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autonomic nervous system activity in patients with fabry disease自主神经系统活动fabry疾病患者
Open Journal of Internal Medicine, 2012, 2, 116-122 OJIM
/10.4236/ojim.2012.22021 Published Online June 2012 (http://www.SciRP.org/journal/ojim/)
Autonomic nervous system activity in patients with
*
Fabry disease
1# 1 2 2 2
Eric Alamartine , Aurore Sury , Frédéric Roche , Vincent Pichot , Jean-Claude Barthelemy
1Department of Nephrology, Saint-Etienne University Hospital, Saint-Etienne, France
2Department of Physiology, Saint-Etienne University Hospital, Saint-Etienne, France
Email: #eric.alamartine@univ-st-etienne.fr
Received 1 November 2011; revised 10 February 2012; accepted 6 March 2012
ABSTRACT golipids, in particular globotriaosylceramide (Gb3), pro-
gressively accumulate within the lysosomes of a large
Background: Fabry disease is an inherited, multisys-
variety of cell types. Affected cells include vascular en-
temic and progressive lysosomal storage disorder.
dothelial cells, neural cells, renal cells and cardiomyo-
The first symptoms of Fabry neuropathy reflect pro-
cytes. A cascade of overlapping pathologic events leads
gressive loss of function of both peripheral somatic
and autonomic nerve cells. We aimed to evaluate to multisystemic disease manifestations with small fiber
autonomic nervous system (ANS) activity in a cohort
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