Many inherited metabolic disorders (IMD) are associated with end-organ damage necessitating organ transplantation. Although utilization of deceased donors with history of IMD warrants caution, there may be circumstances under which such donors could be considered as suitable organ donor candidates. We present the first known report of liver transplantation from a deceased donor with cystinosis. The donor was a 20-year-old male with infantile cystinosis who had previously undergone two deceased donor kidney transplants. Unfortunately, he incurred cranial trauma, and after careful consideration of the metabolic consequences, his liver was deemed suitable for transplantation. The liver was successfully transplanted into a 65-year-old female recipient with hepatitis C (HCV) cirrhosis. The recipient is currently 12 months post-transplant and experiencing good graft function without evidence of cystine crystals on liver biopsy. This case highlights that liver transplantation from donors with rare IMD can result in favorable outcomes. However, it is crucial to approach the use of such livers with caution. These transplants should be considered after a thorough assessment, ensuring that a comprehensive decision-making process is in place to mitigate potential risks.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11717895PMC
http://dx.doi.org/10.1002/jmd2.12467DOI Listing

Publication Analysis

Top Keywords

deceased donor
12
donor cystinosis
8
liver transplantation
8
liver
6
donor
5
liver transplant
4
deceased
4
transplant deceased
4
cystinosis case
4
case report
4

Similar Publications

Background: Machine perfusion (MP), including hypothermic oxygenated machine perfusion (HOPE), dual HOPE, normothermic machine perfusion (NMP), NMP ischemia-free liver transplantation (NMP-ILT), and controlled oxygenated rewarming (COR), is increasingly being investigated to improve liver graft quality from extended criteria donors and donors after circulatory death and expand the donor pool. This network meta-analysis investigates the comparative efficacy and safety of various liver MP strategies versus traditional static cold storage (SCS).

Methods: We searched PubMed, Scopus, Web of Science, and Cochrane Controlled Register of Trials for randomized controlled trials (RCTs) comparing liver transplantation (LT) outcomes between SCS and MP techniques.

View Article and Find Full Text PDF

Impact of Hepatoblastoma on Infectious Complications Following Pediatric Liver Transplantation.

Pediatr Transplant

February 2025

Division of Infectious Diseases, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio, USA.

Background: Liver transplantation is the standard therapy for end-stage liver disease in pediatric patients with biliary atresia (BA), congenital and metabolic conditions, and for an unresectable malignant tumor like hepatoblastoma (HB). BA is the leading indication for pediatric liver transplantation, while HB is the most common childhood liver cancer. Despite improved outcomes through advanced surgical techniques and novel immunosuppression, pediatric liver transplantation (pLT) is complicated by post-transplant infections.

View Article and Find Full Text PDF

The Effects of Nitric Oxide on Choroidal Gene Expression.

J Bioinform Syst Biol

January 2024

Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, 73104, United States.

Purpose: Nitric oxide (NO) is recognized as an important biological mediator that controls several physiological functions, and evidence is now emerging that this molecule may play a significant role in the postnatal control of ocular growth and myopia development. We therefore sought to understand the role that nitric oxide plays in visually-guided ocular growth in order to gain insight into the underlying mechanisms of this process.

Methods: Choroids were incubated in organ culture in the presence of the NO donor, PAPA- NONOate (1.

View Article and Find Full Text PDF

Highly sensitized (HS) patients in need of kidney transplantation (KTx) typically spend a longer time waiting for compatible kidneys, are unlikely to receive an organ offer, and are at increased risk of antibody-mediated rejection (AMR). Desensitization using imlifidase, which is more rapid and removes total body immunoglobulin G (IgG) to a greater extent than other methods, enables transplantation to occur between HLA-incompatible (HLAi) donor-recipient pairs and allows patients to have greater access to KTx. However, when the project was launched there was limited data and clinical experience with desensitization in general and with imlifidase specifically.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!