Abstract
Thalassemias and hemoglobinopathies are the most common hemolytic congenital disorders in Bangladesh as in many parts of the world. This study was done to find the common types of thalassemias and abnormal hemoglobin variants seen in Bangladeshi populations. A total of 4813 samples were analyzed for hemoglobin disorders out of which 2308 (49.95%) showed abnormalities. The samples were analyzed by Bio Rad D 10 Analyzer in 3914 (81.32%) cases, BIORAD VARIANTβ thalassemia short program using the principle of high performance liquid chromatography in 474 (9.85%) cases and by CAPILLARYS 2 FLEX PIERCING utilizing capillary electrophoresis in 425 (8.83%) cases. The samples were analyzed in the Department of Biochemistry and Molecular Biology of Dhaka Shishu (Children) Hospital, Dhaka, Bangladesh. The common hemoglobin disorders seen were β trait 863 (17.94%), Hb E trait 601 (12.50%), Hb E β thalassemia 524 (10.87%), β thalassemia major 192 (4.00 %), Hb E disease 99 (2.05%). Other Hb abnormalities detected were Hb D trait 17 (0.35%), Sickle cell trait 4 (0.08%), hereditary persistence of fetal hemoglobin (HPFH) 2 (0.04%), and Hb Lepore, δ β thalassemia, sickle cell β thalassemia, Sickle cell disease, compound heterozygote for HbE+D and Hb Q band one case each (0.02%).
Highlights
An estimate of World HealthOrganization shows that 7% of the world population are a carrier for hemoglobin disorders and 300,000 to 500,000 children are born with clinically significant hemoglobin disorders every year and majority of them [page 18]Key words: Thalassemia; hemoglobinopathies; high pressure liquid chromatography (HPLC); capillary electrophoresis.Contributions: WAK, concept and design; BB, article revising; SS, data collection; GS, data analysis
HbE β-thalassemia was the most common type of thalassemia seen in 524 cases
Sickle cell trait 4 (0.08%) hereditary persistence of fetal hemoglobin (HPFH) 2 cases (0.04%), and Hb Lepore, double heterozygote for Hb E +D and Q band all one case each (0.02%) (Table 1)
Summary
The aim of this study was to determine the types and frequencies of thalassemias and hemoglobinopathies seen in a tertiary level children hospital
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