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[Liver transplant in children. I]
N Ansaldi-Balocco1, N Cimadamore, C Barbera
1Istituto di Discipline Pediatriche, Università degli Studi di Torino, Italia.
Insights
Pediatric liver transplantation offers improved survival for end-stage liver disease, driven by advancements in immunosuppression like cyclosporine and surgical techniques. Donor organ availability remains a key challenge for pediatric liver transplants.
Area of Science:
- Hepatology
- Pediatric Surgery
- Immunology
Context:
- Liver transplantation is the sole definitive treatment for end-stage pediatric liver diseases.
- Recent survival improvements are attributed to cyclosporine, advanced surgical methods, and novel immunosuppressants.
Purpose:
- To outline the current landscape of pediatric liver transplantation, including indications, contraindications, and limitations.
- To highlight the critical role of immunosuppression and surgical advancements in improving outcomes for children with liver failure.
Summary:
- Key indications include biliary atresia, paucity of intrahepatic bile ducts, metabolic disorders (e.g., alpha-1 antitrypsin deficiency, hereditary tyrosinemia), post-infectious cirrhosis, acute liver failure, and hepatic malignancies.
- Absolute contraindications involve severe systemic illness, cardiac/renal failure, vascular abnormalities, sepsis, and HIV infection. Relative contraindications include malnutrition and certain viral markers.
- Significant limitations include the scarcity of appropriately sized donor livers for pediatric recipients and challenges in post-transplant family care and adherence.
Impact:
- Enhanced understanding of eligibility criteria and challenges in pediatric liver transplantation.
- Provides a foundation for future research into optimizing donor organ utilization and managing contraindications.
- Informs clinical decision-making and resource allocation for pediatric liver failure management.
Abstract:
Hepatic transplantation is the only therapeutic choice for end-stage pediatric liver diseases. The survival improvement, registered in the last few years is mainly due to the employment of cyclosporine in therapy, but also to new and sophisticated surgical techniques and immunosuppressive drugs. The indications in children are: biliary atresia after unsuccessful Kasai procedure, paucity of intrahepatic bile ducts (of syndromic and not syndromic type), some metabolic diseases (alfa1 antitrypsine deficiency, hereditary tyrosinemia), post infective cirrhosis, acute fulminant hepatic failure, hepatic malignancies. Absolute contraindications include severe systemic illness, severe cardiac or kidney failure, thrombosis or abnormalities of caval and portal veins, systemic sepsis, HIV infection. Other drawbacks are mental deficiency and the inability of family to care for the child and follow therapy after discharge. Relative contraindications are: HBsAg positivity, HIV positivity without infection, malnutrition. Finally the scarcity of donors of liver of adequate size is an important limitation for transplant especially in childhood.