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/PMC11717895 | PMC |
http://dx.doi.org/10.1002/jmd2.12467 | DOI Listing |
Liver Transpl
January 2025
Section of Gastroenterology and Hepatology, Department of Medicine, Baylor College of Medicine, Houston, Texas, USA.
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.
Haemophilia
January 2025
Department of Hepatobiliary Surgery & Liver Transplantation, Dr. Rela Institute & Medical Centre, Bharath Institute of Higher Education and Research, Chennai, India.
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 PDFJ 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.
Transpl Int
January 2025
Department of Microbiology, Immunology and Transplantation, KU Leuven, Leuven, Belgium.
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 PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!