Liver and the Spleen Stifness in Adult Patients With Gaucher's Disease Using Ultrasound Shear Wave Elastography

Liver Fibrosis Clinical Trial

Official title:

Prospective Study: Shear-Wave Elastography of the Liver and the Spleen in Adult Patients With Gaucher's Disease

Clinical Trial Details — Status: Terminated

Administrative data

• NCT number: NCT03896607
• Other study ID #: 0135-18-EMC
• Status: Terminated
• Start date: March 30, 2019
• Est. completion date: October 31, 2022

Study information

• Verified date: March 2023
• Source: HaEmek Medical Center, Israel
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational

Clinical Trial Summary

The investigated cohort will examine liver and spleen fibrosis in patients with Gaucher Disease(GD) by using Shear Wave Elastography- SWE to evaluation fibrosis of the tissue and to check the correlation of fibrosis with the biomarkers of disease severity.

Description:

To assess liver and spleen stiffness measurement using Shear Wave Elastography-SWE and evaluate liver and spleen fibrosis in patients with Gaucher Disease. To compare the elastography with the biomarkers that taken at the GD haematology clinic and to check the correlation with severity of liver and spleen fibrosis. SWE is a safe with diagnostic accuracy regarding the liver (& Spleen) fibrosis. Estimating spleen fibrosis is an innovative approach in liver disease and Gaucher. The evaluation of fibrosis with this relatively new and safe method could avoid complications and invasive procedure in GD patients .

Recruitment information / eligibility

• Status: Terminated
• Enrollment: 18
• Est. completion date: October 31, 2022
• Est. primary completion date: October 31, 2022
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

Inclusion Criteria:

1. Patients with Gaucher disease over the age of 18 years.

2. Patients able to sign informed consent form.

3. Women are not pregnant.

4. Patients who read and understood the contents of the consent form and gave their written consent to participate in the study.

Exclusion Criteria:

1. Participants under the age of 18.

2. Pregnant women.

3. Participants are unable to sign an informed consent form.

4. Participants with known liver disease not associated with Gaucher disease such as liver cirrhosis, patients with HBV, HBC or liver cirrhosis.

Related Conditions & MeSH terms

• Fibrosis
• Gaucher Disease
• Liver Cirrhosis
• Liver Fibrosis
• Spleen; Fibrosis

Locations

Country	Name	City	State
Israel	HaEmek Medical Center	Afula	Tsafon

Sponsors (1)

Lead Sponsor	Collaborator
HaEmek Medical Center, Israel

Country where clinical trial is conducted

Israel, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Other	Biomarkers of the Gaucher Disease	Gaucher Disease (GD) patients will be referred from the (GD) hematology outpatient clinic, in different patients different biomarker are already taken (as blood test).; Biochemical abnormalities which might be already taken from patients, for example; blood level of ferritin (unit: ng/mL), angiotensin converting enzyme (unit: kU/L), chitotriosidase (unit: U/L) and polyclonal immunoglobulins (unit: ng/dL) .; Elevated biomarkers can predict the severity of the disease.	Up to 1 month
Primary	Shear Wave Elastography of liver to asses liver fibrosis in Gaucher disease patients.	Shear wave elastography(SWE),an ultrasound imaging technique that can produce 2D and 3D images and quantified measurements of tissue stiffness noninvasively. SWE obtained from the right liver lobe for staging liver fibrosis according to Metavir Fibrosis Score with sensitivity86%-98% and specificity 90%-93%. SWE well corelated with Metavir Fibrosis Score which is system used to assess the extent of inflammation and fibrosis by histopathological evaluation in a liver biopsy. The biomarkers of disease severity will be correlated to METAVIR score. METAVIR score(F0-F4) ,Fibrosis Level measured by KPa(kiloPascal) using SWE. F0 = Below 5 KPa, No fibrosis. F1=5.0-7.1 KPa, Mild Fibrosis. F2=7.1-8.7 KPa, Significant Fibrosis. F3=8.7-10.4 KPa, Sever Fibrosis. F4=10.4-19 KPa ,Cirrhosis. SWE can differentiate patients with no or minimal (F0 and F1) fibrosis from those with severe fibrosis or cirrhosis (F3 and F4) with no need for biopsy unless there are other factors that need to be considered.	0-2 hours
Secondary	Shear Wave Elastography of spleen to asses spleen fibrosis in Gaucher disease patients.	Shear wave elastography (SWE), an ultrasound imaging technique that can produce 2D and 3D images and quantified measurements of tissue stiffness noninvasively.; Participants with Gaucher disease will under go shear wave elastography for spleen.; Ultrasound Elastography is a non-invasive test which uses the technology of Shear Wave Elastography (SWE) obtained from the spleen for staging tissue fibrosis according to a study, Spleen Elastography was performed in 59 healthy individuals and the mean value measured in KPa (kiloPascal) was 16.6 ± 2.5 kPa, independent of patient age, sex or spleen size.; above the normal value it is considerd fibrosis of the tisuue of the spleen.	0-2 hours