Patterns of Primary Immunodeficiency Disorders Among a Highly Consanguineous Population: Cairo University Pediatric Hospital's 5-Year Experience.
J Clin Immunol
; 36(7): 649-55, 2016 10.
Article
em En
| MEDLINE
| ID: mdl-27484503
ABSTRACT
INTRODUCTION:
Primary immunodeficiency disorders (PIDs) are heterogeneous disorders that mainly present with severe, persistent, unusual, or recurrent infections in childhood. Reports from different parts of the world indicate a difference between Western and Eastern populations.AIM:
The aim of this study was to report on the different patterns of PIDs and identify subgroup characteristics in a highly consanguineous population in Egypt.METHODS:
We performed a retrospective chart review for children below 18 years diagnosed with PID at Cairo University Pediatric Hospital from 2010 to 2014.RESULTS:
Four hundred seventy-six children were diagnosed with PID disorders. Major categories included combined immunodeficiency disorders, which constituted a large proportion (30 %) of cases, along with predominantly antibody disorders (18 %) followed by syndromic combined disorders (16.8 %), phagocytic disorders (13.2 %), immune dysregulation disorders (10.5 %), and autoinflammatory disorders (9 %).CONCLUSION:
PIDs have different patterns within inbred populations with high consanguinity.Palavras-chave
Texto completo:
1
Bases de dados:
MEDLINE
Assunto principal:
Fenótipo
/
Consanguinidade
/
Síndromes de Imunodeficiência
Tipo de estudo:
Diagnostic_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
/
Screening_studies
Limite:
Child
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Child, preschool
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Female
/
Humans
/
Male
País/Região como assunto:
Africa
Idioma:
En
Revista:
J Clin Immunol
Ano de publicação:
2016
Tipo de documento:
Article
País de afiliação:
Egito