الفهرس 
Beta-Thalassemia
Manifestations of extra medullary hematopoiesisOnly 14 pages are availabe for public view
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Abstract
beta-thalassaemia major (BTM) is a type of chronic inherited hemolytic anemia that is characterized by impaired biosynthesis of b-globin.Beta thalassemia is an inherited hemoglobin disorder resulting in chronic hemolytic anemia that requires life long transfusion therapy. Repeated blood transfusions and RBCs hemolysis are the major causes of secondary iron overload which leads to deposition of iron in different endocrine glands and affection of their functions. Delayed puberty and hypogonadism are the most
obvious clinical consequences of iron overload. The aim of this study was to
evaluate male sex hormone levels in male children with β- thalassemia major in correlation with iron overload. Method: The present study was conducted in the period between May 2012 and October 2014 on 30 male children with beta thalassemia major under follow up at Hematology unit, Pediatric Department, Tanta University Hospital with their age ranging from 11-18 years and mean age value of 14.16+2.48 and 20 healthy male children as a control group with their age ranging from 11-18 years and mean age value of 14.75 + 1.89. All children included in the study were subjected to the following investigations: complete blood count, Hb electrophoresis, serum ferritin, serum iron, TIBC and serum testosterone . Results: Pallor and mongoloid facies represented the most common presenting signs. Hb, MCV, MCH and TIBC were significantly lower while reticulocytic count , serum iron and serum ferritin were significantly higher in patients than controls. Testosterone serum level was significantly lower in pat ients than controls. There was significant negative correlation between serum testosterone and serum iron and ferritin levels in patients group. Conclusion: Male sex hormone is significantly lower in patients with thalassemia compared with control group with significant negative correlation between male sex hormone and iron load. Recommendations: regular follow up of thalassemic patients for detection of iron overload by measurement of serum ferritin over 3 months to avoid iron over load and its complications including hypogonadism.