Abstract

Consanguineous marriages are the deeply rooted social trend in Pakistan, which lead to the high prevalence of inherited diseases. Thalassemia is an inherited disorder characterized by the abnormal synthesis of hemoglobin leading to anemia. β-thalassemia is the most common inherited disorder in Pakistan particularly in Sindh. The patients of β-thalassemia need repeated transfusion, which might cause complications causing the high rate of mortality in blood transfusion dependent β-thalassemia patients. Several studies have previously reported the high prevalence of β-thalassemia in Pakistan, however, the prevalence of β-thalassemia in the siblings borne from β-thalassemia carrier parents and association of ABO blood group with β-thalassemia have not been extensively studied. The purpose of this study was to find out the prevalence of β-thalassemia in the siblings of β-thalassemia carriers parents. This study was also set to find out the association of β-thalassemia with ABO blood groups. This was a cross sectional study carried out from August 2016 to July 2017 in the Thalassemia centers of district Dadu and district Hyderabad. In this study, 168 children from 50 families were selected for the study. The results indicate that almost half of the births 44.64% from β-thalassemia carriers are the β-thalassemia patients. B blood group was found to be higher in both in male and female β-thalassemia patients. Male had higher prevalence of β-thalassemia than female children. The pattern of ABO blood group distribution was B > O > A > AB, and for the RH factor it was RH + ve > Rh –ve. Conclusively, this study indicates the higher prevalence of β-thalassemia in siblings borne from β-thalassemia carrier’s parents. β-thalassemia was higher in male children and B blood group was higher than other blood groups.

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