الفهرس 
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Abstract
SCD is one of the most common severe monogenic disorders in the world. The main core of the disease is Hb polymerization, leading to erythrocyte rigidity and vaso-occlusion. Recurrent episodes of vaso- occlusion and inflammation result in progressive damage to most organs, including the brain, kidneys, lungs, bones, and cardiovascular system, which becomes apparent with increasing age.
SCR is the most representative ophthalmologic complication of SCD, a hemoglobinopathy affecting both adults and children. SCR presents a wide spectrum of manifestations and may even lead to irreversible vision loss if not properly diagnosed and treated at the earliest. PSR is the main cause of visual loss in patients with sickle cell disease. It is characterized by neovascularization, which may be active or it may be inactive and fibrotic.
The current novel findings of elevated plasma Ang-2 in SCD, alongside raised VEGF, are consistent with the concept of increased angiogenic activity in SCD generally. The precise cause for the increased angiogenesis is systemic tissue hypoxia due to generalized vaso-occlusion that contributes to the elevated plasma Ang-2 and VEGF levels. Marked endothelial dysfunction is associated with SCD and endothelial proliferation as a means of endothelial repair may be a mechanism for attempting to preserve endothelial homeostasis.
The aimof the study was to evaluate serum angiopoietin-2 as a marker of retinopathy in children with sickle cell disease.
This study is a case-control study that was conducted in the Hematology Unit in Pediatric Department, Menoufia University Hospital.
Subjects were divided into two groups:
Summary
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group 1 (Patient group): It included 30 HbSS and sickle betathalassemia patients. Their ages ranged from 5 to 17 years. They were13 males and 17 females.
group 2 (Control group): It included 30 apparently healthy childrenof comparable age, sex and socioeconomic standard as a control group(males and females). Their ages ranged from 5 to 17 years. They were11 males and 19 females.
Patients and controls were subjected to detailed history taking, full clinical examination and laboratory investigations including: complete blood count, reticulocyte count, liver and renal function tests, serum ferritin, and serum angiopoietin-2 by ELISA. Imaging investigations as TCD and echocardiography were done. Ocular examination in the form of visual acuity and fundus examination were also done.
The results of the present study showed:
Height and BMI are statistically significant decreased among casesthan control (p=0.032 and <0.001), respectively. A statisticallysignificant difference between the two groups regarding height,weight and BMI centiles as 56.7%, 66.7% and 60% which were lessthan 5th, respectively among the cases.
Among studied cases, 53.3% are sickle cell thalassemia and 46.7%ofthe cases are sickle cell disease. 56.7% of the studied cases had pallor,40% had jaundice, and 13.3% are spleenectomized, meansplenomegaly is 7.8 cm. 87.5% had VOC and 12.5% had hyperhemolytic crisis with a mean pain rate per year equals 0.95 rangesfrom0 to 4.5 episode /year and mean frequency of crisis is 2.71 /years.
As for hydroxyurea treatment, 50% of cases were on 15 mg/kg dose;with 50% of cases were hydroxyurea compliant. 26.7% of cases weretreated with deferasirox.
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A statistically significant lower hemoglobin level and hematocrit %among cases than control group (p<0.05). A statistical significanthigher RDW, platelet count and serum ferritin were found amongcases than control group.
The median Ang-2 is statistically significant higher among cases thanthe control group (220.47 pg/mL versus 62.5 pg/mL) with everyincrease of pg / ml in Ang-2 increases risk for disease by 1.023 (oddsratio: 1.023, 95%CI: 1.01-1.04).
As for imaging investigations, four cases had abnormalechocardiographic findings (one case had mild pulmonaryhypertension, and mild left ventricular dilatation, the second hadhyperdynamic state, the third case had mild mitral regurge and the lastone had mild mitral regurge and left ventricular dilatation). TCDfindings showed that 9 cases had abnormal findings (one case had highrisk and 8 cases had conditional findings).
Ophthalmological examination showed that 30% of the studied caseshave visual acuity of 6/9 with statistical significant difference betweenthe two groups (p=0.001), mean intraocular pressure is 15.7 versus
15.4 mm/Hg without statistical significant difference between them.No statistical significant difference between studied groups fundusexamination regarding fundus examination as well.