Title: Epidemiological Clinical and Haematological Study of Beta Thalassemia (Homozygous) Pediatric Patients Treated In a Tertiary Care Centre in Odisha State
Authors: Bhagyalaxmi Das, Prafulla Kumar Mohanty, Rabindra Kumar Jena, Dharma Niranjan Mishra
DOI: https://dx.doi.org/10.18535/jmscr/v5i10.65
Abstract
Background: Beta thalassemia is an autosomal recessive disorder that affects red blood cells both in the decreased as well as absence in production of adult haemoglobin (HbA) and characterized by severe anaemia, splenomegaly and bone deformities and require lifelong transfusion therapy, iron chelation and/ or bone marrow transplantation for successful control. The aim of the study is to evaluate the haematological and epidemiological parameters in relation to socioeconomic status and quality of life of the study group.
Methods: The blood samples were collected from 100 diagnosed cases of beta thalassemia. Haematological and epidemiological parameters were studied by asking research questionnaire developed for this purpose and analyzing various clinical complaints and laboratory data. The haemoglobin variants were analysed by fully automated capillary zone electrophoresis to confirm the diagnosis of homozygous state of beta thalassemia.
Result: In the present study majority of the patients (81%) were below the age of 10 years. The clinical examination showed anaemia in all the patients with hepatomegally and spleenomegally in 15% and 90% cases respectively. Blood transfusions were required in 90% cases as 1-2 units per month. In coastal districts of eastern and northern odisha 82(82%) patients were observed in comparison to 18 (18%) cases in western odisha. The mean haemoglobin was markedly decreased to 5.4±1.8 ranges 3-8 gm% followed by fetal hemoglobin (HbF) was raised up to 83.58 ± 9.30% ranges from 70.2% to 94.5% and adult haemoglobin (Hb A2) was decreased upto 2.63 ±1.09 ranges from 1.1 to 4.3%.
Conclusion: The incidence of beta thalassemia is common in costal districts without having any correlation with caste, religion and financial status. Anaemia and spleenomegally is seen in all and 90% patients respectively. The facilities of molecular diagnosis and comprehensive control programme should be available in Odisha state.
Keywords: Epidemiology, Anaemia, haemoglobin variants, β-Globin Gene.
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