STUDY OF CLINICAL PROFILE OF CHILDREN WITH SICKLE CELL ANEMIA AND EVALUATION OF CARDIAC FUNCTION IN THEM USING 2D ECHOCARDIOGRAPHY

Abstract

Introduction: Sickle-cell anemia (SCA), a severe form of sickle-cell disease, is a global hemoglobinopathy with a signicant on affected individuals. The disease is characterized by a single point mutation in the β-globin gene, leading to the synthesis of unstable sickle haemoglobin (HbS). This results in HbS polymerization, oxidative stress, inammation, and endothelial activation. Despite advancements in treatment strategies, mortality remains high in this population. Cardiac complications, although recognized, are not well-explored. This study aims to investigate the clinical prole of children with SCA and assess cardiac function using 2D echocardiography. Aims & Objectives: 1.To assess clinical prole of children with sickle cell anemia 2.Evaluation of cardiac function in them using 2D Echocardiography. Materials and Methods: This prospective observational study was conducted at a Government General Hospital, enrolling children aged 5 to 12 years with SCA.Detailed demographic information, family history, and clinical data were collected. All enrolled children underwent investigations, including complete blood count (CBC) and 2D Echocardiography was performed by a cardiologist. Data was analysed with appropriate statistical methods. Results: The study included 100 children with SCA, with a mean age of 8.7 years. Clinical presentations revealed a prevalence of vascular occlusion (73%), splenomegaly (14%), infections (7%), and hepatomegaly (6%). Electrocardiographic parameters indicated sinus bradycardia (24%), left atrial enlargement (19%) and diastolic dysfunction (84%). Mean values of echocardiographic parameters were also reported. Hydroxyurea therapy showed statically signicant improvement in haemoglobin levels, number of blood transfusions, hospital admissions and other clinical parameters. This study provides valuable insights into the clinical prole especially cardiac abnor Conclusions: malities among children with SCA.All SCA childrens should be routinely screened for cardiac abnormalities and counselled appropriately. The clinical benits of hydroxyurea therapy highlights the importance of early initiation of this drug in all childrens with SCA.