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NCT Number: NCT02702765

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

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.

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Key information

Conditions

Wilsons Disease Basal Ganglia Diseases Brain Diseases Brain Diseases, Metabolic Brain Diseases, Metabolic, Inborn Central Nervous System Diseases Congenital, Hereditary, and Neonatal Diseases and Abnormalities Digestive System Diseases Fibrosis Genetic Diseases, Inborn Hepatolenticular Degeneration Heredodegenerative Disorders, Nervous System Liver Cirrhosis Liver Diseases Metabolic Diseases Metabolism, Inborn Errors Metal Metabolism, Inborn Errors Movement Disorders Nervous System Diseases Neurodegenerative Diseases Nutritional and Metabolic Diseases Pathologic Processes Pathological Conditions, Signs and Symptoms

Age range

18 year–100 year

Sex eligibility

All sexes

Study type

Interventional

Phase

Not applicable

Primary location

Department of Hepatology and Gastroenterology, Aarhus University Hospital

Aarhus, 8000, Denmark

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

  • diagnosed with Wilsons disease

Exclusion criteria

-

Treatment and study plan

Fibroscan

Procedure

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

Ultrasound

Procedure

Ultrasound is a non-invasive procedure

galactose

Drug

Galactose elimination capacity is performed to evaluate metabolic liver function.

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.

Other names: Galactose elimination capacity

Liver biopsy

Procedure

Histological disease activity at time of diagnosis evaluating if any liver fibrosis

Functional hepatic nitrogen clearance

Procedure

Functional hepatic nitrogen clearance to evaluate metabolic liver function Functional hepatic nitrogen clearance requires a 12-hour fast, two venflons, the infusion of alanine, and urine sampling for 4 hours

Primary outcomes

  1. 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.

  2. 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.

Secondary outcomes

  1. 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

  2. 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.

  3. 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.

  4. 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.

  5. 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.

  6. 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.

  7. 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.

  8. 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).

Sponsors and collaborators

Lead sponsor

University of Aarhus

Other

Collaborators

  • Aarhus University Hospital

Registry information

Official study title

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

Important dates

Study start
2016
Primary completion
2020
Study completion
2022
First posted
Mar 9, 2016
Registry last updated
Nov 2, 2022

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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