الفهرس 
1-Acknowledgment.Only 14 pages are availabe for public view
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Abstract
The β-Thalassemia syndromes are most common hereditary
chronic hemolytic anemia due to impaired globin chain synthesis.
This impairment leads to deficient hemoglobin resulting in
hemolytic anemia.
Sickle cell disease is a genetic disorder which causes red
blood cells (RBCs) to acquire a sickle shape under conditions of
hypoxia, resulting in anemia, cell adhesion, vaso-occlusion, severe
pain, stroke and organ failure.
ANG plays several roles in angiogenesis which is a crucial
process in the pathogenesis of several inflammatory, autoimmune
and malignant diseases.
Endothelial damage and inflammation make a significant
contribution to the pathophysiology of sickle cell disease (SCD) and
the beta-β-Thalassemia syndromes.
Angiogenesis is triggered by hypoxia and ANG secretion is
enhanced by hypoxia.
The aim of this study was to compare the serum ANG level as
a marker of angiogenesis in patients with beta thalassemia and sickle
cell disease, and to assess the possible risk factors related to ANG
level in those patients. This study was performed on 72 patients; 32
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with β-thalassemia major, 20 patients with β-thalassemia intermedia
and 20 patients with Sickle cell disease.
All patients are subjected to complete history and full
examination. The following investigations were done:
Complete blood count, Hb electrophoresis, serum ferritin, and
serum ANG level.
The study revealed the following results:
The ANG level was significantly higher in patients with SCD
compared to β-Thalassemia group and control with significant
elevation in those with severe form of SCD (HbSS) compared with
HbS/beta-thal.
This study revealed that β-Thalassemia major group had
significantly higher ANG level compared to controls specially β-
Thalassemia intermedia with non significant difference in ANG
level between splenectomized thalassemic patients and non
splenectomized thalassemic patients (p=0.199).
In our study, patients with β-Thalassemia had significant
higher transfusion index (99 ±31.6 ml/kg/year) and transfusion
frequency per year (12 ±2.8) compared to those with SCD with no
significant correlation between blood transfusion index per year (ml ̷
kg ̸ year) and ANG level in SCD, β-Thalassemia major, β-
Thalassemia intermedia with a highly significant negative
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correlation between ANG level and frequency of blood transfusion
only in SCD(r=-0.731, p<0.001).
In the current study, There was a significant inverse
correlation between ANG level and the age of patients in β-
Thalassemia major group and β-Thalassemia intermedia (p<0.001)
(P=0.009) respectively.
In this study, there was a significant negative correlation
between ANG level and age of onset of disease in β-Thalassemia
major group and in β-Thalassemia intermedia group (r=-0.414,
p=0.019) (r=-0.486, p=0.30) but not in SCD.
In this study, there was direct significant correlation between
serum level of ANG and age of first transfusion in β-Thalassemia
major(r=0.379 p=0.032).
In the present study, there was significant inverse correlation
between ANG level and BMI in SCD (r=-0.545, p=0.013).
In this study there was significant inverse correlation between
ANG level and ferritin in β-thalassemia intermedia group(r=-0.573,
p=0.008).
In our study, there was highly significant inverse correlation
between ANG level and desferoxamine or deferiprone duration of
therapy among β-Thalassemia intermedia(r=-0.830, r=-0.774)
(p<.001) respectively while there was a highly significant inverse
correlation between serum ANG and total duration of chelation in β-
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Thalassemia intermedia (p<0.001) and β-Thalassemia major
(p=0.003) but no significant correlation in SCD (P=0.384).
In this study there was a significant inverse correlation
between serum ANG and hydroxyurea duration in SCD (p=0.017)..
In our study, WBC count were direct correlated with ANG
level in patient with SCD only while platelet count were correlated
inversely with ANG level in patient with β-Thalassemia major only.