ABSTRACT
The thalassemia's are a heterogeneous group of genetic disorders in which the production of not-ma1 hemoglobin is partly or completely suppressed because of a defective synthesis of one or more globin chain-In Iraq, B - thalassemia major is widely distributed all over the country. An active program for the hereditary blood diseases had been adopted in cooperation with WHO in 1989. This study is done to evaluate the burden of the disease and its treatment on thalassemic patients in Ramadi. Thirty-one children with B- thalassemia major [17 males and 14females] aged 9 months- 21 years attending the thalassemic clinic in MCH in Ramadi during the period from 1[st] Dec. 2001 to 31[st] May 2002 were studied prospectively. _History, clinical examination, investigations, treatment and its complications were noted and analyzed. Statistical analysis was done by the use of SD, t-test and P value of <0.05was considered statistically significant. 90% had pretransfusion Hb <9gm/ dl. Only 27% received Desferal regularly subcutaneously by infusion pumps. Splenectomy was done in 19.3%, all of them received pneumococcal vaccine and half of them received Benzathine penicillin regularly monthly. Body weight and height below 3rd percentile were noticed in 22.5% and 32.2% respectively. About 32% had myocardial systolic or diastolic dysfunction detected by Doppler echocardiography. None had hypocalcaemia. HBs Ag positive in 6.4%. Anti HCV was positive in 12.5%. None was positive for HIV. _The majority of patients were undertreated and various complications were probably related to chronic anemia rather than iron overload