الفهرس 
Sickle Cell Disease (SCD)Only 14 pages are availabe for public view
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Abstract
Introduction: The vessel wall endothelium plays a role in the vascular pathobiology of sickle cell disease (SCD). Circulating endothelial cells (CECs) may serve as a new marker for micro-vascular injury.
Aim: to evaluate circulating endothelial cells (CD146%) and their relationship to cardiovascular dysfunction in patients with SCD.
Patients and methods: This study included 30 patients with sickle cell disease (group I) and 30 age- and sex-matched healthy subjects as a control group (group II). Data collected from patients included history taking, clinical examination, CBC, Hb electrophoresis, mean serum ferritin, CD146% by flowcytometry, ECG and echocardiography.
Results: LAD, LVEDD, LVMI and RVMI were significantly higher in patients than control. All patients had increased pulmonary artery pressure (RVESP > 25mmHg). 26 (86.7%) had diastolic dysfunction (E/A ratio <1 or E/Em >8). CIMT was increased in SCD patients compared to control (P=0.000). GLS, apical, Midventricular, basal LS, RS and CS of LV were significantly decreased in patients than control (P=0.000). CD146% was significantly higher in patients than controls (P=0.001), with a cutoff level of >0.22%. CIMT was significantly increased in patients with CD146% >0.22 (group Ia) (P=0.000). CD146% was positively correlated to CIMT (r=0.775, p=0.000). A significant decrease of GLS, LS, RS and CS was reported in group Ia, with a significant negative correlation to CD146% (r= -0.616, -0.878, -0.752, -0.625 and p= 0.000, 0.000, 0.000, 0.000 respectively). Multiple stepwise regression analysis revealed that CD146%, LV Em/Am, RV ET/AT, LVMI, RS and apical LS were significant predictors of vascular complications in SCD patients. ECG revealed that 80% of patients had sinus tachycardia, 40% had premature ventricular contractions (PVCS) and 30% had premature atrial contractions (PACS).
Conclusions: Patients with SCD are at higher risk of pulmonary hypertension and diastolic dysfunction. CD 146% as a marker of endothelial dysfunction was elevated in patients than control at a cutoff level >0.22. Multivariate logistic regression analysis showed that elevated levels of CD146% as well as LVMI, LV Sm, LV Em/Am, RV ST, LV ET/AT, RS and apical LS are significant predictors of vascular complications in patients with SCD