Comparative Study of Different Non-invasive Measurement Methods of Hepatic Fibrosis in Cystic Fibrosis
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 56
- 试验地点
- 2
- 主要终点
- Measure of hepatic elasticity by Fibro Test
研究概览
简要总结
Non-invasive methods of the quantification of fibrosis may help to assess the development of fibrosis at a specific moment of the evolution of the disease, in the order to decide the initiation of treatment with ursodeoxycholic acid which may slow the progression to hepatic cirrhosis:
- FibroTest,
- The ultrasound impulse elastography, Fibroscan (FS)
- Ultrasound Elastography by ShearWave (SWE)
- Magnetic resonance elastography (MRE). The purpose of this study is to assess the contribution of these methods in the diagnosis of hepatic fibrosis during the evolution of the cystic fibrosis.
详细描述
The incidence of hepatobiliary disease in cystic fibrosis in the first 10 years of life is 2.5 to 1.8 / 100 patient-years, significantly decreasing during the second decade; generally 30-40% of children develop hepatic disease before 12 years. However, the hepatobiliary damage varies in its clinical and histological expression. The typical histological appearance of the liver is the focal biliary cirrhosis (or fibrosis) characterized by a heterogeneous distribution of portal fibrosis in the liver.
Complications of cirrhosis are responsible for 2.5% of deaths in patients with cystic fibrosis and represent the second cause of death after lung disease if we exclude deaths secondary to lung transplantation.
The diagnosis of hepatic disease at an early stage of development and the assessment of the progression of fibrosis are so difficult in the absence of sensitive and specific non-invasive diagnostic tests.
Non-invasive assessment of hepatic fibrosis is an important topic of study in recent years, because of the emergence of biological methods and quantitative elastography methods using ultrasound or Magnetic Resonance Imagery (MRI) and allowing quantification of the hepatic elasticity, which is assumed to be a reflection of fibrosis. The advantages of these methods are numerous:
- The assessment of wider hepatic parenchyma then hepatic biopsy, allowing overcome the sampling bias
- Painless and non-invasive methods for the patient
- Simple to make
- Do not require hospitalization and can be performed as outpatient
- Immediately known results
- Can be repeated as often as necessary during the patient's follow-up
- Lower cost, morbidity and mortality compared to hepatic biopsy. Several non-invasive methods of fibrosis diagnosis have been studied in adults primarily for viral diseases and helped to limit the indications of hepatic biopsy. However, no method is now validated for the early diagnosis of hepatic disease and monitoring of children with cystic fibrosis.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Screening
- 盲法
- None
入排标准
- 年龄范围
- 6 Years 至 18 Years（Child, Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Children with cystic fibrosis aged 6-18 years. The diagnosis of cystic fibrosis will be based on testing the abnormal sweat (> 60 mEq / L of chloride sweat) and / or on the presence of two pathogen mutations of CFTR.
- •Written consent of parents or holders of parental rights.
- •Affiliated or benefiting from a national social insurance
排除标准
- •Presence of another disease associated with cystic fibrosis that may be responsible for a chronic hepatic disease such as deficiency of alpha-1antitrypsine ZZ genotype or chronic infection by virus B or C.
- •Child grafted
- •Actual febrile pulmonary infection that can change the value of Fibrotest
- •Important peri-hepatic ascites preventing the elasticity measurements by Fibroscan®
- •Severe respiratory insufficient preventing attainment of Magnetic Resonance Imagery
研究组 & 干预措施
MUCO-FIBRO
Measures of hepatic elasticity
干预措施: Measure of hepatic elasticity (Other)
结局指标
主要结局
Measure of hepatic elasticity by Fibro Test
时间窗: Day 0
The value of hepatic elasticity measured by each of the tests performed on the same day in the same patient
Measure of hepatic elasticity by the ultrasound impulse elastography, Fibroscan (FS)
时间窗: Month 12
The value of hepatic elasticity measured by each of the tests performed on the same day in the same patient.
次要结局
- Measure of hepatic elasticity by Ultrasound Elastography by ShearWave (SWE):(Day 0)
- Measure of hepatic elasticity by Magnetic resonance elastography (MRE):(Month 12)