الفهرس 
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Abstract
Thalassemia is a hereditary disease caused by defective
globin synthesis resulting in abnormal and/or decreased globin
quantity. Although the multitransfusion and chelation therapy
increased the life expectancy of thalassemia major ™
patients, it induced new medical complications, namely, the
bone diseases or osteoporosis-osteopenia syndrome of both
sexes.
In patients with TM, progressive ‘‘aging’’ of the bone
starts as early as childhood, through the gradual imbalance
between augmented osteoclastic resorption and insufficient
osteoblastic bone formation. Several factors acting
independently or in concert may affect bone metabolism in
thalassemia–as the primary disease is associated with bone
marrow expansion, genetic factors, endocrine complications,
vitamins and trace minerals deficiencies, decreased physical
activity, iron overload, and direct iron and chelation therapy
toxicities.
Identification of the exact pathogenic factors of bone
disease in TM is of primary importance for prevention and
treatment.
Close follow-up and early recognition of osteopenia,
together with proper management, are crucial for every
thalassemic patient for a better quality of life. In addition, we
Summary
hope that the use of new generation oral chelator would be
beneficial for more effective removal of iron from cells, and
thus reduce damage by hemosiderosis. However, resolution of
the puzzling multifactorial etiology and complex mechanisms
behind the bone disease in thalassemia requires further research
particularly using neutralizing antibodies and knockout models
for the major effectors such as RANKL and OPG.
Assessment of Bone Mineral Density BMD was
measured from the lumbar spines (L2 to L4) by dual energy
X-ray absorbtiometry method using a computed densitometry
device (Hologic QDR Delphi W S/N 70232) and Z-Scores of
BMD were calculated. Past fracture histories after the completion
of the treatment were asked. Age and sex based Z-Scores of
BMDs indicating osteopenia or osteoporosis risks were
calculated. Z-Score values above -1 SD were considered as
normal, between -1 SD and -2 SD as osteopenia, and below -2SD
as osteoporosis. Serum calcium, phosphate, alkaline phosphates,
(calorimetric method, Abbott Laboratories aero set autoanalyser),
parathormone (chemiluminescence’s immunumetric assay-
I’mmulite 2000 Intact PTH).