الفهرس 
Hypothalamic Pituitary Gonadal Axis in ?-ThalassemiaOnly 14 pages are availabe for public view
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Abstract
Beta thalassemia syndromes are a group of hereditary disorders characterized by genetic deficiency in the synthesis of beta-globin chains. In the homozygous state, beta thalassemia major causes severe transfusion-dependent anemia.
Free radicals and other reactive oxidants are generated in biological systems by both endogenous processes and exposure to external stimuli such as radiation, nitrogen oxides, mineral fibers and dusts. Reactive oxygen species (ROS) include superoxide anion (O2−), hydrogen peroxide (H2O2) and hydroxyl radical (HO•). Experimental data confirm the progression of oxidative stress in patients with β-thalassemia major due to activation of free radical processes and lipid peroxidation and decreased antioxidant capacity.
The aim of this study was to determining the magnitude of oxidative damage of sperms in thalassemic patients after long period of repeated blood transfusions and assessment of the effect of antioxidant treatment by reassessment of sperm parameters after 6 months of treatment.
Patients and Methods:
Total number of patients screened to be included in the study was 125 patients with beta thalassemia major who were regularly attending the hematology/oncology clinic at Ain Shams University Hospitals. Fully pubertal thalassemic adults above 16 years with no endocrinal affection, particularly diabetes mellitus and hypothyroidism, were included. An informed consent was taken from the patients.
Only 10 patients fulfilled the inclusion criteria and were compared to 10 other patients who were found to be azoospermic regarding their hormonal profile and anthropometric measures.
The medical records of patients were reviewed and a standardized data sheet was completed for all study subjects. The collected data included: history, clinical examination, laboratory evaluation including:
Initial assessment of serum calcium, phosphorous, assessment of serum parathyroid hormone, assessment of serum thyroid stimulating hormone and serum free thyroxin, assessment of fasting serum glucose and fasting serum insulin, assessment of basal serum follicle stimulating hormone and serum luteinizing hormone, initial semen analysis including seminal volume, color, sperm count, motility, abnormal forms and types of abnormality and comparison to the WHO reference ranges, sperm chromatin structure assay test to assess sperm DNA damage initially, assessment of superoxide dismutase, glutathione peroxidase and reductase levels in seminal plasma.
The 10 non-azospermic patients received L-carnitine 2g and N-acetyl cysteine 600 mg daily for 6 months, followed by reassessment of semen analysis and sperm chromatin structure assay, assessment of superoxide dismutase, glutathione peroxidase and reductase levels in seminal plasma.
Results:
This study showed significant differences in mean pretransfusional hemoglobin level, frequency of blood transfusion and testicular volumes of azoospermic and non azoospermic patients showing improvedtesticular and sexual maturation in non azospermic patients and suggesting the cause to be more frequent transfusions, more iron deposition and more oxidative damage in azospermic patients.
With regards to the hormonal profile in azoospermic patients and non azoospermic patients, the study showed calcium to be significantly higher in non-azoospermic males as well as the total testosterone and basal FSH. Serum FT4 was higher in azoospermic males. Although the difference is statistically significant, still all cases in both groups are euthyroid. Antioxidants in seminal plasma showed a significant positive correlation between the level of super oxide dismutase and the spermatogenic cells in millions/ml after treatment (r=0.699, p= 0.024).
Serum ferritin before and after treatment showed a significant positive correlation with the mean pre transfusional hemoglobin level (r=0.703, p = 0.023), (r=0.798, p=0.006) respectively.
This study showed differences between pH, total number of defects in all heads, number of middle piece with defect, teratozospermia index, and sperm deformity index before and after treatment by antioxidants being increased by antioxidant treatment. There were no differences in seminal plasma parameters and DNA fragmentation or serum ferritin before and after treatment.