Role of L-Carnitine in Treatment of β-Thalassemia Major Disease

Abstract

β-thalassemia syndromes are a group of hereditary disorders characterized by a genetic deficiency in the synthesis of β-globin chains. L-Carnitine is often sold as a nutritional supplement. L-Carnitine has been proposed as a treatment for many conditions because it helps reducing oxidative stress. This study was carried out on 30 β-thalassemia major patients (15 females), aged from 1.3 to 14 years old and their weights ranged from 7 kg to 32 kg; collected from hematology clinic at Beni Suef University Hospital. Group A received dose 100mg\kg\day, group B received dose 50mg\kg\day, control group received L-Carnitine but with an inadequate dose or in irregular manner. Patients were represented to full history taking, clinical examination, laboratory investigations and determination of blood transfusion frequency on admission and every month for six months. There was an improvement in general health of children after therapy, an improvement in pallor, decreasing in elevation of serum ferritin level, decreasing in blood transfusion times, increasing in blood transfusion intervals and increasing in hemoglobin level. The increase in Reticulocyte count% (retics count %) was in all groups, more in control group, but still with no significant difference between the three groups. Using of dose 100mg\kg\day of L-Carnitine showed more improvement than dose 50mg\kg\day in pallor and laboratory findings except retics count % and more effective in reduction of times of blood transfusion.