Abstract α1-Antitrypsin deficiency (A1ATD) is an inherited disorder caused by mutations in
SERPINA1, leading to liver and lung disease. It is not a rare disorder but frequently goes …

Each Association appointed at least one author to serve on the writing group. The Chair of
the writing group was appointed by the AASLD. Members of the writing group were not …

Living donor liver transplantation (LDLT) has been increasingly embraced around the world
as an important strategy to address the shortage of deceased donor livers. The aim of this …

BACKGROUND & AIMS: We sought to estimate the risk of perioperative mortality or acute
liver failure for live liver donors in the United States and avoid selection or ascertainment …

The clinical presentation of liver disease is highly variable in homozygous ZZ alpha-1
antitrypsin (AAT) deficiency, and not all patients with the homozygous ZZ genotype develop …

Objectives: To evaluate mortality in adolescents and young adult patients with chronic
diseases followed in a Latin American tertiary hospital. Methods: A cross-sectional …

Orthotopic liver transplantation (OLT) in the care of children with inborn errors of metabolism
(IEM) is well established and represent the second most common indication for pediatric …

Maple syrup urine disease (MSUD) is caused by a deficiency in the activity of the branched‐
chain α‐ketoacid dehydrogenase (BCKD) complex, promoting the accumulation of the …

Thrombosis is a major postoperative complication in pediatric liver transplantation. There is
marked heterogeneity in prophylactic antithrombotic therapies used, without established …

Alpha-1 antitrypsin deficiency is predominantly caused by point mutations that alter the
protein's folding. These mutations fall into two broad categories: those that destabilize the …