الفهرس 
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Abstract
According to the TIF, about 7% of the global population are carries of a hemoglobin disorder, and approximately 300 000 to 500 000 children are born every year with a severe hemoglobinopathy. In Egypt, β- Thalassemia is the most frequent hemoglobinopathy. The carrier rate of this disease varies between 5.3-9% and the gene frequency is 0.03%. The disease hallmarks include imbalance in the α/β-globin chain ratio, ineffective erythropoiesis, chronic hemolytic anemia, compensatory hemopoietic expansion, hypercoagulability and increased intestinal iron absorption.
The complications of iron overload, arising from blood transfusion, that represent the basis of disease management in patients with severe thalassemia, might complicate the clinical phenotype. The mechanisms leading to hypogonadism in thalassemia patients are complex. chronic anemia, iron overload and chronic liver disease play the major roles.
A case control study was carried out on 20 male patients with prior diagnosis of β-TM and 20 age and sex matched healthy persons, from hematology unit, pediatric and medicine department, Menoufia University. All prospective patients were evaluated for age, sex, weight and sexual maturation by Tanner scale based on the development of genitalia and pubic hair. Blood samples were obtained in the morning after an overnight fast and before transfusion in all patients. Levels of LH, FSH and TT were measured.
Delayed puberty is defined as the absence of any sign of pubertal development in boys aged 14 years. Arrested puberty is considered to be present if the patient has some clinical signs of sexual maturation, but no progression in pubertal development has been observed for1 year. Hypogonadism after complete sexual maturation is diagnosed in patients with normal pubertal progression who
Summary
have reached tanner stage V (adult stage of sexual maturation) and present at time of evaluation with testosterone deficiency syndrome or decreased body hair associated with low TT. Data regarding treatment of the hematological disease (age at start of transfusional treatment, age at start of chelation treatment, hemoglobin levels, the transfusion requirement, serum ferritin levels, history of splenectomy and deferoxamine dose) were obtained from medical records.
About 80% of the thalassemic patients had hypogonadism, in the form of delayed (60%), and arrested (20%) puberty, while 20% hadn’t. There were no statistically significant differences between thalassemia with and without hypogonadism groups regarding age, weight, height, BMI, blood transfusion index, age of starting chelation and dose of chelation (P>0.05). While, age of starting transfusion was significantly lower among thalassemic patients with hypogonadism than those without hypogonadism (P=0.030), While, platelets count and serum ferritin level were significantly higher among thalassemic patients with hypogonadism than those without hypogonadism (P<0.05).