Background and Aim: Thalassemia is the name of a group of genetic blood disorders. Thalassemia is an inherited disease of the red blood cells, classified as a hemoglobinopathy. In the research patients were selected major thalassemia for aldolase, hexokinase enzymes activity level, Serum Ferritin arterial blood oxygen were measured.Materials and Methods: In the experimental research, 50 patients were selected among 700 patients with major thalassemia who were treated in Sayedolshohada hospital in Isfahan. Serum Ferritin, arterial blood oxygen and Aldolase – Hexokinase enzymes activity level were measured in these patients.Results: A comparison was performed between mean level of serum ferritin, mean level of arterial blood oxygen, and activity level of Aldolase- Hexokinase enzymes.Conclusion: Mean level of Serum Ferrtin in 50 patients with major THALASEMIA 237 µg/ml was made. According to the findings the increase of Aldolase- Hexokinase is increase with the increase of Serum Ferritin level.

Background: As universal leukocyte (WBC) reduction (VIR) is being considered as a new standard, few data are available on the performance of WBC- reduction filtration in routine practice the performance of WBC- reduction in RBCs, using varied filtration practices, in meeting the current FDA requirement (<5×106), council of Europe(EC) recommendation , the proposed FDA requirement (<1×106). And more stringent proposal (<5×105) for residual WBCs per RBC unit was assessed and compared. Methods: Our study was descriptive analytic.We studied 51 patient with major talasemia by 3 ways: with filter pre storage and bedside and without filter. The times of infusion, average of Hb, blood volume and blood volume requested (cc/kg) were studied the 3 ways. Results: The average results in the 3 ways were not same (by T_test). the average in 2 ways (without filter and Bedside) were same (p_value=0.744) and in the ways without filter and pre storage were same (p_value=0.001) and in the pre storage and bedside ways were markable (p_value=0.001) The Hb average in the 3 ways were not significant (p_value=0.101).patients had the same HB. There is significant difference in blood volume in 3 ways (p_value<0.001) by T_test. The average volume was attentionable by 2 ways. p_value<0.001, p_value<0.002 There is significant difference inaverage blood volume requested (cc/kg) in 3 ways methods. Without filter and Bedside (p_value=0.002). and in the other methods p_value<0.001. the result is no different in pre storage and bedside (p_value=0.164).

Background: Restless legs syndrome (RLS) is an annoying sensation and an important cause of sleep disturbances in major thalassemia.Materials and Methods: In a cross-sectional study in 58 major thalassemia patients aged 3 to 25 years in Tabriz children hospital, restless legs syndrome prevalence was evaluated.Results: RLS is a common problem in major thalassemia with prevalence of 24%. It included the most common reason of sleep disorder in thalassemia patients (83%). Restless legs syndrome; is not related to ferritin or iron level and age. Although RLS is more common in females, it is not statistically significant.Conclusion: RLS is a common disorder in thalassemia patients but usually unnoticed by physicians and nursing.

Introduction: The b-thalassaemias represent a heterogenous group of diseases resulting from decreased b-globin mRNA expression and imbalanced alpha/beta globin chain synthesis which are clinically manifested by ineffective erythropoiesis and excessive hemolysis. Increasing levels of hemoglobin F (HbF) by improving the balance in globin chain synthesis. Hydroxyurea (HU), as an effective with low toxicity for activatingy-globin gene, has been shown to enhance HbF synthesis.Methods: To analyze the clinical presentation and possible side effects of HU treatment 80 thalassemia intermedia patients were selected and followed-up for one year regularly. All of them started their regular blood transfusion beyond of 2 years of age and were being treated with 10-12mg/kg/d HU orally.Results: The study group consists of 38 men and 42 women. The average age was 16.8(±6.9) years and thalassemia was diagnosed according to the RBC indexes and Hb electrophoresis. An improvement of erythropoiesis was reflected by an increase in hemoglobin concentration, the state of energy; fatigability, and mood. About 83% (69) of the patients tolerated the HU well and showed a dramatic response to the drug. Thirty of 80 transfusion-dependent patients became completely transfusion-free and 23 cases had one or two transfusions throughout the study.Vomiting and thrombocytopenia were seen in two patients.Conclusion: During this close observation, no malignant change was seen in our patients. HU therapy appears to be safe and effective when administered in thalassemic patients.

Introduction: there are about 23000 THALASEMIA patients in Iran, however 1800 patients are added to each year, but its mortality rate is not clear. In spite of the development in medical sciences in recent years and new transfusion methods and iron removing as the basic treatment and some factor such as anemia and its side effects. Iron increase, late diagnosing, transfusion side effects shortage of equipments and treatment centers is a big and expensive problem for the patients and their 2 to 3 decades of painful life is not lasted any more and kill them soon. A way out must be taught, we should help the patients with better and more equipments and their parents with proper teaching, not to watch their life shading.Methodology: this research is a semi experimental of two variables in two groups and is done to survey teaching impact on THALASEMIA children mothers for physical cares from children. the samples were all major the THALASEMIA mothers referring to mashhad treatment centers in two groups of case and control. The data was collected by an interview sheet paper. 25 samples referring to imam Reza hospital as the case group and 26 referring to Gheam and 17 shahrivar hospitals as the control group. After the first test teaching program was done for both of the groups. The data was analyzed by proper soft wares.Findings: the results showed that average of the scores in case group in the first test 5.48 and in control group was 6.23, but after teaching program it increased to 13.56 in the case and 6.27 in control group that showed the significant teaching impact in the case group. T test was used for meaningful teaching impact and showed that there is a meaningful relation between teaching and scores in the case group (P< 0.001). T test was used also for scores difference in two groups and it showed that teaching has a positive impact in the case group.Conclusion: considering the finding, teaching of patients and their parents in care and controlling fields is a necessity and supplement of nursing services. Then it is a duty of men of responsible to care and pay more attention to teaching programs and get start and effective steps to ward economic, social and mental conditions of the families.

