Purpose Of Review: Advances in surgical techniques and combinations of conventional immunosuppressants have made paediatric liver transplantation the success story it is today. However, the increasing numbers of survivors reaching adulthood highlight important issues of long-term quality of life and drug induced complications. The aim of this review is to describe the trends and advances in immunosuppression for paediatric liver transplantation over the last 12 months.
Recent Developments: As our knowledge of the immune cell populations and intracellular mechanisms involved in alloreactivity improves, induction immunosuppression has emerged as a powerful therapeutic manoeuvre to counter the initial alloimmune response. Many centres have adopted a more focused use of biological agents at induction to improve immunosuppression in the critical peritransplant period and to reduce the level of subsequent maintenance requirements. Corticosteroid avoidance and calcineurin inhibitors minimization trials have obtained encouraging results. New immunosuppressive strategies have evolved towards the goal of inducing operational tolerance, and paediatric liver transplant recipients seem to be a particularly promising target. New strategies are being developed also to improve quality of life and reduce nonadherence in adolescents and young adults who underwent liver transplantation. New drugs target B-cell and complement driven rejection and new monoclonal antibodies and small molecules are under trial to inhibit specific signals in the immune response.
Summary: We review current trends of immunosuppressive protocols in paediatric liver transplantation, focusing on induction agents, corticosteroid avoidance and calcineurin inhibitor sparing protocols, protocols for adult transition and new drugs currently under development.
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http://dx.doi.org/10.1097/MOT.0b013e32833d8936 | DOI Listing |
World J Gastroenterol
January 2025
Senior Department of Hematology, The Fifth Medical Center of PLA General Hospital, Beijing 100071, China.
In this article, we comment on an article published in a recent issue of the . We specifically focus on the roles of human leukocyte antigen (HLA) and donor-specific antibodies (DSAs) in pediatric liver transplantation (LT), as well as the relationship between immune rejection after LT and DSA. Currently, LT remains the standard of care for pediatric patients with end-stage liver disease or severe acute liver failure.
View Article and Find Full Text PDFCureus
December 2024
Department of Pediatrics, Military Hospital, Bahrain Defence Force Royal Medical Services, Riffa, BHR.
Trichohepatoenteric syndrome (THES) is a rare genetic disorder inherited in an autosomal recessive manner. THES primarily leads to neonatal enteropathy, typically manifesting as severe, persistent diarrhea, distinctive facial features such as frontal bossing and a broad flat nasal bridge, woolly and fragile hair, immunodeficiency resulting in recurrent infections, failure to thrive (FTT), and liver complications including fibrosis or cirrhosis. This multisystem disorder is linked to mutations in the tetratricopeptide repeat domain 37 (TTC37) gene, also known as superkiller complex (SKIC) protein 3, responsible for THES type 1, and the Ski2-like ribonucleic acid (RNA) helicase (SKIV2L) gene, also known as SKIC2, responsible for THES type 2.
View Article and Find Full Text PDFRadiol Case Rep
March 2025
Department of Radiology, Addis Ababa University, College of Health Sciences, Addis Ababa, Ethiopia.
Niemann-Pick disease (NPD) is an autosomal recessive disease caused by deficient lysosomal enzyme or faulty cholesterol transport. A 9-year-old male patient presented with 6 years of abdominal swelling, previously treated as tuberculosis. He exhibited hepatosplenomegaly, delayed growth, and pancytopenia.
View Article and Find Full Text PDFExp Clin Transplant
December 2024
>From the Jacobs School of Medicine and Biomedical Sciences, Buffalo, New York, USA.
Objectives: The risks of mortality and graft failure in those receiving organ transplants from donors who have drowned are unclear. We compared 5-year mortality and graft survival from those receiving a drowned donor kidney or liver to those receiving organs from alternative causes of nondrowning donor death.
Materials And Methods: In this retrospective analysis, we searched the United Network for Organ Sharing database between January 1, 2010, and June 30, 2022.
Background: Pediatric ultrasound (US)-guided percutaneous liver biopsy is a commonly performed procedure in children, and may be performed in a variety of clinical settings. However, there is little research on the relative costs associated with different sedation methods and locations.
Objective: This study uses time-driven activity-based costing (TDABC) to identify relevant costs associated with different biopsy sedation techniques and locations to help inform providers and patients as well as guide value-conscious care.
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