Plasma Exchange and Continuous Hemodiafiltration in Treatment of Wilson’s Disease-related Liver Failure

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Brief Title

Plasma Exchange and Continuous Hemodiafiltration in Treatment of Wilson's Disease-related Liver Failure

Official Title

Clinical Efficacy of Artificial Liver Support System Using Combination of Plasma Exchange and Continuous Hemodiafiltration in Treatment of Wilson's Disease - Related Liver Failure

Brief Summary

      This study is to investigate the clinical efficacy of artificial liver support system using
      combination of plasma exchange and continuous hemodiafiltration in treatment of Wilson's
      Disease - related liver failure. 30 patients will receive treatment of plasma exchange and
      continuous hemodiafiltration and internal medicine. 30 patients will receive treatment of
      internal medicine.
    

Detailed Description

      Wilson's disease (WD) is an inherited disorder in which defective biliary excretion of copper
      leads to its accumulation, particularly in liver and brain. Presenting symptoms of liver
      disease can be highly variable, ranging from asymptomatic, with only biochemical
      abnormalities, to overt cirrhosis with all its complications. Acute liver failure (ALF)
      presentation is the most dramatic and may arise with catastrophic suddenness. It is
      considered rare, constituting only 3% of ALF cases in the pediatric ALF series in USA and
      associated with a high mortality reaching 95%. Patients with acute liver failure due to WD
      require liver transplantation, which is lifesaving. Kido J et al reported clinical outcomes
      of patients with liver failure were improved by artificial liver support system using
      combination of plasma exchange and continuous hemodiafiltration. So the investigators design
      this protocol: 30 patients will receive treatment of plasma exchange and continuous
      hemodiafiltration and internal medicine, 30 patients will receive treatment of internal
      medicine.
    


Study Type

Interventional


Primary Outcome

survival rate

Secondary Outcome

 liver function improvement

Condition

Wilson Disease

Intervention

artificial liver support system

Study Arms / Comparison Groups

 Artificial liver support system group
Description:  30 patients will receive treatment of artificial liver support system using combination of plasma exchange and continuous hemodiafiltration and internal medicine.

Publications

* Includes publications given by the data provider as well as publications identified by National Clinical Trials Identifier (NCT ID) in Medline.

Recruitment Information


Recruitment Status

Other

Estimated Enrollment

60

Start Date

June 1, 2018

Completion Date

July 31, 2020

Primary Completion Date

June 28, 2020

Eligibility Criteria

        Inclusion Criteria:

          1. Clinical diagnosis of Wilson Disease: serum ceruloplasmin < 0.2g/L, 24 hours urinary
             copper > 40μg, Kayser-Fleischer ring present;

          2. Do not receive drugs to promote the excretion of copper ever before;

          3. Serum total bilirubin > 10 times upper limit of normal, prothrombin time activity <
             40% or prothrombin time international ratio > 1.5.

        Exclusion Criteria:

          1. Other active liver diseases;

          2. Hepatocellular carcinoma or other malignancy;

          3. Pregnancy or lactation;

          4. Human immunodeficiency virus infection or congenital immune deficiency diseases;

          5. Severe diabetes, autoimmune diseases;

          6. Other important organ dysfunctions.
      

Gender

All

Ages

18 Years - 65 Years

Accepts Healthy Volunteers

No

Contacts

Liang Peng, Doctor, , 

Location Countries

China

Location Countries

China

Administrative Informations


NCT ID

NCT03589820

Organization ID

XWX1


Responsible Party

Principal Investigator

Study Sponsor

Third Affiliated Hospital, Sun Yat-Sen University


Study Sponsor

Liang Peng, Doctor, Principal Investigator, Third Affiliated Hospital, Sun Yat-Sen University


Verification Date

July 2018