Liver transplantation in children: long-term outcome and quality of life

Liver transplantation has become a standard therapy in acute and chronic li ver failure. Since 1968, 2554 paediatric patients receiving a liver transpl ant have been registered in the European Liver Transplant Registry (ELTR). Compared with 22,600 total transplants registered in the ELTR over the same period of time this means that about 10% of all liver transplants performe d in Europe concern paediatric recipients, aged from 0 to 15 years. The ind ications in the paediatric population differ significantly from those of ad ult patients: More than 50% of patients suffer from cholestatic disorders, followed by hepatic based metabolic disorders, acute liver failure, non-cho lestatic cirrhosis and liver tumours. The results of liver transplantation in paediatric patients have improved r emarkably since the early 1980s. In 1997 a survival rate of 80% is almost t he international standard. This improvement is due to the use of better imm unosuppressive agents such as cyclosporin A and tacrolimus, followed by imp rovement in surgical techniques and finally by improvement in intensive car e, better diagnostic tools for viral, bacterial and fungal infections and c orresponding appropriate therapies. Quality of life as a measure of transplant results has not been sufficientl y studied. The majority of paediatric liver transplant recipients has a goo d quality of life; only 10% suffer from significant morbidity. The impact o f pretransplant damage to other organs such as brain, kidneys, bone and lun gs and the influence of immunosuppression on somatic growth, neurological d evelopment, infection and metabolic balance are subjects of increasing conc ern. Conclusion The results available today show convincing evidence that liver transplantation is a therapeutic option in otherwise fatal hepatic disorder s, Much effort, however, has to be made in order to achieve further improve ments by increasing our knowledge of the pathophysiology of both pre- and p osttransplant conditions.