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Research Project

Transient Elastography for Liver Assessment in severe Alpha-1 Antitrypsin Deficiency (PiZZ): Defining Normal Values for Clinical Practice

Principal Investigator:
Hanan Tanash
Center:
Skäne University Hospital
City/Country:
Malmö (Sweden)
Start date:
April 2026
Status:
Ongoing
Contact E-mail:
hanan.tanash@med.lu.se
_ALT

Introduction

Transient elastography (FibroScan®) is widely used for non-invasive assessment of liver stiffness, with established reference values derived from heterogeneous liver disease populations.
These reference values are currently applied to individuals with AATD, despite their unique disease mechanism. It is therefore plausible that normal or reference FibroScan values may be higher in individuals with AATD who have no clinical or biochemical evidence of liver disease.

The lack of AATD-specific reference values may lead to misclassification and unnecessary referral to specialist hepatology care. Establishing AATD-specific reference values for FibroScan may improve risk stratification, reduce unnecessary hepatology referrals, and support broader involvement of non-hepatology clinicians in the monitoring of liver disease in individuals with severe AATD.

Objectives

The primary aim of this study is to identify reference (normal) FibroScan values in individuals with severe AATD (PiZZ) without evidence of liver disease.

The secondary aims are:
-To establish sex-specific reference values for liver stiffness in severe AATD.
-To analyse the correlation between liver stiffness measured by transient elastography and non-invasive serum-based fibrosis scores, including FIB-4 and APRI.

Inclusion criteria

To derive reference values, a subgroup of PiZZ individuals without evidence of liver disease is defined using the following criteria:

-No known history of chronic liver disease
-No clinical signs or symptoms of liver disease
-Normal liver biochemistry (AST, ALT, ALP, GGT, bilirubin, albumin, PT/INR, platelet count)
-Negative viral hepatitis serology (hepatitis A, B, and C)
-Negative autoimmune liver disease markers (ANA, AMA, SMA)
-FIB-4 <1.3
-APRI <0.5
-No history of excessive alcohol consumption based on questionnaire data

Brief summary

This will be a retrospective analysis on data from EARCO International registry in PiZZ individuals