الفهرس يوجد فقط 14 صفحة متاحة للعرض العام
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المستخلص
Summary and Conclusion
β-thalassemia is highly prevalent and world wide in its distribution. β-thalassemia major is the most common cause of transfusion dependent anemia in childhood. In Egypt, it is the most common chronic hemolytic anemia, over 1000 of the annual 1.5 million newborn are expected to be affected. Recently the over all carrier rate in Egypt is 9-10%.
Anterior pituitary gland continues to pose a serious problem in patients with ß. thalassemia particularly in terms of gonadal function. Hypogonadotrophic hypogonadism is one of the most common endocrine abnormalities. Growth retardation, short stature and failure of puberty are frequent features of patients with ß. Thalassemia. Leptin is clearly required for appropriate pubertal development and maintance of secondary sexual characteristics.
The quality of life of these patients is an important aim, so it is vital to monitor carefully the growth and pubertal development in order to detect abnormalities and to initiate appropriate and early treatment.
Role played by scarce leptin production in the deranged sexual development observed in patients with ß. thalassemia received little investigations so study was designed to assess and correlate leptin level, F.S.H. and L.H. level in thalassemic females during puberty. Through the evaluation of 28 females with thalassemia major aged 10-18 years and 28 matched healthy females aged 10-18 years as a control.
The study was cross section analytic study in type and conducted in Ismailia, in out patient hematological pediatric clinic of Suez Canal University Hospital.
Each child was evaluated by Full history taking, Anthropometric measures, Examination for puberty by Tanner classification ,Assessment of quality of life using PedsQLTM 4.0 Scoring Algorithm and Laboratory investigations including: serum Leptin, FSH, LH, ALT, AST, Serum creatinine, Haemoglobin % and Serum ferritin level for thalassemic females.
The data were collected, revised and summarized then results were tabulated with the final conclusion was:
• Body Mass Index, weight and height of thalassemic females were significantly less than control group.
• Delayed puberty was 39.3 % among thalassemic females.
• AST and ALT are significantly higher in thalassemic group than control group.
• Serum leptin level is significantly lower in thalassemic group than control group.
• Weak non significant positive correlation between leptin and BMI in thalassemic group but in control group there was moderate positive correlation between leptin and BMI.
• There is no statistically significant correlation between leptin and ALT, AST, Serum creatinine and HB% in thalassemic group.
• Tإhere is significant negative correlation between serum leptin level and serum ferritin in thalassemic females.
• In thalassemic group, serum leptin level is significantly higher in females with normal puberty than those with delayed puberty.
• FSH and LH levels were significantly lower in thalassemic group than control group.
• The main factors which increase level of FSH and LH are leptin and BMI.
• There is significant positive correlation between leptin hormone level and the levels of FSH and LH in thalassemic group.
• Quality of life of thalassemic patients is indeed much lower than the quality of life of healthy control.