The clinical introduction of liver transplantation in the sixties and
seventies has improved the survival rates of patients suffering from t
erminal liver insufficiency, who otherwise would have died. Later on,
the development of selective and effective immunosuppressive drugs sin
ce the early eighties has significantly improved the prognosis for pat
ients after liver transplantation. Between 1978 to 1994 178 orthotopic
liver transplantations were performed in 144 children and adolescents
aged between six weeks and 23 years at the Medizinische Hochschule Ha
nnover. The underlying diseases, which led to terminal liver disease,
were biliary cirrhosis (n = 68), metabolic diseases (n = 40), liver ci
rrhosis of other origin (n = 15), primary liver tumors (n = 6) and ful
minant hepatic failure (n = 15). The development of reduced-size liver
transplantation technique enabled us to operate infants and children
below the age of 5 years with increasing frequency (94 transplantation
s). The survival rate after one and five years was in biliary cirrhosi
s 67,3/56%, in metabolic diseases 87,4/80,4%, in cirrhosis of various
origin 80/66,7%, in fulminant hepatic failure 66,7/56% and in liver tu
mors 66,7/50%. Eightythree percent of children, who survived at least
six months after transplantation, had normal laboratory parameters and
showed complete clinical rehabilitation, in 17% there were pathologic
al laboratory parameters without severe clinical impairment. Liver tra
nsplantation has developed to be a well established treatment of end-s
tage liver disease in childhood, the prognosis of these diseases could
be improved dramatically.