Department of Neurology, University of Würzburg, Josef-Schneider-Strasse 11, 97080 Würzburg, Germany.
J Ultrasound Med. 2012 Mar;31(3):463-8. doi: 10.7863/jum.2012.31.3.463.
The purpose of this study was to investigate cervical and cerebral blood flow characteristics in patients with Fabry disease at baseline and under enzyme replacement therapy.
In this case-control study we prospectively studied 68 patients with Fabry disease with extracranial and transcranial Doppler sonography. We compared extracranial and transcranial cervical and cerebral blood flow properties in all patients with Fabry disease and in subgroups of those with or without enzyme replacement therapy, male and female, and with normal or impaired renal function. Eight male patients were investigated at baseline and 1 year after initiation of enzyme replacement therapy.
We show that cervical and cerebral blood flow parameters in patients with Fabry disease are not different from normal values regardless of sex, renal function, or enzyme replacement therapy.
Cervical and cerebral blood flow measured with extracranial and transcranial Doppler sonography is not altered in patients with Fabry disease. Enzyme replacement therapy does not change blood flow characteristics.
本研究旨在探讨法布里病患者在基线和酶替代治疗下的颈内和脑血流特征。
在这项病例对照研究中，我们前瞻性地对 68 例法布里病患者进行了颅外和经颅多普勒超声检查。我们比较了所有法布里病患者以及接受或不接受酶替代治疗、男性和女性、肾功能正常或受损亚组的患者的颅外和颈内及脑血流特性。8 名男性患者在基线和酶替代治疗开始后 1 年接受了检查。
我们发现，法布里病患者的颈内和脑血流参数无论性别、肾功能或酶替代治疗如何，均与正常值无差异。
经颅外和经颅多普勒超声测量的颈内和脑血流在法布里病患者中没有改变。酶替代治疗不会改变血流特征。
