Aim of the study is to evaluate the role of liver transplantation in m
odifying CF liver disease patient outcome. Since April 1996 four patie
nts affected by hepatic cirrhosis and severe portal hypertension under
went liver transplantation. Post-transplant immunosuppression was base
d on Neoral with steroids withdrawn in 3 months period. We have never
observed mortality of major complications related to surgery. Liver tr
ansplantation, in our experience provided in all cases a progressive i
mprovement of patient respiratory function, nutritional status and qua
lity of life.