Fabry's Disease and Pregnancy (PREFAB)

Terminated

• Conditions: Fabry's Disease; Pregnancy

• Registration Number: NCT02582294
• Lead Sponsor: Groupe Hospitalier Diaconesses Croix Saint-Simon

Brief 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.

Detailed Description

Not available

Study Timeline

• Study Start: 2015-10
• Study First Submitted: 2015-10-15
• Study First Submitted QC: 2015-10-19
• Study First Posted: 2015-10-21
• Primary Completion: 2018-06-01
• Study Completion: 2018-06-01
• Status Verified: 2019-02
• Last Update Submitted: 2019-02-14
• Last Update Posted: 2019-02-15

Recruitment & Eligibility

• Status: TERMINATED
• Sex: Female
• Target Recruitment: 12

Inclusion Criteria

• Fabry's disease patients, aged 18 years or more, having had at least one childbirth

Exclusion Criteria

• Patient under a legal protection procedure
• Patient denying to participate to the study
• Lack of affiliation to a social security system

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Proportion of Fabry's disease patients having had an epidural block in childbirth.	The duration of the interview for this study with women with Fabry disease's will be one hour

Trial Locations

Locations (1)

Groupe hospitalier Diaconesses Croix Saint Simon; 🇫🇷 Paris, Ile De France, France