Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Name | Class |
|---|---|
| Aarhus University Hospital | OTHER |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
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.
Not provided
Not provided
Not provided
Not provided
Not provided
| Label | Type | Description | Intervention Names |
|---|---|---|---|
| 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. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| 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 |
|
| Measure | Description | Time Frame |
|---|---|---|
| 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 |
| Measure | Description | Time Frame |
|---|---|---|
| Urinary copper excretion in 24 hour urine collection | 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 | Baseline, 1 year, 2 year, 3 year |
| Ultrasound is performed for signs of liver cirrhosis. |
Not provided
Inclusion Criteria:
Exclusion Criteria:
-
Not provided
Not provided
Not provided
Not provided
Not provided
| Name | Affiliation | Role |
|---|---|---|
| Henning Grønbæk, Professor | Aarhus University Hospital, Nørrebrogade 44, Aarhus C, Denmark, 8000 | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Department of Hepatology and Gastroenterology, Aarhus University Hospital | Aarhus | 8000 | Denmark |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 30241550 | Derived | 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. |
Not provided
Not provided
Not provided
| ID | Term |
|---|---|
| D006527 | Hepatolenticular Degeneration |
| D008103 | Liver Cirrhosis |
| ID | Term |
|---|---|
| D008107 | Liver Diseases |
| D004066 | Digestive System Diseases |
| D001480 | Basal Ganglia Diseases |
| D001927 | Brain Diseases |
Not provided
Not provided
| ID | Term |
|---|---|
| D019220 | High-Energy Shock Waves |
| D005690 | Galactose |
| ID | Term |
|---|---|
| D000069453 | Ultrasonic Waves |
| D013016 | Sound |
| D011840 | Radiation, Nonionizing |
| D011827 | Radiation |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| 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. |
|
|
| 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 |
|
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. |
| Baseline, 1 year, 2 year, 3 year |
| Fibroscan is performed to evaluate liver stiffness (fibrosis) | 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. | Baseline, 1 year, 2 year, 3 year |
| Continous Reaction Time to evaluate brain dysfunction | Continous Reaction Time is a computerized 10 minutes test that measures and combines motor reaction speed and sustained attention. | Baseline, 1 year, 2 year, 3 year |
| 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. | Baseline, 1 year, 2 year, 3 year |
| Histological disease activity at time of diagnosis and liver biopsy, evaluating if any liver fibrosis | Liver fibrosis will be also be determined on liver biopsies. | Baseline, 1 year, 2 year, 3 year |
| Functional hepatic nitrogen clearance to evaluate metabolic liver function | 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. | Baseline, 1 year, 2 year, 3 year |
| The Portosystemic Encephalopathy to evaluate brain dysfunction | 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). | Baseline, 1 year, 2 year, 3 year |
| D002493 |
| Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
| D020739 | Brain Diseases, Metabolic, Inborn |
| D001928 | Brain Diseases, Metabolic |
| D009069 | Movement Disorders |
| D020271 | Heredodegenerative Disorders, Nervous System |
| D019636 | Neurodegenerative Diseases |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D008661 | Metabolism, Inborn Errors |
| D008664 | Metal Metabolism, Inborn Errors |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
| D005355 | Fibrosis |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |
| D055585 |
| Physical Phenomena |
| D006601 | Hexoses |
| D009005 | Monosaccharides |
| D000073893 | Sugars |
| D002241 | Carbohydrates |