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Background & Objective: Major ?-Thalassemia is the most common form of anemia, which has a relatively high prevalency especially in the northern part of the country one of this disease side effect the hearing abnormality. This study has been carried out in Gorgan for the determination of the hearing level of ?-Thalassemic patients, and its relation with the level of serum Ferritin, the rate and the duration of blood transussion and dyspheral. Materials & Methods: In this study 95 patients with major ?-Thalassemia have been studied for the rate of hearing level. The variation parameter include age, gender, Ferritin level, the rate and duration of disferal consumption. Audiometry, tempanometry and physical examination carried out on all the patients. The findings from this research gathered and were analyzed using the SPSS statistical software. Results: 95 patients (190 ears) with age 3-29 year of old were gone under this study and only 72 ears had the threshold over 15 decibel, from this 43.9% were from sensorineural type of hearing. The 80% of ears’s thempograms were type A. The results from this study showed that there is a meaningful statistical correlation between the hearing loss and serum Ferritin level. The rate of dyspheral consumption, in each time and its duration (P<0.05). Conclusion: This study showed that high serum Ferritin level and the increased length of dyspheral consumption lead to the hearing loss in major ?-Thalassemia, therefore clinical examination of hearing interrally has to be carried out.

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Background and Objective: Thalassemia major is a genetic disorder. Blood transfusion is critical for survival in these patients. Over the course of the past two and three decade’s hyper transfusion therapy in these patients has increased significant improvement in life expectancy and quality of life. Unfortunately this type of therapy increased the frequency of complication due to iron overloud. The aim of this study was to evaluate the prevalencey of diabetes, impaired fasting glucose and impaired glucose tolerance in patients with thalassemia major, with 10-27 years of age in Tabriz. Materials and Methods: This descriptive study was done on 56 patients between 10-27 years of age with thalassemia major. The demographic informaiton theraputic regiment, the age of first trasfussion. The level of blood transfusion, the history and dosage of familial history of diabetes, Fe, TIBC, ferritin levels were assessed and recorded. For each patient glucose tolerance test, blood glucose level are performed. Results: In this study prevalence of diabetes mellitus, impaired fasting glucose and impaired glucose tolerance test were found in 8.9%, 28.6% and 7.1% of patients respectively. Conclusion: This study showed that despite recent therapy with Desferal in the management of beta-thalassemia major, the risk of secondary endocrine dysfunction remains high. Prevalence of diabetes mellitus, impaired fasting glucose and impaired glucose tolerance test are greater than general population. Endocrine evaluation in patients with thalassemia major must be carried out regularly especially in those patients over the age of 10 years.

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Background and Objective: Thalassemias is the most common inherited disease in the world. This study was designed to assess general health of patients with beta-thalassemia major in Gorgan, northern Iran. Materials and Methods: This descriptive study was performed on 163 patients with beta-thalassemia major whom referred to Thalghani hospital in Gorgan, northern Iran. Socio-Psychosocial status of patients was assessed by using general health questionnaire-28 (GHQ-28). Results: Socio-Psychosocial status of 44.2%, 46% and 9.8% of patients was normal, suspected and suffered from psychosocial disorders, respectively. Social dysfunction (12.9%) and depression (10.4%) were the most prevalent and anxiety was the less prevalent (4.9%) in the patients. There were significant correlations between psychosocial status and mother’s ocupation (P<0.05), patients level of education (P<0.05) and history of associated diseases (P<0.05). Conclusion: Social dysfunction and depression were the two major general health complications in beta-thalassemia patients in this region.

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Background and Objective: Deferoxamin is the current “gold standard” chelator in comparison with new chelators. Combined therapy of Deferiprone and deferoxamin reduces the cardiac iron overload in patients with major talassemia. This study was done to evaluate the effect of defriprone-deferoxamine on heart function in patients with major thalassemia. Methods: In this historical cohort study, 8 patients with major beta thalassemia treated by subcutaneous deferoxamine were randomly selected and LVEF (the rate of blood that exited from heart in each beat) and serum ferritin were measeared. The patients were treated by deferiprone (50-100 mg/kg/day) compained with dferoxamine (30-50 mg/kg as 3 times in a week). In the end of each year, LVEF and serum ferritin of patients were measured. Results: The ferritin level changed from 3243.12 in the first year to 2672.75 mg/kg at the end of third year. The mean of LVEF changed from 71.12% to 64.62 %. The correlation of serum ferritin and LVEF only at the end of third year was significant (P<0.05). Conclusion: Combined therapy of deferiprone-deferoxamine during 3 years reduces ferritin and LVEF in patients with major thalassemia.