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

NCT02702765 | Completed DMC

• 1 other identifier: Wilson sCD163
• Study Type: interventional
• Target: 33
• Locations: 1 country
• Sites: 1

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.

Conditions

Wilsons Disease

Keywords

sCD163; liver cirrhosis; disease severity

Outcome Measures

Primary Outcomes (2)

• Measurement of the macrophage activation markers sCD163

  For the investigations a total of 100 ml of blood is drawn, all stored in a research biobank.

  Baseline, 1 year, 2 year, 3 year

• Measurement of soluble mannose receptor (sMR)

  For the investigations a total of 100 ml of blood is drawn, all stored in a research biobank.

  Baseline, 1 year, 2 year, 3 year

Secondary Outcomes (8)

• 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

• Galactose elimination capacity is performed to evaluate metabolic liver function

  Baseline, 1 year, 2 year, 3 year

Study Arms (1)

Wilsons disease

EXPERIMENTAL

All patients will receive all interventions (galactose elimination capacity test , ultrasound, fibroscan, continuous reaction time test and functional hepatic nitrogen clearance ), except liver biopsy.

Procedure: Fibroscan; Procedure: Ultrasound; Drug: Galactose; Procedure: Liver biopsy; Procedure: Functional hepatic nitrogen clearance

Interventions

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

Wilsons disease

Ultrasound PROCEDURE

Ultrasound is a non-invasive procedure

Wilsons disease

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.

Also known as: Galactose elimination capacity

Wilsons disease

Liver biopsy PROCEDURE

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

Wilsons disease

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

Wilsons disease

Eligibility Criteria

• Age: 18 Years - 100 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• diagnosed with Wilsons disease

Sponsors & Collaborators

• University of Aarhus: lead
• Aarhus University Hospital: collaborator

Study Sites (1)

Department of Hepatology and Gastroenterology, Aarhus University Hospital

Aarhus, 8000, Denmark

Location

Related Publications (1)

• Bjorklund J, Laursen TL, Sandahl TD, Moller HJ, Vilstrup H, Ott P, Gronbaek H. High hepatic macrophage activation and low liver function in stable Wilson patients - a Danish cross-sectional study. Orphanet J Rare Dis. 2018 Sep 21;13(1):169. doi: 10.1186/s13023-018-0910-7.

  PMID: 30241550 DERIVED

MeSH Terms

Conditions

Hepatolenticular Degeneration; Liver Cirrhosis

Interventions

High-Energy Shock Waves; Galactose

Condition Hierarchy (Ancestors)

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

Intervention Hierarchy (Ancestors)

Ultrasonic Waves; Sound; Radiation, Nonionizing; Radiation; Physical Phenomena; Hexoses; Monosaccharides; Sugars; Carbohydrates

Study Officials

• Henning Grønbæk, Professor

  Aarhus University Hospital, Nørrebrogade 44, Aarhus C, Denmark, 8000

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NA
• Masking: NONE
• Purpose: DIAGNOSTIC
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: February 26, 2016
• First Posted: March 9, 2016
• Study Start: February 1, 2016
• Primary Completion: February 1, 2020
• Study Completion: February 1, 2022
• Last Updated: November 2, 2022

Record last verified: 2022-11

Locations

DK (1)