Related Experiment Video
Updated: Oct 7, 2026
Heterotopic Auxiliary Whole Liver Rat Transplant Model Utilizing a Hepaticoureterostomy for Allograft Rejection Studies
Published on: March 8, 2024
A pediatric patient with classical citrullinemia who underwent living-related partial liver transplantation
K Ban1, N Sugiyama, K Sugiyama
1Department of Pediatrics, Nagoya City University Medical School, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya 467-8601, Japan. kban@med.nagoya-cu.ac.jp
Insights
Living-related liver transplantation using a carrier mother
Area of Science:
- Hepatology
- Metabolic Disorders
- Transplantation Immunology
Background:
- Inborn errors of metabolism (IEMs) often necessitate liver transplantation.
- The efficacy of using livers from carrier donors for IEM transplantation remains unclear.
Observation:
- A 6-year-old girl with classical citrullinemia experienced recurrent hyperammonemia.
- She received a partial liver transplant from her mother, a known heterozygote carrier for classical citrullinemia.
Findings:
- Post-transplantation, the patient's hyperammonemia significantly improved, enhancing her quality of life.
- Plasma and urinary citrulline levels remained elevated, but plasma citrulline showed no significant difference compared to orthotopic liver transplant cases.
Implications:
- Partial liver grafts from carrier donors can be a viable option for treating certain IEMs.
- This approach may offer a life-improving alternative for patients awaiting suitable donor organs.
- Further research is needed to fully understand long-term outcomes and donor-recipient dynamics.
Abstract:
Patients with inborn errors of metabolism undergo liver transplantation, but the effect of transplanting the liver of healthy carriers of these conditions has not been fully clarified. A 6-year-old girl with classical citrullinemia, who repeatedly suffered from hyperammonemia, underwent living-related liver transplantation by using a segment of the liver of her mother, who was a heterozygote carrier for classical citrullinemia. Hyperammonemia alleviated in the patient after the transplantation, thereby dramatically improving her quality of life. Although the levels of plasma and urinary citrulline remained high postoperatively, there was no marked difference in the level of plasma citrulline up to 1 month after surgery when compared with that of previously reported orthotopic liver transplantation cases with classical citrullinemia.
Related Concept Videos
01:28Kidney Transplant I: Introduction
01:26Kidney Transplant II: Surgical Procedure