Acute liver failure.

Acute liver failure in children is a rare but potentially fatal disease. Causes of ALF in neonatal period are different from those in early or late childhood. Despite the improvement in the paediatric intensive care, liver transplantation remains the only effective treatment. Use of newer treatment modalities (liver assist devices and hepatocyte transplantation) is still in experimental phase. Management requires early recognition, prompt diagnosis of treatable condition, supportive therapy and prevention of complications hence these children should ideally be treated in a specialist unit.

Liver transplantation in children.

The results of liver transplantation has improved significantly in the last decade with one year survival figures close to 90% for children with chronic liver disease. This can be attributed to improvement in surgical techniques, better postoperative care and newer immunosuppresive drugs. As a result of this, increasing number of children are referred for transplantation with no significant increase in the number of solid organ donors. The earliest transplants in children were performed using organs from size matched pediatric donors. However, as the pediatric donor numbers were limited, liver reduction techniques were developed to transplant small children before deterioration. Increasing experience with reduced livers led to the development of split liver, living donor and auxiliary liver transplantation. Better management of immunosuppressive drugs and newer agents such as Mycophenolate Mofetil have reduced the incidence of graft loss due to chronic rejection and long-term renal toxicity. The goal for the future will remain to be transplantation without the use of long-term immunosuppression.

Acute liver failure.

Acute liver failure (ALF) is a rare condition in the pediatric population. Patients who present with severe failure of liver synthetic function have a high mortality with medical therapy alone. The main causes of death are cerebral edema, hemorrhage, renal failure and sepsis. The etiology of ALF is age specific, with a significant number due to inborn errors of metabolism especially in neonates and infants. Treatment of children with ALF is supportive, aimed at preventing and managing associated complications until the native liver recovers or liver transplantation. Sedation should not be administered unless a decision for artificial ventilation has been made. As all children are potential transplant candidates, transfer to and management in a liver transplant centre is recommended. Prognostic criteria for mortality are less well defined compared to the adult population, although a significantly elevated INR > or = 4 carries a high chance of death, and liver transplantation should be considered at this stage. Auxiliary transplantation is an attractive option in selected individuals and provides the chance to stop immunosuppression should sufficient hepatic regeneration occur. The use of various liver assist devices and hepatocyte transplantation as a bridge to liver transplantation show promise, although when used in isolation, they do not have an impact on overall patient survival.