Report of the Tunisian Registry of Primary Immunodeficiencies: 25-Years of Experience (1988-2012).
J Clin Immunol
; 35(8): 745-53, 2015 Nov.
Article
em En
| MEDLINE
| ID: mdl-26464197
ABSTRACT
PURPOSE:
Primary immunodeficiencies (PIDs) are a large group of diseases characterized by susceptibility to not only recurrent infections but also autoimmune diseases and malignancies. The aim of this study was to describe and analyze the distribution, clinical features and eventual outcome of PID among Tunisian patients.METHODS:
We reviewed the record of 710 patients diagnosed with Primary Immunodeficiency Diseases (PIDs) from the registry of the Tunisian Referral Centre for PIDs over a 25-year period.RESULTS:
The male-to-female ratio was 1.4. The median age at the onset of symptoms was 6 months and at the time of diagnosis 2 years. The estimated prevalence was 4.3 per 100,000 populations. The consanguinity rate was found in 58.2 % of families. According to the International Union of Immunological Societies classification, spectrums of PIDs were as follows combined T-cell and B-cell immunodeficiency disorders account for the most common category (28.6 %), followed by congenital defects of phagocyte (25.4 %), other well-defined immunodeficiency syndromes (22.7 %), predominant antibody deficiency diseases (17.7 %), diseases of immune dysregulation (4.8 %), defect of innate immunity (0.4 %) and complement deficiencies (0.4 %). Recurrent infections, particularly lower airway infections (62.3 %), presented the most common manifestation of PID patients. The overall mortality rate was 34.5 %, mainly observed with combined immunodeficiencies.CONCLUSION:
The distribution of PIDs was different from that reported in Western countries, with a particularly high proportion of Combined Immunodeficiencies and phagocyte defects in number and/or function. More is needed to improve PID diagnosis and treatment in our country.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Linfócitos B
/
Linfócitos T
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Sistema de Registros
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Síndromes de Imunodeficiência
/
Anticorpos
Tipo de estudo:
Prevalence_studies
/
Risk_factors_studies
Limite:
Female
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Humans
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Infant
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Male
País/Região como assunto:
Africa
Idioma:
En
Revista:
J Clin Immunol
Ano de publicação:
2015
Tipo de documento:
Article
País de afiliação:
Tunísia