Prevalence of Acute Hepatic Porphyria in Population With Suggestive Clinical Picture
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 发起方
- 入组人数
- 150
- 试验地点
- 1
- 主要终点
- Binary criterion corresponding to the diagnosis of acute hepatic porphyria (acute hepatic porphyria + / acute hepatic porphyria -) via physiological parameter.
研究概览
简要总结
To determine the proportion of patients suffering from acute hepatic porphyria (AHP) from different hospital departments and referred to an internist referent for a suggestive clinical picture with a first negative etiological assessment.
详细描述
In this context, this study aims to assess the prevalence of PHA in a population of patients with a suggestive clinical picture. A better knowledge of the pathology will make it possible to better guide patients and prevent them from diagnostic wandering fraught with physical and psychological consequences.
This is an observational, ambispective, transversal, multicenter study carried out in France. The goal is to recruit a cohort that will reflect current practice. 500 patients will be recruted.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Cross Sectional
入排标准
- 年龄范围
- 18 Years 至 60 Years（Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Man or woman aged 18 to 60;
- •Presenting severe abdominal pain, afebrile and diffuse over several days, evolving for less than 5 years;
- •With at least one of the following symptoms:
- •Peripheral neurological symptoms: decreased driving force and pain in the limbs (roots of the thighs), etc. ;
- •Central neurological symptoms: psychiatric (depression, anxiety, insomnia), disturbances of consciousness, etc. ;
- •Autonomous neurological symptoms: tachycardia, nausea, vomiting, constipation, etc.
- •With a first negative etiological assessment requested by an emergency physician, gastroenterologist, gynecologist, neurologist, internist or another specialty, or with a diagnosis of acute hepatic porphyria less than 6 months;
- •Patient able to understand the information related to the study and having indicated his non-objection to the collection of data concerning him;
排除标准
- •Patient with a diagnosis of acute hepatic porphyria for more than 6 months;
- •Patient unfit to participate in the study, due to cognitive or linguistic difficulties;
- •Protected patient (under legal protection, or deprived of liberty by judicial or administrative decision).
结局指标
主要结局
Binary criterion corresponding to the diagnosis of acute hepatic porphyria (acute hepatic porphyria + / acute hepatic porphyria -) via physiological parameter.
时间窗: 1 day
The diagnosis of acute hepatic porphyria is based on urine testing of the neurotoxic precursors of haem: Delta-aminolevulinic acid and Porphobilinogen. A patient will be considered to have acute hepatic porphyria (acute hepatic porphyria +) if: * Delta-aminolevulinic acid ≥ 3 µmol / mol Cr And or * Porphobilinogen ≥ 1 µmol / mol Cr Otherwise, the patient will be considered to be acute hepatic porphyria free (acute hepatic porphyria -).
次要结局
未报告次要终点