Descriptive clinical study of children with beta-thalassemia at Damanhour Medical National Institute

Abstract

Background Thalassemia is a genetic disorder of hemoglobin synthesis. Beta-thalassemia is particularly prevalent among Mediterranean people. Long-term transfusion therapy and iron chelating agents are the mainstay treatments of thalassemia patients. Aim The aim of this study was to identify the demographic, clinical, laboratory profile, and therapeutic features of B-thalassemia patients attending the Pediatric Hematology Clinic at Damanhour Medical Institute. Patients and methods This study included 74 thalassemia patients in the age range of 2–16 years, mostly from rural areas (el Behera Governorate). The total number of thalassemia patients followed up in the Pediatric Hematology Unit at Damanhour Medical Institute is up to 400 cases; the patients of this study were selected randomly from among them. Results Dysmorphic features were demonstrated in 44% of cases; 74% of cases were receiving packed red blood cells every month and 26% were receiving packed red blood cells more than once a month. Oral iron chelator (Deferasirox) was the main chelator used. Most of the patients had serum ferritin greater than 1000\u2009µg/ml; thyroid dysfunction was evident in 21% of cases, with no definite clinical manifestations.