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Background: Sickle cell anemia (SCA) is a genetic disorder caused by homozygosity for a single β-globin gene mutation (β6GAG→GTG), in which glutamic acid has been substituted for valine at the sixth codon of β-globin chain. Despite this fact, the clinical course of patients suffering from SCA is extremely variable, the severity of manifestations ranging from asymptomatic to a very severe course. The phenotypic variability maybe explained by some genetic factors, those related to globin genes have been well recognized. Severity of sickle cell anaemia (SCA) results from sickling of Hb S due to oxidation, which is augmented by accumulation of oxygen-free radicals. The glutathione system plays an important role in the removal of endogenous products of peroxidation of lipids, thus protecting cells and tissue against damage from oxidative stress. Impairment of the glutathione system due to genetic polymorphisms of glutathione S-transferase (GST) genes is expected to increase the severity of SCA manifestations. Objectives: This study aimed to determine the frequency of GSTM1 gene polymorphisms and its association with the severity of SCA among Sudanese patients. Materials and Methods: GSTM1gene polymorphisms were determined by multiplex polymerase chain reaction (PCR). Results: The frequency of GSTM1 null was higher in SCA patients compare with the positive gene patients. No significant correlation was found between the GSTM1 null age and gender (P.value >0.05). On the other hand a significance difference in hemoglobin concentration, packed cell volume and red cell count results between GSTM1 null patient's and GSTM1positive patients (P.value< 0.05). Conclusion: The GSTM1null were significantly associated with increased severity of sickle cell anemia among Sudanese patients.
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