title: Cerebral hemodynamics and endothelial function in patients with Fabry disease.
abstract: BACKGROUND
  Cerebral vasculopathy have been described in Fabry disease, in which altered cerebral blood flow, vascular remodelling or impairment of endothelial function could be involved. Our study aims to evaluate these three possibilities in a group of Fabry patients, and compare it to healthy controls.
  
  
  METHODS
  Cerebral hemodynamics, vascular remodelling and systemic endothelial function were investigated in 10 Fabry patients and compared to data from 17 healthy controls. Transcranial Doppler was used to study blood flow velocity of intracranial arteries and cerebral vasomotor reactivity. For the study of vascular remodelling and endothelial function, intima-media thickness of common carotid arteries, flow-mediated dilation in brachial artery and serum levels of soluble VCAM-1, TNF-α, high-sensitive CRP and IL-6 were measured. Differences between groups were evaluated using appropriate tests.
  
  
  RESULTS
  No relevant differences were observed in cerebral hemodynamic parameters, intima-media thickness or flow-mediated dilation. There was a trend for low serum levels of IL-6 and high serum levels of TNF-α and high-sensitive CRP in Fabry patients; plasma concentrations of soluble VCAM-1 were significantly higher in Fabry disease patients than in healthy volunteers (p = 0.02).
  
  
  CONCLUSIONS
  In our sample, we did not find relevant alterations of cerebral hemodynamics in Fabry disease patients. Increased levels of plasmatic endothelial biomarkers seem to be the most important feature indicative of possible vascular dysfunction in Fabry disease patients.
authors.affiliation: Department of Neurology, Hospital General Universitario de Albacete, C/Hermanos Falcó S/N, Albacete 02006, Spain. tsmtsm08@yahoo.es.
authors.forename: Tomás
authors.initials: T
authors.lastname: Segura
authors.forename: Oscar
authors.initials: O
authors.lastname: Ayo-Martín
authors.forename: Isabel
authors.initials: I
authors.lastname: Gómez-Fernandez
authors.forename: Carolina
authors.initials: C
authors.lastname: Andrés
authors.forename: Miguel A
authors.initials: MA
authors.lastname: Barba
authors.forename: José
authors.initials: J
authors.lastname: Vivancos
mesh_terms: D000293:Adolescent; D000328:Adult; D001783:Blood Flow Velocity / Q000502:physiology; D002560:Cerebrovascular Circulation / Q000502:physiology*; D003625:Data Collection / Q000379:methods; D004730:Endothelium, Vascular / Q000502:physiology*; D000795:Fabry Disease / Q000175:diagnosis* / Q000453:epidemiology* / Q000503:physiopathology; D005260:Female; D006439:Hemodynamics / Q000502:physiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D055815:Young Adult