Fabry's Disease and Pregnancy (PREFAB)

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

Status

Terminated

Phases

Unknown

Study type

Observational

Source

ClinicalTrials.gov

Registry ID

NCT02582294

Acronym

PREFAB

Enrollment

Registered

2015-10-21

Start date

2015-10-31

Completion date

2018-06-01

Last updated

2019-02-15

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

Fabry's Disease, Pregnancy

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.

Interventions

OTHERQuestionnaire

Study design

Observational model

OTHER

Time perspective

RETROSPECTIVE

Eligibility

Sex/Gender

FEMALE

Age

18 Years to 45 Years

Healthy volunteers

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

Design outcomes

Primary

Measure	Time frame
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

Countries

France

Outcome results

None listed

Source: ClinicalTrials.gov · Data processed: Feb 4, 2026