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Liver transplantation in childhood has evolved quickly and is now a highly successful treatment for end-stage liver disease. One-year survival is greater than 90% in experienced centres, and the vast majority of these children will survive into adult life.1 World wide, many thousands of adults have received a liver transplant in childhood. These survivors require active monitoring, which will involve collaboration between transplant centres and local services. While the specifics of the monitoring process will differ between programmes, there are some general principles. Monitoring can be considered in the following general areas: liver function, complications and consequences of immunosuppression and lifestyle and quality issues.
The risk of acute rejection is time related and in most patients immunosuppression can be reduced by 1-year after transplantation but not discontinued. Some patients could achieve tolerance, but currently there is no way to identify this group without withdrawing immune suppression and risking severe rejection. Most children are maintained on long-term calcineurin inhibitor treatment, usually in combination with low-dose (0.1 mg/g/day) corticosteroids. More recently, there has been a trend towards adding extra drugs such as azathioprine or mycophenolate to facilitate reduced levels of calcineurin inhibitors.
Either ciclosporin or tacrolimus is used. Both these drugs inhibit calcineurin, which is a component in the activation cycle of T lymphocytes. Whichever drug is used, it is usually taken orally twice daily, although a once daily preparation of tacrolimus is available. Dosing is usually based on 12 h trough blood levels but ciclosporin can also be based on 2 h peak levels. In long-term maintenance treatment, typical target trough levels are 50–90 μg/l for ciclosporin and 2–4 μg/l for tacrolimus. The pharmacokinetics of both drugs are highly variable. As a result, any change of formulation to generic substitutes should be avoided where possible. If this cannot be avoided, …
Competing interests None.
Provenance and peer review Commissioned; externally peer reviewed.