title: [Evaluation of vascular function in patients with Fabry disease].
abstract: OBJECTIVE
  To evaluate the endothelial functions and autoregulation capacity of cerebral blood flow in patients with Fabry disease.
  
  
  METHODS
  Brachial artery vasodilation was assessed in 8 patients with Fabry disease and 14 healthy controls by means of flow-mediated dilation (FMD) and Nitroglycerin-mediated dilation (NMD). Cerebrovascular reactivity was calculated in terms of breath-holding index (BHI) and vascular motor reactivity (VMR) by TCD-CO2 test in 4 patients and 14 healthy controls.
  
  
  RESULTS
  Compared with the controls, brachial artery vasodilation experiment showed no difference (the patients: FMD 15.94%±5.03% and NMD 23.92%±7.23%, the controls: FMD 14.57%±5.84% and NMD 22.64%±6.96%), there was no relationship between FMD or NMD and the age, course of disease, MSSI or enzyme activity. In respect of cerebrovascular autoregulation capacity, there was no difference in anterior circulation, while cerebrovascular reactivity tended to be impaired in posterior circulation.
  
  
  CONCLUSIONS
  Endothelial function showed no decline in patients with Fabry disease, but cerebrovascular autoregulation capacity tended to be impaired in posterior circulation.
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing 100034, China.
authors.forename: Qian
authors.initials: Q
authors.lastname: Gao
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing 100034, China.
authors.forename: Qing
authors.initials: Q
authors.lastname: Peng
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing 100034, China.
authors.forename: Jing
authors.initials: J
authors.lastname: Chen
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing 100034, China.
authors.forename: Wei
authors.initials: W
authors.lastname: Zhang
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing 100034, China.
authors.forename: Zhao-xia
authors.initials: ZX
authors.lastname: Wang
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing 100034, China.
authors.forename: Yun
authors.initials: Y
authors.lastname: Yuan
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing 100034, China.
authors.forename: Yue-huan
authors.initials: YH
authors.lastname: Zuo
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing 100034, China.
authors.forename: Jing
authors.initials: J
authors.lastname: Liu
mesh_terms: D001916:Brachial Artery; D016022:Case-Control Studies; D004730:Endothelium, Vascular / Q000503:physiopathology*; D000795:Fabry Disease / Q000503:physiopathology*; D006801:Humans; D005996:Nitroglycerin; D012307:Risk Factors; D014664:Vasodilation
chemical_list: D005996:Nitroglycerin