Liver transplantation for alpha 1 antitrypsin deficiency (A1ATD) using a heterozygous donor: Outcomes and review of the literature

• Published in: Pediatric transplantation Vol. 27; no. 4; pp. e14488 - n/a
• Main Authors: Sood, Vikrant, Lee, Eliza J., Raghu, Vikram, Reyes‐Mugica, Miguel, Salgado, Claudia M., Squires, James, Mazariegos, George
• Format: Journal Article
• Language: English
• Published: Denmark Wiley Subscription Services, Inc 01.06.2023
• Subjects: alpha 1-Antitrypsin - analysis; alpha 1-Antitrypsin - genetics; alpha 1-Antitrypsin Deficiency - genetics; alpha 1-Antitrypsin Deficiency - surgery; alpha‐1 antitrypsin deficiency; Child; Cirrhosis; Female; Heterozygote; Heterozygotes; Humans; Literature reviews; Liver - chemistry; Liver - pathology; liver transplant; Liver Transplantation; Liver transplants; Living Donors; Male; Metabolic disorders; outcomes; pediatric; Pediatrics; Tissue and Organ Procurement; alpha-1 antitrypsin deficiency; outcomes; liver transplant; pediatric
• ISSN: 1397-3142; 1399-3046
• DOI: 10.1111/petr.14488

Introduction Alpha 1 antitrypsin deficiency (A1ATD) accounts for 21% of all pediatric liver transplants due to metabolic disease in the western world. Donor heterozygosity has been evaluated in adults but not to a recipient with A1ATD. Methods The data of patient were retrospectively analyzed and a literature review performed. Results We present a unique case of living related donation from a A1ATD heterozygote female to a child for decompensated cirrhosis due to A1ATD. In the immediate postoperative period, the child had low‐alpha 1 antitrypsin levels, but these normalized by 3 months posttransplant. He is currently 19 months post‐transplant with no evidence of recurrent disease. Conclusion Our case provides initial evidence that A1ATD heterozygote donors may be safely used for pediatric patients with A1ATD, thus expanding the donor pool.