CLINICAL PROFILE OF CHILDREN WITH THALASSEMIA MAJOR AGED 5 TO 14 YEARS
Ipsita Jena, Sristi Ganguly* and Narendra Nath Soren
ABSTRACT
Background: Thalassemia is one of the commonest genetic disorders in India, causing children to be transfusion dependent. Despite high burden, there is delay in recognition, screening, diagnosis and adequate management, making study on their clinical profile a priority in Odisha. Methodology: This was a hospital based cross-sectional study done in SCBMCH and SVPPGIP, Cuttack during September 2019 to January 2020. Diagnosed cases of Thalassemia major patients aged 5 to 14 years, on blood transfusion were included in the study and those with other hemoglobinopathies or chronic illness not attributed to thalassemia were excluded. The demographic and clinical characteristics of the patients noted and data analysed using SPSS and expressed in percentages. Results: Among the 200 patients studied, male to female ratio was 2.03, with mean age 7.9 years. 71.5% of the study population had malnutrition, with 39% having stunting and 21% having both stunting and wasting. The average pre-transfusion haemoglobin was 6.3g/dl, age at diagnosis of 53% was between 6 to 9 months. Most (66%) of the cases received blood transfusion at frequency of once a month. Majority (40%) had their ferritin values >2000 (average-2096.45 ng/ml).Fatigue (56%) and splenomegaly (97.5%) were the most common symptom and clinical finding seen. 79% and 63% had good compliance and good knowledge about the disease respectively. No mortality was seen in our population, though 3.5% had complications. Conclusion: Owing to the high prevalence, measures for early detection, prompt diagnosis and optimum management of thalassemia patients, with easy access to services and education of masses is the need of the hour.
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