Research Journal of Medical Sciences
ISSN: Online 1993-6095ISSN: Print 1815-9346
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Abstract
Thalassemia is an inherited disorder of autosomal recessive gene caused by impaired synthesis of one or more globin chains. The impairment alters production of normal hemoglobin (Hb). In people with beta thalassemia, low levels of hemoglobin lead to a lack of oxygen in many parts of the body. Affected individuals also have decreased red blood cells (anemia), which will manifest as pale skin, weakness, fatigue and serious complications. It is estimated that 1.5% of the world’s population are carriers of β‐thalassemia with an estimated 60,000 new carriers born each year. This is a prospective observational cohort study done in pediatric wards of two hospitals C. R. Gardi Hospital (C.R.G.H.) and associated hospitals, from September 2016 may 2018. Main aim of this study is: “Thalassemia major‐ ’Clinical profile, management, complication and outcome in tertiary care center’’. Out of 60 patients of thalassemia were enrolled and their demographic hematological profile was taken. The mean age was 10.2 years, 75% were males and 81% were Hindus, 40% belonged to upper lower class. Most common clinical features were (97%) icterus followed by (90%) pallor, 80% of thalassemia patients had moderate to severe hepatomegaly. Mean height was 105.77±14 cm, Mean Hb was 8.5±0.9 g dL-1. Mean ferritin level was 1281.8±219.9 ng dL-1 , 45% of thalassemic patients had serum ferritin level between 1000‐2000 ng dL-1 followed by 39% of patients had serum ferritin level more than 2500 ng dL-1, Mean frequency of blood transfusion 16±2 times a year, 55% of had blood transfusion 5‐10 times a year followed by 33% had frequency of blood transfusion 10‐15 times a year and only 12% had frequency more than 15 times a year, Mean interval between transfusion 22±2 days, 73% had an interval of 15‐25 days between two transfusion. About 86% had euglycemia and 10% had hyperglycemia when blood sugar was tested randomly. Tablet deferasirox was taken as chelating agent in all thalassemia patients, 51% of which were taking it after 2 years of age, 35% were had started between 1‐2 years of age and only 13% started below 1 year of age. The common adverse reaction in thalassemic patients were diarrhea (26.1%), abdominal pain (23%), skin rash (20%), seizures (16%), blurring of vision (3%). Hepatitis C virus infection was found in 76% and hepatitis B surface antigen (HBsAg) was positive in 48% of thalassemia patients. Two patients (4%) out of 60 thalassemic patients had decreased left ventricular ejection fraction of 35‐40%.
How to cite this article:
Mohit Sharma, Shreya Shrivastava, Sunil Kumar Kasundriya and Amit Kumar Singh. Thalassemia Major: Clinical Profile, Management, Complication and Outcome in Tertiary Care Center.
DOI: https://doi.org/10.36478/10.59218/makrjms.2023.503.511
URL: https://www.makhillpublications.co/view-article/1815-9346/10.59218/makrjms.2023.503.511