Primary Immunodeficiency Diseases in Different Age Groups: A Report on 1,008 Cases from a Single Brazilian Reference Center

Primary immunodeficiencies (PIDs) represent a large group of diseases that affect all age groups. Although PIDs have been recognized as rare diseases, there is epidemiological evidence suggesting that their real prevalence has been underestimated. We performed an evaluation of a series of 1,008 infa...

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Bibliographic Details
Published in:Journal of clinical immunology 2013-05, Vol.33 (4), p.716-724
Main Authors: Carneiro-Sampaio, Magda, Moraes-Vasconcelos, Dewton, Kokron, Cristina M., Jacob, Cristina M. A., Toledo-Barros, Myrthes, Dorna, Mayra B., Watanabe, Letícia A., Marinho, Ana Karolina B. B., Castro, Ana Paula Moschione, Pastorino, Antonio C., Silva, Clóvis Artur A., Ferreira, Maurício D., Rizzo, Luiz V., Kalil, Jorge E., Duarte, Alberto J. S.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Age Factors
Biomedical and Life Sciences
Biomedicine
Brazil
Child
Child, Preschool
Female
Humans
Immunoglobulin A - genetics
Immunoglobulin M - genetics
Immunologic Deficiency Syndromes - epidemiology
Immunologic Deficiency Syndromes - immunology
Immunology
Infant
Infant, Newborn
Infectious Diseases
Internal Medicine
Male
Medical Microbiology
Original Research
Phagocytes - pathology
Population Groups
Prevalence
Sex Factors
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Summary:Primary immunodeficiencies (PIDs) represent a large group of diseases that affect all age groups. Although PIDs have been recognized as rare diseases, there is epidemiological evidence suggesting that their real prevalence has been underestimated. We performed an evaluation of a series of 1,008 infants, children, adolescents and adults with well-defined PIDs from a single Brazilian center, regarding age at diagnosis, gender and PID category according to the International Union of Immunological Societies classification. Antibody deficiencies were the most common category in the whole series (61 %) for all age groups, with the exception of 30-year-old group, antibody deficiencies comprised 84 % of the diagnoses, mostly consisting of common variable immunodeficiency, IgA deficiency and IgM deficiency. Combined immunodeficiencies represented the most frequent category in
ISSN:0271-9142
1573-2592
DOI:10.1007/s10875-013-9865-6