Abstract

 Background and Objectives

 Beta major thalassemia is one of the most prevalent diseases in Iran where 20000 affected patients live They should receive regular blood transfusion and desferal iron chelation to lead a normal quality of life. In this study, we evaluated transfusion and laboratory status of those patients referred to Mofid Children Hospital during the year of 2006-07.

 Materials and Methods

 In this retrospective analytic-descriptive study, transfusion status of 121 patients referred to Mofid Children Hospital during 2006-07 was evaluated through their medical records. SPSS software and Mann-Whitney and Spearmann tests were used for data analysis.

 Results

 121 patients within the age range of 2-26 (average of 13 ± 6.19) years, including 66 (54.5%) females and 55 males (45.5%) participated in the present study. Their pretransfusion hemoglobin level was in the range of 7.5-11.5 g/dl (average of 9.6 ± 0.97 g/dl). In 82 patients (67.8%), hemoglobin level was above 9 g/dl. Serum ferritin level was 250-9500 ng/ml (average of 2133 ± 1515 ng/ml). Serum folate was checked in 100 patients out of whom only 3% were below 3 ng/ml (normal reference range: 3-17.5 ng/ml). Positive HCV antibody and HCV RNA (PCR) was detected in 11 (9.1%) of patients who were between 16 to 26 years old, but only one (0.8%) had positive HBS antigen. HCV RNA (PCR) test was also positive in the patients. No patients were detected with HIV antibody. Serum ferritin level in hepatitis C patients fell within the range of 568-9500 ng/ml with the average of 3172 ± 2772 ng/ml.

 Conclusions

 It seems that beta major thalassemic patients in Mofid Children Hospital use appropriate blood transfusion, and iron chelation agents they are also tested for transfusion transmitted infections. Precise control over blood transfusion and use of iron chelation agents, blood donor screening for transfusion transmitted infections especially hepatitis C, and vaccination against hepatitis B are important in improvement of the status of beta major thalassemics, but prevention is yet the most effective way to reduce the incidence disease and it's of complications.

 �

 Key words : Beta thalassemia, Ferritin, Desferal, Hepatitis C