London, United Kingdom 2013 supporting the hypothesis of the presence of endothelial dysfunction in this disease and its po-tential role in modulating phenotype. Reference: 1Pescini F et al. Stroke 2010;41:218-223 Cerebrovasc Dis 2013; 35 (suppl 3)1-854 41 6 Small vessel stroke and white matter disease 15:20 - 15:30 ENDOTHELIAL DYSFUNCTION IN CADASIL: ROLE OF CIRCULATING PROGEN-ITOR CELLS AND BIO-HUMORAL MARKERS F. Pescini1, A.M. Gori2, F. Cesari3, B. Giusti4, L. Ciolli5, M.T. Dotti6, I. Donnini7, S. Nannuc-ci8, R. Valenti9, E Adriano10, M. Balestrino11, A.M. Basile12, L. Pantoni13, R. Abbate14, D. Inzi-tari15 Department of Neurological and Psychiatric Sciences, University of Florence and Azienda Ospedaliero Universitaria Careggi, Florence, ITALY1,Dep of Medical and Surgical Critical Care, Atherothrombotic Diseases Center, University of Florence and Azienda Ospedaliero Uni-versitaria Careggi, Florence, ITALY2, Dep of Medical and Surgical Critical Care, Atherothrom-botic Diseases Center, University of Florence and Azienda Ospedaliero Universitaria Careggi, Florence, ITALY3, Dep of Medical and Surgical Critical Care, Atherothrombotic Diseases Cen-ter, University of Florence and Azienda Ospedaliero Universitaria Careggi, Florence, ITALY4, Department of Neurological and Psychiatric Sciences, University of Florence and Azienda Ospedaliero Universitaria Careggi, Florence, ITALY5, Department of Neurological, Neurosur-gical and Behavioural Sciences, University of Siena, Policlinico “Le Scotte”, Siena, ITALY6, Department of Neurological and Psychiatric Sciences, University of Florence and Azienda Ospedaliero Universitaria Careggi, Florence, ITALY7, Department of Neurological and Psy-chiatric Sciences, University of Florence and Azienda Ospedaliero Universitaria Careggi, Flor-ence, ITALY8, Department of Neurological and Psychiatric Sciences, University of Florence and Azienda Ospedaliero Universitaria Careggi, Florence, ITALY9, Department of Neuroscience, Ophthalmology and Genetics, University of Genova, Genoa, IT-ALY10, Department of Neuroscience, Ophthalmology and Genetics, University of Genova, Ge-noa, ITALY11, Department of Neurosciences, University of Padua, Padua, ITALY12, Department of Neurological and Psychiatric Sciences, University of Florence and Azienda Ospedaliero Uni-versitaria Careggi, Florence, ITALY13, Dep of Medical and Surgical Critical Care, Atherothrom-botic Diseases Center, University of Florence and Azienda Ospedaliero Universitaria Careggi, Florence, ITALY14, Department of Neurological and Psychiatric Sciences, University of Flor-ence and Azienda Ospedaliero Universitaria Careggi, Florence, ITALY15 Background: Among pathogenic processes in CADASIL, endothelial dysfunction has been hy-pothesized. Recently, we found an association between low levels of bone marrow-derived cir-culating cells and the disease1 1. Objective: To evaluate the role of biomarkers of endothelial dysfunction (endothelial progen-itors cells EPCs, circulating progenitors cells CPCs, von Willebrand factor vWF, and thrombomodulin TM) in a large CADASIL series. Methods: 45 CADASIL patients and 45 sex and age comparable controls were enrolled. EPCs and CPCs were measured in peripheral blood using flow cytometry. EPCs were defined as posi-tive for CD34/KDR, CD133/KDR and CD34/CD133/KDR; CPCs as positive for CD34, CD133 and CD34/CD133. vWF and TM were measured in plasma samples using ELISA kits. Results: In comparison with controls, CADASIL patients had significantly lower EPCs lev-els CD34/KDR: 0.07 vs 0.1 cells/μl, p=0.002; CD133/KDR: 0.07 vs 0.1 cells/μl, p=0.004; CD34/CD133/KDR: 0.06 vs 0.09 cells/μl, p=0.005 and higher vWF levels 129 vs 96%, p=0.008. The differences remained significant in multivariate logistic regression models ad-justed for age, gender, hypercholesterolemia, hypertriglyceridemia, statins and ACE-inhibitors use. TM levels were similar in the 2 groups. CPCs were not significantly lower in CADA-SIL, but patients with stroke or dementia had significantly reduced CPCs levels than patients without CD34:1.88vs2.83 cells/μl, p=0.015; CD133:1.80vs2.76 cells/μl, p=0.013; CD34/ CD133:1.80vs2.73 cells/μl, p=0.014. Conclusions: This is the largest series of CADASIL patients in which cellular and bio-humoral markers of endothelial dysfunction have been studied. We confirmed the previously reported1 association between EPCs and CPCs and the disease, and we found an association with vWF,
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