6%). The male to female ratio was 1.3 to 1. Immunoglobin A deficiency was the most frequent disorder (60 cases), followed by transient hypogammaglobulinemia (14 cases), chronic granulomatous disease (14 cases), and X-linked agammaglobulinemia (9 cases). Allergic symptoms occurred in 41% of cases. During the observation period, 23 children (13.8%) died, primarily of recurrent infections. Although improved diagnostic facilities have facilitated the recognition of immunodeficient children, the true incidence is likely to be higher than that detected in this study. Increased international collaboration is urged to improve the early detection of PID.

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