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

TY - JOUR

T1 - Liver transplantation in children: long-term outcome and quality of life.

AU - Burdelski, M

AU - Nolkemper, D

AU - Ganschow, Rainer

AU - Sturm, E

AU - Malago, M

AU - Rogiers, X

AU - Brölsch, C E

PY - 1999

Y1 - 1999

N2 - Liver transplantation has become a standard therapy in acute and chronic liver failure. Since 1968, 2554 paediatric patients receiving a liver transplant 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 performed in Europe concern paediatric recipients, aged from 0 to 15 years. The indications in the paediatric population differ significantly from those of adult patients: More than 50% of patients suffer from cholestatic disorders, followed by hepatic based metabolic disorders, acute liver failure, non-cholestatic cirrhosis and liver tumours. The results of liver transplantation in paediatric patients have improved remarkably since the early 1980s. In 1997 a survival rate of 80% is almost the international standard. This improvement is due to the use of better immunosuppressive agents such as cyclosporin A and tacrolimus, followed by improvement in surgical techniques and finally by improvement in intensive care, better diagnostic tools for viral, bacterial and fungal infections and corresponding appropriate therapies.Quality of life as a measure of transplant results has not been sufficiently studied. The majority of paediatric liver transplant recipients has a good quality of life; only 10% suffer from significant morbidity. The impact of pretransplant damage to other organs such as brain, kidneys, bone and lungs and the influence of immunosuppression on somatic growth, neurological development, infection and metabolic balance are subjects of increasing concern. CONCLUSION: The results available today show convincing evidence that liver transplantation is a therapeutic option in otherwise fatal hepatic disorders. Much effort, however, has to be made in order to achieve further improvements by increasing our knowledge of the pathophysiology of both pre- and posttransplant conditions.

AB - Liver transplantation has become a standard therapy in acute and chronic liver failure. Since 1968, 2554 paediatric patients receiving a liver transplant 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 performed in Europe concern paediatric recipients, aged from 0 to 15 years. The indications in the paediatric population differ significantly from those of adult patients: More than 50% of patients suffer from cholestatic disorders, followed by hepatic based metabolic disorders, acute liver failure, non-cholestatic cirrhosis and liver tumours. The results of liver transplantation in paediatric patients have improved remarkably since the early 1980s. In 1997 a survival rate of 80% is almost the international standard. This improvement is due to the use of better immunosuppressive agents such as cyclosporin A and tacrolimus, followed by improvement in surgical techniques and finally by improvement in intensive care, better diagnostic tools for viral, bacterial and fungal infections and corresponding appropriate therapies.Quality of life as a measure of transplant results has not been sufficiently studied. The majority of paediatric liver transplant recipients has a good quality of life; only 10% suffer from significant morbidity. The impact of pretransplant damage to other organs such as brain, kidneys, bone and lungs and the influence of immunosuppression on somatic growth, neurological development, infection and metabolic balance are subjects of increasing concern. CONCLUSION: The results available today show convincing evidence that liver transplantation is a therapeutic option in otherwise fatal hepatic disorders. Much effort, however, has to be made in order to achieve further improvements by increasing our knowledge of the pathophysiology of both pre- and posttransplant conditions.

M3 - SCORING: Zeitschriftenaufsatz

VL - 158

SP - 34

EP - 42

JO - EUR J PEDIATR

JF - EUR J PEDIATR

SN - 0340-6199

IS - 2

M1 - 2

ER -