History and Objectives: Considering the incidence of ?-thalassemia major and the presence of hypogonadism in affected individuals and the absence of the effect of dysferal on Zinc level and the significance of Zinc in spermatogenesis, this study was carried out to determine the effect of Zinc therapy on hypophyseal-gonadal axis in patients with ?-thalassemia major and Zinc deficiency in 1996.

Materials and Methods: The clinical trial and two-blind strategy of this study was conducted on 23 male and female patients with an age range of 14-18 years. They were randomly divided into control and case groups. Case group received 25-mg capsules and control group received its vapor for 3 months. LHRH test was done before and after Zinc therapy and hormonal responses were recorded at minutes 0, 30 and 60.

Results: Four patients were excluded from the study and the research was conducted on 19 patients including 9 cases in control group and 10 cases in case group. In case group, Zinc level increased from 15±6 µg/dl to 120±49 µg/dl (P<0.001), but there was no such increase in the placebo group. In addition, there was no increase for FSH and LH level after treatment in both groups.

Conclusion and Recommendations: It is concluded that Zinc therapy for three months could not affect the response of hypophyseal-gonadal axis. It is strongly recommended to conduct researches whether Zinc deficiency may lead to irreversible damages or Zinc is not the only factor responsible for hypogonadism.

Background: β thalassaemia is an inherited blood disorder that needs repeated blood transfusions. Serum lipid abnormalities have been repeatedly reported in these patients. Although thalassemia is common in some regions of Iran, there is little evidence on serum lipids and lipoproteins profile in these patients.�The aim of this study was to evaluate plasma lipids and lipoproteins in thalassemic patients referring to Yazd Blood Transfusion Center.

Materials and Methods: In this cross-sectional case-control study, serum lipids, lipoproteins and lipoprotein(a) from 58 patients (32 females, 26 males) with β thalassemia major, were compared to 68 (40 females, 28 males) age matched healthy control. Blood samples were taken after at least 12 hours fasting and before the patients' blood transfusion. Total cholesterol (TC), triglycerides (TG), and high-density lipoprotein-cholesterol (HDL-C), were determined enzymatically using laboratory test kits. Lipoprotein (a) assay was done by electro-immunoassay method. SPSS software (V.11), t-test and U-test were used to compare lipids, lipoprotein, and lipoprotein (a) and Pearson correlation test was used to determine the correlation between variables.

Results: β thalassemic patients had significantly lower TC, HDL-C and LDL-C values compared to the control (113.8±17.7, 32.3±8, 58.2±14.7 vs 158.3±30.6, 43.8±9.2, 96.2±26.4, p<0.001), while serum TG and lipoprotein(a) levels had no significance difference in two groups. Nineteen percents of patients and 27% of controls had serum lipoprotein (a) levels equal to or higher than 30 mg/dl. There was no significant correlation between lipids, lipoproteins and age in two groups, however in control group a significant correlation was seen (r = 0.5, p < 0.001).

Conclusion: The results indicate that in patients with β thalassemia major, cholesterol and its carrier lipoproteins are lower than those in the healthy controls, but triglycerides and lipoprotein (a) have no significant difference. It seems that endocrine disorders in patients with β thalassemia has more effects on serum lipid profile. Further studies are needed to clarify the exact effect of different conditions on lipid profile in thalassemic patients.

Background: At present, the only curative treatment for β-thalassemia major is allogenic bone marrow transplantation accompanied with considerable mortality and morbidity in class III β-thalassemia. Regarding few case reports on successful non-myeloablative stem cell transplantation in class III β-thalassemic cases, we evaluated the effectiveness of this type of allogenic stem cell transplantation, considering less toxic non-myeloablative conditioning regimen.

Materials and Methods: In this prospective study in Shariati Hospital bone marrow transplantation center during 2001-3, 13 class III β-thalassemia patients (on the basis of history and physical examination and liver biopsy) were transplanted with peripheral blood and bone marrow stem cells from their HLA-identical siblings. Non-myeloablative conditioning regimen included fludarabine busulan antithymocyte globulin. Graft versus host disease (GVHD) prophylactic regimen was cyclosporin and metothrexate. In the case of the declining chimerism, the patients were treated with donor lymphocyte infusions (DLI).

Results: The conditioning regimen was tolerated well without any considerable toxicity in hematologic, gastrointestinal and pulmonary systems. Five (38.5%) patients had acute and 2(15.4%) had chronic GVHD. Two patients died after transplantation. While two cases had a thalassemia-free survival.

Conclusion: Although associated with high graft failure and the recurrence of disease, non-myeloablative stem cell transplantation may be used as a curative, less toxic post-transplantation treatment for class III ß-thalassemia.

Background: Patients with thalassemia major are clinically dependent on red blood cell (RBC) transfusions . Performing multiple transfusions increase the risk of transfusion-related complications including blood-borne infections, iron overload and also RBC alloimmunization .

Materials and Methods: This cross-sectional descriptive study was conducted on 70 thalassemia cases with regular blood transfusions. The serum samples were screened for the detection of the unexpected antibodies and the positive samples were subjected to antibody identification.

Results: Among 70 cases, 6 (8.6%) were identified as unexpected alloantibody three cases as Anti-K, one as Anti-E and another one as Anti-D. Coincidence of Anti D and Anti-E was detected in one case. Eighteen patients (25.7%) were splenectomized. No significant correlation was seen between the presence of alloantibody and age, sex, the time of first transfusion and spleen condition.

Conclusion: Considering that the most prevalent unexpected antibodies (8.6%) identified in this study were against the Kell and Rh system antigens, the evaluation of compatibility for antigens found can be recommended before the performing of transfusion. Therefore, this strategy may decrease the possibility of recipient immunization and production of the unexpected antibodies against donor RBCs.

Background: Thalassemia is an inherited blood disorder which despite treatment its clinical manifestations affect the patients' quality of life. Therefore, psychological support and rehabilitation programs are needed to improve the quality of life in such patients. The purpose of this study was to evaluate the effect of an 8-week walking program on quality of life in patients with thalassemia major.

Materials and Methods: In this semi-experimental study, 61 patients with thalassemia major (18 years and older) were randomly divided into the experimental and control groups. Patients in the experimental group performed an 8-week walking program. Quality of life was assessed using the SF36 questionnaire before and after the intervention in both groups.

Results: Results showed significant improvements in quality of life in the experimental group compared with the control group after an 8-week regular walking program (P<0.05).

Conclusion: An 8-week walking program has a positive effect on the quality of life in thalassemia major patients and a walking program designed for such patients is recommended to enhance their quality of life.