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Standard Clinical Dosage 200500 mcg daily, delivered as a subcutaneous injection in the abdominal region

Disclosures: Barbara Coco: Nothing to Disclose, Lamia Ait Al: Nothing to Disclose, Lorenzo De Stefano: Nothing to Disclose, Giovanni Petralli: Nothing to Disclose, Ferruccio Bonino: Nothing to Disclose, Pierluigi Festa: Nothing to Disclose, Maurizia Brunetto: Nothing to Disclose 586 A FIRST-IN-CLASS TOOL TO PREDICT FUTURE RISK OF PORTAL HYPERTENSION, LIVER TRANSPLANT AND DEATH IN CHILDREN WITH ALPHA-1-ANTITRYPSIN DEFICIENCY BASED ON PROSPECTIVE DATA FROM THE CHILDHOOD LIVER DISEASE RESEARCH NETWORK Jeffrey Teckman 1 Catherine Spino 2 Suiyan Huang 2 Gregor Horvath 2 John Magee 3 Philip Rosenthal 4 , 1 Saint Louis University, 2 University of Michigan Hospital, 3 University of Michigan Hospitals and Health Centers, 4 University of California, San Francisco Background: Children with homozygous ZZ and SZ Alpha-1-antitrypsin deficiency (AATD) exhibit a wide range of liver disease outcomes from asymptomatic without fibrosis to portal hypertension progressing to transplant or death