Mechanisms of immunity in acutely decompensated cirrhosis and acute‐on‐chronic liver failure

C Engelmann, IW Zhang, J Clària - Liver International, 2023 - Wiley Online Library
The identification of systemic inflammation (SI) as a central player in the orchestration of
acute‐on‐chronic liver failure (ACLF) has opened new avenues for the understanding of the …

Alpha1-antitrypsin deficiency: An updated review

JF Mornex, J Traclet, O Guillaud, M Dechomet… - La Presse Médicale, 2023 - Elsevier
Abstract Alpha1-antitrypsin deficiency (AATD) is a rare autosomal recessive disease
associated with the homozygous Z variant of the SERPINA1 gene. Clinical expression of …

[HTML][HTML] Alpha-1 antitrypsin Pi∗ Z allele is an independent risk factor for liver transplantation and death in patients with advanced chronic liver disease

L Balcar, B Scheiner, M Urheu, P Weinberger… - JHEP Reports, 2022 - Elsevier
Background & Aims Alpha-1 antitrypsin (AAT) deficiency causes/predisposes individuals to
advanced chronic liver disease (ACLD). However, the role of the SERPINA1 Pi∗ Z allele in …

The polymorphic variant of SerpinB3 (SerpinB3‐PD) is associated with faster cirrhosis decompensation

A Martini, C Turato, S Cannito, S Quarta… - Alimentary …, 2024 - Wiley Online Library
Background SerpinB3 is a cysteine protease inhibitor involved in liver disease progression
due to its proinflammatory and profibrogenic properties. The polymorphic variant SerpinB3 …

Pediatric and Adult Liver Disease in Alpha-1 Antitrypsin Deficiency

M Ruiz, F Lacaille, C Schrader, M Pons… - Seminars in Liver …, 2023 - thieme-connect.com
Alpha-1 antitrypsin deficiency (AATD) arises due to inherited variants in SERPINA1, the AAT
gene that impairs the production or secretion of this hepatocellular protein and leads to a …

Cleaning up alpha-1 antitrypsin deficiency related Liver Disease

L Rademacher, M Fromme… - Current Opinion in …, 2023 - journals.lww.com
Cleaning up alpha-1 antitrypsin deficiency related liver dis... : Current Opinion in
Gastroenterology Cleaning up alpha-1 antitrypsin deficiency related liver disease : Current …

Longitudinal Evaluation of Individuals With Severe Alpha-1 Antitrypsin Deficiency (Pi∗ ZZ Genotype)

M Fromme, A Payancé, M Mandorfer, KH Thorhauge… - Gastroenterology, 2024 - Elsevier
Background and Aims Homozygous Pi∗ Z mutation in alpha-1 antitrypsin (Pi∗ ZZ genotype)
predisposes to pulmonary loss-of-function and hepatic gain-of-function injury. To facilitate …

Fibrosis, biomarkers and liver biopsy in AAT deficiency and relation to liver Z protein polymer accumulation

A Suri, Z Zhang, B Neuschwander‐Tetri… - Liver …, 2024 - Wiley Online Library
Abstract Background and Aims The course of adults with ZZ alpha‐1‐antitrypsin deficiency
(AATD) liver disease is unpredictable. The utility of markers, including liver biopsy, is …

PNPLA3 risk allele is associated with risk of hepatocellular carcinoma but not decompensation in compensated cirrhosis

E Urias, NR Tedesco, DA Burkholder… - Hepatology …, 2024 - journals.lww.com
Background: The PNPLA3-rs738409-G, TM6SF2-rs58542926-T, and HSD17B13-rs6834314-
A polymorphisms have been associated with cirrhosis, hepatic decompensation, and HCC …

New developments in biomarkers and clinical drug development in alpha‐1 antitrypsin deficiency‐related liver disease

R Loomba, G Clark, J Teckman… - Alimentary …, 2024 - Wiley Online Library
Background Alpha‐1 antitrypsin liver disease (AATLD) occurs in a subset of patients with
alpha‐1 antitrypsin deficiency. Risk factors for disease progression and specific …