Biomarkers Associated with Future Severe Liver Disease in Children with Alpha-1-antitrypsin Deficiency

Children with Alpha-1-antitrypsin deficiency (AATD) exhibit a wide range of liver disease outcomes from portal hypertension and transplant, to asymptomatic without fibrosis. Individual outcomes cannot be predicted. High circulating Z polymer levels and high GGT early in life are associated with future CEPH in AATD, and use of predictive cutoffs may assist in future clinical trial design.