Macrophages and the Macrophage Activation Markers sCD163 and Mannose Receptor (sMR) in Patients With Wilsons Disease - Associations With Liver Disease Severity and Fibrosis
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 33
- 试验地点
- 1
- 主要终点
- Measurement of the macrophage activation markers sCD163
研究概览
简要总结
The aim is to investigate macrophage activation markers and correlations to liver fibrosis in patients with Wilsons Disease. Researchers wish to investigate associations to neurologic and metabolic liver function. Researchers will assess this by comparing blood samples with fibrosis and liver function analyses. This study provides new insight into macrophages and their involvement in Wilsons Disease.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Diagnostic
- 盲法
- None
入排标准
- 年龄范围
- 18 Years 至 100 Years（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •diagnosed with Wilsons disease
排除标准
- 未提供
研究组 & 干预措施
Wilsons disease
All patients will receive all interventions (galactose elimination capacity test , ultrasound, fibroscan, continuous reaction time test and functional hepatic nitrogen clearance ), except liver biopsy.
干预措施: Fibroscan (Procedure)
Wilsons disease
All patients will receive all interventions (galactose elimination capacity test , ultrasound, fibroscan, continuous reaction time test and functional hepatic nitrogen clearance ), except liver biopsy.
干预措施: Ultrasound (Procedure)
Wilsons disease
All patients will receive all interventions (galactose elimination capacity test , ultrasound, fibroscan, continuous reaction time test and functional hepatic nitrogen clearance ), except liver biopsy.
干预措施: Galactose (Drug)
Wilsons disease
All patients will receive all interventions (galactose elimination capacity test , ultrasound, fibroscan, continuous reaction time test and functional hepatic nitrogen clearance ), except liver biopsy.
干预措施: Liver biopsy (Procedure)
Wilsons disease
All patients will receive all interventions (galactose elimination capacity test , ultrasound, fibroscan, continuous reaction time test and functional hepatic nitrogen clearance ), except liver biopsy.
干预措施: Functional hepatic nitrogen clearance (Procedure)
结局指标
主要结局
Measurement of the macrophage activation markers sCD163
时间窗: Baseline, 1 year, 2 year, 3 year
For the investigations a total of 100 ml of blood is drawn, all stored in a research biobank.
Measurement of soluble mannose receptor (sMR)
时间窗: Baseline, 1 year, 2 year, 3 year
For the investigations a total of 100 ml of blood is drawn, all stored in a research biobank.
次要结局
- Urinary copper excretion in 24 hour urine collection(Baseline, 1 year, 2 year, 3 year)
- Ultrasound is performed for signs of liver cirrhosis.(Baseline, 1 year, 2 year, 3 year)
- Fibroscan is performed to evaluate liver stiffness (fibrosis)(Baseline, 1 year, 2 year, 3 year)
- Continous Reaction Time to evaluate brain dysfunction(Baseline, 1 year, 2 year, 3 year)
- Functional hepatic nitrogen clearance to evaluate metabolic liver function(Baseline, 1 year, 2 year, 3 year)
- The Portosystemic Encephalopathy to evaluate brain dysfunction(Baseline, 1 year, 2 year, 3 year)
- Galactose elimination capacity is performed to evaluate metabolic liver function(Baseline, 1 year, 2 year, 3 year)
- Histological disease activity at time of diagnosis and liver biopsy, evaluating if any liver fibrosis(Baseline, 1 year, 2 year, 3 year)