Fabry's Disease and Pregnancy (PREFAB)

NCT ID: NCT02582294

Last Updated: 2019-02-15

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

TERMINATED

Total Enrollment

12 participants

Study Classification

OBSERVATIONAL

Study Start Date

2015-10-31

Study Completion Date

2018-06-01

Brief Summary

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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

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Conditions

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Fabry's Disease Pregnancy

Study Design

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Observational Model Type

OTHER

Study Time Perspective

RETROSPECTIVE

Interventions

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Questionnaire

Intervention Type OTHER

Eligibility Criteria

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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
Minimum Eligible Age

18 Years

Maximum Eligible Age

45 Years

Eligible Sex

FEMALE

Accepts Healthy Volunteers

No

Sponsors

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Groupe Hospitalier Diaconesses Croix Saint-Simon

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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Groupe hospitalier Diaconesses Croix Saint Simon

Paris, Île-de-France Region, France

Site Status

Countries

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France

Other Identifiers

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OLE_2015_4

Identifier Type: -

Identifier Source: org_study_id

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