Description of Obstetrical Care, Mainly Anesthesic, in Patients With Fabry's Disease
试验速览
- 阶段
- 不适用
- 状态
- 终止
- 发起方
- 入组人数
- 12
- 试验地点
- 1
- 主要终点
- Proportion of Fabry's disease patients having had an epidural block in childbirth.
研究概览
简要总结
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.
研究设计
- 研究类型
- Observational
- 观察模型
- Other
- 时间视角
- Retrospective
入排标准
- 年龄范围
- 18 Years 至 45 Years（Adult）
- 性别
- Female
- 接受健康志愿者
- 否
入选标准
- •Fabry's disease patients, aged 18 years or more, having had at least one childbirth
排除标准
- •Patient under a legal protection procedure
- •Patient denying to participate to the study
- •Lack of affiliation to a social security system
结局指标
主要结局
Proportion of Fabry's disease patients having had an epidural block in childbirth.
时间窗: The duration of the interview for this study with women with Fabry disease's will be one hour
次要结局
未报告次要终点