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Objective: Genetic factors contribute significantly to type 1 diabetes (T1D) etiology. A single nucleotide polymorphism in the CD226 gene (rs763361 C>T) has been associated with T1D susceptibility in European patients, but data from other populations is limited. Our aim was to study the contribution of this polymorphism to T1D susceptibility among Egyptian children., Methods: A case-control study including 74 children with T1D and 82 healthy children as a control group. Genotyping of CD226 gene polymorphism was performed for all participants by DNA extraction followed by polymerase chain reaction and restriction fragment length polymorphism., Results: The frequency of T allele was 78.4% in patients and 68.3% in controls (OR, 1.68; 95% CI, 1.01-2.8; P=0.046). TT, TC, and CC genotypes were found in 62.2%, 32.4%, and 5.4% of the patients, respectively, and in 41.5%, 53.7%, and 4.9% of controls, respectively. Under the recessive model, TT genotype was significantly associated with T1D risk (OR, 2.32; 95% CI, 1.21-4.41; P=0.010). The mean age at diabetes onset was significantly lower in patients carrying T allele compared with C allele (8.03±3.8 year vs. 10.5±2.54 year; P<0.001) and among those with TT genotype compared with the pooled TC+CC genotypes (7.5±2.6 year vs. 10.6±2.6 year; P<0.001). No significant difference was found between genotypes or alleles regarding the HbA1c level., Conclusion: T allele and TT genotype of the CD226 rs763361 polymorphism is associated with susceptibility to T1D and with a lower age of disease onset among Egyptian children., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)