Pyrophosphate Homeostasis and Hepatic Expression of ABCC6.Pyro-TH

NCT03576859 | Completed

• 1 other identifier: 18-AOI-11
• Study Type: interventional
• Target: 38
• Locations: 1 country
• Sites: 1

Brief Summary

In animals, normal hepatic expression of ABCC6 (ATP-binding transporter cassette, subfamily C, member 6) determines plasma pyrophosphate (PPi) concentration. PPi prevents the formation of hydroxyapatite crystals on tissues by precipitation of calcium and inorganic phosphate (Pi). It is an endogenous compound whose deficiency causes diffuse vascular calcifications in certain rare monogenic diseases, including the elastic pseudoxanthoma caused by the mutation of ABCC6. PPi is produced by enzymatic transformation of extracellular ATP and, in animals, the liver is the main supplier of ATP and PPi (more than 90%). In humans, liver transplantation offers the possibility of correlating the plasma concentration of PPi (\[PPi\]pl) with hepatic expression of ABCC6. Liver transplantation is performed in the treatment of chronic liver failure (Child B or C) or, in the absence of liver failure, in the treatment of hepatocellular carcinoma. By measuring\[PPi\]pl before transplantation and after liver function restoration and by measuring ABCC6 in the diseased liver and healthy liver, it is possible to determine whether liver failure is associated with decreased\[PPi\]pl and decreased liver expression of ABCC6, which is the objective of our pilot study. Its interest is to establish a physiopathological link between the frequent vascular calcifications in obese patients with hepatic steatosis and the production of PPi. prupose: Look for a deficit in\[PPi\]pl in patients before the transplant compared to the phase of restoration of liver function

Conditions

Hepatic Failure

Outcome Measures

Primary Outcomes (1)

• plasma PPi concentration between before transplantation and liver function restoration in the both arms

  24 months

Study Arms (2)

cirrhotic patients with chronic liver failure

OTHER

Other: blood sample

cirrhotic patients without chronic liver failure

OTHER

Other: blood sample

Interventions

blood sample OTHER

Peripheral venous blood is collected before liver transplantation and after restoration of liver function

cirrhotic patients with chronic liver failure; cirrhotic patients without chronic liver failure

Eligibility Criteria

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

You may qualify if:

• Indication for liver transplantation Age greater than or equal to 18 years Patients affiliated to social security

You may not qualify if:

• Patient not affiliated to social security Non-consenting patient or unable to understand the protocol and how it works Fulminant Hepatitis

Sponsors & Collaborators

• Centre Hospitalier Universitaire de Nice: lead

Study Sites (1)

Untiversity Hospital of Nice

Nice, 06000, France

Location

Related Publications (1)

• Laurain A, Rubera I, Razzouk-Cadet M, Bonnafous S, Albuquerque M, Paradis V, Patouraux S, Duranton C, Lesaux O, Leftheriotis G, Tran A, Anty R, Gual P, Iannelli A, Favre G. Arterial Calcifications in Patients with Liver Cirrhosis Are Linked to Hepatic Deficiency of Pyrophosphate Production Restored by Liver Transplantation. Biomedicines. 2022 Jun 24;10(7):1496. doi: 10.3390/biomedicines10071496.

  PMID: 35884801 RESULT

MeSH Terms

Conditions

Liver Failure

Interventions

Blood Specimen Collection

Condition Hierarchy (Ancestors)

Hepatic Insufficiency; Liver Diseases; Digestive System Diseases

Intervention Hierarchy (Ancestors)

Specimen Handling; Clinical Laboratory Techniques; Diagnostic Techniques and Procedures; Diagnosis; Punctures; Surgical Procedures, Operative; Investigative Techniques

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NON RANDOMIZED
• Masking: NONE
• Purpose: BASIC SCIENCE
• Intervention Model: PARALLEL
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: June 22, 2018
• First Posted: July 3, 2018
• Study Start: November 6, 2018
• Primary Completion: October 29, 2020
• Study Completion: October 29, 2020
• Last Updated: March 24, 2026

Record last verified: 2026-03

Data Sharing

• IPD Sharing: Will not share

Locations

FR (1)