sCD163 and sMR in Wilsons Disease - Associations With Disease Severity and Fibrosis

Not Applicable

Completed

• Conditions: Wilsons Disease
• Interventions: Procedure: Fibroscan; Procedure: Ultrasound; Drug: Galactose; Procedure: Liver biopsy; Procedure: Functional hepatic nitrogen clearance

• Registration Number: NCT02702765
• Lead Sponsor: University of Aarhus

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 Description

Not available

Study Timeline

• Study Start: 2016-02
• Study First Submitted: 2016-02-26
• Study First Submitted QC: 2016-03-07
• Study First Posted: 2016-03-09
• Primary Completion: 2020-02
• Study Completion: 2022-02
• Status Verified: 2022-11
• Last Update Submitted: 2022-11-01
• Last Update Posted: 2022-11-02

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 33

Inclusion Criteria

• diagnosed with Wilsons disease

Exclusion Criteria

Study & Design

• Study Type: INTERVENTIONAL
• Study Design: SINGLE_GROUP

Arm && Interventions

Group	Intervention	Description
Wilsons disease	Fibroscan	All patients will receive all interventions (galactose elimination capacity test , ultrasound, fibroscan, continuous reaction time test and functional hepatic nitrogen clearance ), except liver biopsy.
Wilsons disease	Ultrasound	All patients will receive all interventions (galactose elimination capacity test , ultrasound, fibroscan, continuous reaction time test and functional hepatic nitrogen clearance ), except liver biopsy.
Wilsons disease	Liver biopsy	All patients will receive all interventions (galactose elimination capacity test , ultrasound, fibroscan, continuous reaction time test and functional hepatic nitrogen clearance ), except liver biopsy.
Wilsons disease	Functional hepatic nitrogen clearance	All patients will receive all interventions (galactose elimination capacity test , ultrasound, fibroscan, continuous reaction time test and functional hepatic nitrogen clearance ), except liver biopsy.
Wilsons disease	Galactose	All patients will receive all interventions (galactose elimination capacity test , ultrasound, fibroscan, continuous reaction time test and functional hepatic nitrogen clearance ), except liver biopsy.

Primary Outcome Measures

Name	Time	Method
Measurement of the macrophage activation markers sCD163	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)	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.

Secondary Outcome Measures

Name	Time	Method
Urinary copper excretion in 24 hour urine collection	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.	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)	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	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.
Functional hepatic nitrogen clearance to evaluate metabolic liver function	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	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).
Galactose elimination capacity is performed to evaluate metabolic liver function	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	Baseline, 1 year, 2 year, 3 year	Liver fibrosis will be also be determined on liver biopsies.

Trial Locations

Locations (1)

Department of Hepatology and Gastroenterology, Aarhus University Hospital; 🇩🇰 Aarhus, Denmark