تقييم قصور الاقناد عند الذكور المصابين بالثلاسيميا بيتا المعتمد على نقل الدم وعلاقته بهرمونات اللبتين والكرلين

Evaluation of Hypogonadism in Males with Blood Transfusion Dependent β-Thalassemia and its Relation with Leptin and Ghrelin Hormones

Summary
β_thalassemia is one of most common genetic disorders characterized by abnormal synthesis of beta globin chain of hemoglobin’s molecule. Complications in β-thalassemia patients such as iron deposition in vital organs (liver, heart, endocrine glands) occurred regardless of adequate chelation therapy; Hypogonadism is the most frequently reported endocrine complication caused by iron deposit in hypothalamus, pituitary, and gonads; hormones such as leptin and ghrelin play a key role in regulation of energy balance, reproductive activities and endocrine function. In this study 100 males with mean age (21.46±4.22 year) were included and grouped as intact control(n=30) and thalassemic patients(n=70). The thalassemic patient was grouped according to their spleen status as non splenectomy (n=35) and splenectomy (n=35) group; both groups (thalassemia patients without splenectomy and thalassemia patients with splenectomy) classified according to the BMI as: underweight, normal weight, overweigh. Body mass index was calculated and the levels of some Hematological parameters (red blood cells,white blood cells, hemoglobin,hematocrit and platelets), biochemical parameters(iron, total iron binding capacity and ferritin), biomarkers levels(leptin and ghrelin) and level of some sex hormone (luteinizing hormone, follicle stimulating hormone and testosterone) were measured for both control and thalassemic male patients’ groups. The results of present study showed significant decrease (P≤0.05) of body mass index, hemoglobin, red blood cells, hematocrit, total iron binding capacity, leptin, luteinizing hormone, follicle stimulating hormone and testosterone in thalassemic patients as compared to control group, while there was significant increase(P≤0.05) of ferritin, iron and ghrelin in thalassemia group as compared to control group. The thalassemic patients with splenectomy showed significant increase (P≤0.05) in hematological parameters, leptin, LH, FSH, testosterone and significant decrease in ghrelin levels as compared to non splenectomized group. The finding of present study showed significantly positive correlation between leptin and body mass index. The results revealed significantly positive correlation between serum leptin and LH, FSH and testosterone for both splenectomized and non splenectomized groups. On the other hand there was significantly negative correlation between serum leptin and iron and ferritin, the results of present study showed no significant correlation between leptin and red blood cells, white blood cells, hematocrit, hemoglobin, platelets and total iron binding capacity for thalassemic patients. The results revealed a negative relationship between ghrelin and body mass index. The results showed significantly negative correlation between serum ghrelin and luteinizing hormone, follicle stimulating hormone and testosterone for thalassemic patients; there was significantly negative correlation between serum ghrelin and ferritin, while there was no significant correlation between ghrelin and red blood cells, white blood cells, hematocrit, hemoglobin, platelets and total iron binding capacity for thalassemic patients. According to this study the adipose tissue of beta thalassemic individuals are unable to maintain adequate leptin synthesis. These findings indicate that adipose tissue dysfunction is one of the endocrinopathies that afflict beta thalassemic individuals. In thalassemia male patients, leptin and ghrelin play a significant influence in sexual maturity and fertility. Splenectomy has the ability to reflect the negative changes caused by thalassemia. This effect is not only on metabolic hormones, but it’s highly effective for fertility and normalization if reproductive problems in adult male patients