Massive ascites and the heterozygous alpha 1 antitrypsin (α₁AT) living related donor liver in the homozygous child

Abstract

The following is a short report on the use of a heterozygous (PiMZ) alpha 1 antitrypsin (α1AT) living related donor liver in a homozygous (PiZ) child that was complicated by massive ascites early after transplant. This clinical report is then followed by a brief summary of present knowledge on the α 1 AT protein and management of massive ascites in the pediatric liver transplant recipient.

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

“…8,10 In this work, we add a unique pediatric experience with living-related LT for A1ATD from a heterozygous donor with excellent post-LT outcomes and no evidence of recurrence of the disease. In comparison to previously published reports demonstrating the use of an A1AT heterozygous donor for living donor liver transplantation into a child affected with A1ATD, 13 our case demonstrates a sustained excellent outcome in the recipient several years posttransplant.…”

Living Related Liver Transplantation for Metabolic Liver Diseases in Children

“…MZ heterozygotes appear to be at higher risk of liver disease than the general population, and hence there have been concerns about their use as LrLT donors. Published cases where this has been done have been reassuring in terms of both donor safety and recipient efficacy, and thus it is certainly not a contraindication to donation (88)(89)(90). However, long-term surveillance of both donor and recipient appears sensible in order to understand how the heterozygous state affects long-term outcomes.…”