NCT02582294

Fabry's Disease and Pregnancy (PREFAB)

Description of Obstetrical Care, Mainly Anesthesic, in Patients With Fabry's Disease

• Status: Terminated
• Phase: —
• Study type: Observational
• Enrollment: 12 (actual)
• Sponsor: Groupe Hospitalier Diaconesses Croix Saint-Simon · Academic / Other
• Sex: Female
• Age: 18 Years – 45 Years
• Healthy volunteers: Not accepted

Summary

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.

Conditions

• Fabry's Disease
• Pregnancy

Interventions

Type	Name	Description
OTHER	Questionnaire

Timeline

• Start date: 2015-10-01
• Primary completion: 2018-06-01
• Completion: 2018-06-01
• First posted: 2015-10-21
• Last updated: 2019-02-15

Locations

1 site across 1 country: France