Background: Major thalassemia is relatively common in Iran and in looking of their need to recurrent transfusion; their high risk for acquisition of HCV is revealed. These patients also suffer form liver hemosiderosis that accelerate disease excursion to cirrhosis and hepatocellular carcinoma. In this study combined therapy with interferon and amantadin has been evaluated.Methods: This prospective clinical trail has been done on thalasemic patients that had been contaminated by HCV and had laboratory signs of hepatitis. During years of 81-82, 26 patients admitted at Children Mmedical Center with major thalassemia and HCV hepatitis. Inclusion criteria were positive HCVRNA, high ALT and histologic evidence of hepatitis in liver biopsy and exclusion criteria were history of incomplete treatment any contraindication of IFN or amantadin-emergence of drugs adverse reactions, to intending of these criteria 10 case of them had situation for treatment and follow-up.Results: In 10 cases, treatment with IFN α - 2b in doses 3 mu for every square meter of body surface three times in week subcutaneously and cap. Amantadin in doses 100 mg po B.d for 6 months was done and after it, 8 cases were negative for HCV-RNA (8%) and in 6 patients, ALT turned to normal (60%) and in 2 other case ALT decrease to lower than 50% of pretreatment value. None of them showed drug adverse reactions and response to therapy was better in lower ages. No relation between response to therapy and liver hemosiderosis, inflammation and sexuality was found.Conclusion: Combined therapy with IFN and Amantadin is effective in HCV treatment and for lower recurrence, treatment period longer than 24 wk, such as 48 wk is recommended.

The aim of this study was the evaluation of hearing threshold in patients with beta THALASEMIA major. For this purpose, 178 patients who were under regular chelation therapy with desferrioxamine (DFO) were selected and underwent ear, nose, throat examinations and audiological evaluations. Patients were between 4 to 25 years old and 54.9% were male and 45.1% were female. Three patients were excluded from the study due to middle ear disease and tympanic membrane perforation. Mean hearing threshold was 12.46db. The incidence of hearing loss in speech frequencies was 1.8% and there was a correlation between hearing threshold increase and the duration of disease and desferrioxamine consumption. But no correlation was found between hearing threshold and serum ferritin level (mean 4046 ng/ml) or DFO dose (mean=38.68mg/kg/day).

Background: Hepatitis B is a major problem occurred in patients with THALASEMIA major due to repeated blood transfusion. Regarding its acute (fulminant hepatitis) and chronic (cirrhosis, hepatic neoplasm) complications, vaccination could be helpful. As time elapsed, the antibody level is diminished. The present study was conducted to define the exact time of booster vaccination to be generalized as a uniform guideline for thalasemic patients. Materials and Methods: For this historical cohort study, 50 patients with THALASEMIA major and 50 normal subjects who have received hepatitis B vaccine were selected. HBS-Ab was determined using ELISA technique. Then, reduced antibody level was judged with elapsed following vaccination. Results: In the normal subjects, 8 years following the vaccination, there exist a suitable level of antibody (99.5±5 U/l), however, in the case group, those who have received vaccine within the recent 7 years have still suitable level of antibody (90±5 U/l), but others (time elapsed following vaccination was greater than 7 years) lacked sufficient level of antibody (12.5±2.5 U/l) (P<0.01). Conclusion: We recommend determining the antibody level 7 years following the initial vaccination in patients with THALASEMIA major, so that if sufficient level is absent, booster vaccination should be applied.

Introduction: hypochrome microcytic anemia is one of the most common types of anemia which is mostly due to iron deficiency and minor thalassemia.Differentiation of these two diseases, from therapeutic and preventive points of view, is very important. There are different way of differentiate these disease that some of them are expensive and inapplicable in most of laboratories.Our aim is to study hematological indices in these two groups to determine the sensitivity and specificity of them and recommend the easiest and most reliable diagnostic method.Material and Method: This diagnostic case series study was done on 125 hypochrome microcytic anemia patients referring to shahid sadughi hemotologic clinic from 2005 until 2007. 17 cases were excluded as they were under trearment.Serum iron, TIBC, serum ferritin and electrophoresis of all 108 recruited patients were checked. According to the laboratory finding, patients were divided into 3 minor THALASEMIA (40 cases), iron deficiency (50 cases) and mixed (18cases) groups. The last group was excluded from our study.Finally 90 patients were included and all hematological indices were check through a CBC test evaluated in a single laboratory by a single device. His results were analyzed by SPSS version 15 software using ROC curve to determine the sensitivity and specificity of indices.Results: according to our finding , there was no significant differences between WBC and platelets of two groups , but the differences of hb, MCV, RBC count, RDW, MCV/RBC and(RDW)MCV/RBC index between two group were stastically significant.Conclusion: It should be noted that RDW, MCV and MCV/RBC were the most sensitive and specific indices to differentiate minor THALASEMIA from iron deficiency anemia.