الفهرس 
? ThalassemiaOnly 14 pages are availabe for public view
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Abstract
T
halassemia is considered the most common genetic disorder worldwide, about 3℅ of the world population (150 million people) carryβ-thalassemia genes ,B thalassemia major causes several endocrine disorders. A decrease of bone mineral density and early onset of osteoporosis have been reported in young patients .Among the factors in thalassemia that contribute to development of osteoporosis are chronic hypoxia, medullar expansion, iron accumulation,, abnormal calcium-phosphorus balance, high bone turnover, and hormonal insufficiency. Delayed puberty and hypogonadism are important risk factors in the decline of bone mineral density (BMD) in thalassemia patients more often and seriously
Sclerostin is a protein produced by the osteocyte serve as a marker of osteocyte activity. Furthermore, patients with thalassemia- associated osteoporosis have increased bone turnover.
This study aimed to evaluate serum circulating Sclerostin level in pediatric thalassemia major patients and their correlation with bone mineral density.
This study comprised:
1. Fourty five thalassemia major patients. They were 17 males and 28 females. Their ages ranged from 12 years to 19 years with mean of 12.42 years.
2. Fourty five healthy children, serving as the control group. They were 18 males and27 females. Their ages ranged from 12years to 19 years with mean of 12.42 years.
The patients were divided into three groups
group 1:
Patients aged (15-19) years. It included 25 patients. They were 7 males (28%) and 18 females (72%)
group 2:
Patients aged (14-15) years. It included 12 patients. They were 6 males (50%) and 6 females (50%),
group 3:
Patients aged (12-13) years it included 8 patients. They were 4 males (50%) and 4 females (50%),
All those children were subjected to detailed medical history, Full clinical examination, laboratory tests (HB%, ALT, AST, Calcium, Phosphorous, Alkaline phosphatase and mean serum Ferritin), DEXA Total lt femur, Neck of femure and Lumbar spine and serum Sclerostin level.
from this study, we can conclude that Circulating Sclerostin level was elevated in patients with Beta-TM than control group and its level was also elevated in patients with high Z-score of DEXA Total lt femur, Neck of femure and Lumbar spine.
The Age, weight, hight, body mass index Delay in sexual maturation, delayed puberty, the iron toxicity on osteoblasts, low serum calcium have been found to show a significant correlation with elevated circulating serum Sclerostin level.
There was a significant positive correlation between level of Sclerostin and age, weight, hight, BMI, Z-score of of DEXA Total lt femur, Neck of femure and Lumbar spine. Ferritin level as evident by P value < 0.05.
No significant correlation between level of Sclerostin and alkaline phosphatase, iron chelatores and liver enzymes (ALT, AST) as evident by P value > 0.05.
Also in the present study, there were significant positive correlations between DEXA Lumbar spine and Sclerostin, DEXA Total body, Blood transfusion, weight and Age as evident by p-value <0.05.
There was significant negative correlation between DEXA Lumbar spine and serum calcium as evident by p-value <0.05.
There was no significant correlation between DEXA Lumbar spine and, mean serum ferritin Phosphorous, Alkaline phosphatase and liver enzymes (ALT and AST) as evident by p-value >0.05.