Clinical Trial: sCD163 and sMR in Wilsons Disease - Associations With Disease Severity and Fibrosis

Study Status: Recruiting
Recruit Status: Recruiting
Study Type: Interventional

Official Title: Macrophages and the Macrophage Activation Markers sCD163 and Mannose Receptor (sMR) in Patients With Wilsons Disease - Associations With Liver Disease Severity and Fibrosi

Brief Summary: 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.

Detailed Summary:
Sponsor: University of Aarhus

Current Primary Outcome:

• Measurement of the macrophage activation markers sCD163 [ Time Frame: 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) [ Time Frame: 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.

Original Primary Outcome:

• Measurement of the macrophage activation markers sCD163 [ Time Frame: 1 year ]
  For the investigations a total of 100 ml of blood is drawn, all stored in a research biobank.
• Measurement of soluble mannose recepto (sMR) [ Time Frame: 1 year ]
  For the investigations a total of 100 ml of blood is drawn, all stored in a research biobank.

Current Secondary Outcome:

• Urinary copper excretion in 24 hour urine collection [ Time Frame: Baseline, 1 year, 2 year, 3 year ]
  The patient collects urine for 24 hours at home in a designated container, which is handed out at the department. The container is kept refrigerated and is brought to the control
• Ultrasound is performed for signs of liver cirrhosis. [ Time Frame: Baseline, 1 year, 2 year, 3 year ]
  Ultrasound is a non-invasive procedure. Signs og liver cirrhosis by ultrasound are surface modularity, a smaller liver, heterogeneous echo texture and signs of portal hypertension.
• Fibroscan is performed to evaluate liver stiffness (fibrosis) [ Time Frame: Baseline, 1 year, 2 year, 3 year ]
  Liver fibrosis will be determined using fibroscan, and reported as changes in the amount of fibrosis in the liver. The fibroscan is a non-invasive procedure.
• Continous Reaction Time to evaluate brain dysfunction [ Time Frame: Baseline, 1 year, 2 year, 3 year ]
  Continous Reaction Time is a computerized 10 minutes test that measures and combines motor reaction speed and sustained attention.
• Galactose elimination capacity is performed to evaluate metabolic liver function [ Time Frame: Baseline, 1 year, 2 year, 3 year ]
  The metabolic liver function test Galactose elimination capacity requires a 6-hour fast, the infusion of galactose, blood sampling from the ear lobe, and collection of urine for 4 hours.
• Histological disease activity at time of diagnosis and liver biopsy, evaluating if any liver fibrosis [ Time Frame: Baseline, 1 year, 2 year, 3 year ]
  Liver fibrosis will be also be determined on liver biopsies.
• Functional hepatic nitrogen clearance to evaluate metabolic liver function [ Time Frame: Baseline, 1 year, 2 year, 3 year ]
  Functional hepatic nitrogen clearancerequires a 12-hour fast, two venflons, the infusion of alanine, and urine sampling for 4 hours. It evaluates the metabolic liver function by measuring the clearance of alanine from blod by analyzing the amount og urea in the urine collected 4 hours after the start og the alanine infusion.
• The Portosystemic Encephalopathy to evaluate brain dysfunction [ Time Frame: Baseline, 1 year, 2 year, 3 year ]
  The Portosystemic Encephalopathy test is a 15-minute paper-pencil test battery comprised of 5 sub-tests: Digit Symbol test (DST), Number Connection Test A (NCT-A), Number Connection Test B (NCT-B), Serial Dotting Test (SDOT), and Line Tracing Test (LTT, time and errors).

Original Secondary Outcome:

• Urinary copper excretion in 24 hour urine collection [ Time Frame: 1 year ]
  The patient collects urine for 24 hours at home in a designated container, which is handed out at the department. The container is kept refrigerated and is brought to the control
• Ultrasound is performed for signs of liver cirrhosis. [ Time Frame: 1 year ]
  Ultrasound is a non-invasive procedure. Signs og liver cirrhosis by ultrasound are surface modularity, a smaller liver, heterogeneous echo texture and signs of portal hypertension.
• Fibroscan is performed to evaluate liver stiffness (fibrosis) [ Time Frame: 1 year ]
  Liver fibrosis will be determined using fibroscan, and reported as changes in the amount of fibrosis in the liver. The fibroscan is a non-invasive procedure.
• Continous Reaction Time to evaluate brain dysfunction [ Time Frame: 1 year ]
  Continous Reaction Time is a computerized 10 minutes test that measures and combines motor reaction speed and sustained attention.
• Galactose elimination capacity is performed to evaluate metabolic liver function [ Time Frame: 1 year ]
  The metabolic liver function test Galactose elimination capacity requires a 6-hour fast, the infusion of galactose, blood sampling from the ear lobe, and collection of urine for 4 hours.
• Histological disease activity at time of diagnosis and liver biopsy, evaluating if any liver fibrosis [ Time Frame: 1 year ]
  Liver fibrosis will be also be determined on liver biopsies.
• Functional hepatic nitrogen clearance to evaluate metabolic liver function [ Time Frame: 1 year ]
  Functional hepatic nitrogen clearancerequires a 12-hour fast, two venflons, the infusion of alanine, and urine sampling for 4 hours. It evaluates the metabolic liver function by measuring the clearance of alanine from blod by analyzing the amount og urea in the urine collected 4 hours after the start og the alanine infusion.
• The Portosystemic Encephalopathy to evaluate brain dysfunction [ Time Frame: 1 year ]
  The Portosystemic Encephalopathy test is a 15-minute paper-pencil test battery comprised of 5 sub-tests: Digit Symbol test (DST), Number Connection Test A (NCT-A), Number Connection Test B (NCT-B), Serial Dotting Test (SDOT), and Line Tracing Test (LTT, time and errors).

Information By: University of Aarhus

Dates:
Date Received: February 26, 2016
Date Started: February 2016
Date Completion: February 2020
Last Updated: October 27, 2016
Last Verified: October 2016