Introduction

Acute and chronic end-stage liver disease may be treated successfully by liver transplantation. With organ and patient survival now approaching 80–90% [1], these results represent a considerable improvement compared with the early days of transplantation. As a natural consequence, increasing numbers of disorders have been accepted for transplantation. In order to achieve optimal patient survival and efficient use of limited organ resources, objective criteria are needed for transplant candidate selection. Thus, accurate estimates of prognosis became an essential element in hepatology.

Initially, prognosis was derived from the natural course of a given disorder. In paediatric liver disorders, for instance, there is a rapid progressive course in cholestatic syndromes. The time between diagnosis and death from chronic end-stage liver cirrhosis ranges from months in extrahepatic biliary atresia to a few years in progressive familial intrahepatic cholestasis [2, 3]. This rapid progressive course is not observed in adult liver disorders such as primary biliary cirrhosis (PBC), sclerosing cholangitis (PSC) or hepatitis B and C [4–6] where liver cirrhosis is observed between 10 and 20 years after the initial symptoms.

The option to perform liver transplantation in these disorders changed requirements for the quality of prognostic information. Clinical scores such as the Child–Pugh score in adults and the Malatack score in paediatric patients were developed in order to identify those patients most at risk of dying after being placed on the transplant waiting list [7, 8].