Pregnancy Clinical Trial
Official title:
Description of Obstetrical Care, Mainly Anesthesic, in Patients With Fabry's Disease
Fabry's disease is a progressive systemic disease X-linked which combines neurological
(Fabry's pain crises), dermatologic (angiokeratomas), renal (renal failure), cardiovascular
(hypertrophic cardiomyopathy, valvular disease, conduction disorder, coronary heart disease)
and cerebral vascular (stroke) symptoms .
It is a glycosphingolipid metabolism disorder due to deficient or absent activity of the
alpha-galactosidase A, causing accumulation of globotriaosylceramide in the lysosomes.
The incidence is estimated being 1/40 000. Some patients suffering from Fabry's disease today
are of childbearing age and their multidisciplinary care (by neurologists, obstetricians and
anesthetists) raises several questions.
About the anesthetic, the question of epidural block is debated in patients with neurological
diseases and recommendations are not unequivocal.
Indeed one of the problems of the management in those conditions is the potential worsening
of the disease because of the anesthetic procedure. In addition, the possibility of an
antiplatelet and / or an anticoagulant treatment in these patients may also contre-indicate
an epidural block.
The rate of epidural block achieved in patients with Fabry's disease is not currently known.
Moreover, only sparse data on pregnancy outcomes in these patients are reported.
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