D. Alfani et al., LIVER-TRANSPLANTATION IN CYSTIC-FIBROSIS - MONITORING AND FOLLOW-UP -PERSONAL-EXPERIENCE, Rivista italiana di pediatria, 24(2), 1998, pp. 32-33
About 5-15% of children with cystic fibrosis develop hepatic cirrhosis
and progressive portal hypertension. When the portal hypertension wor
sens these children are likely to develop serious variceal bleeding an
d other complications including malnutrition and a decline in respirat
ory function. Isolated liver transplantation is the therapy of choice
in patients with cystic fibrosis affected by liver cirrhosis and initi
al clinical manifestations of portal hypertension in presence of a wel
l preserved pulmonary function (REV1 > 40%). Although the optimal time
for liver transplantation is difficult to judge this procedure provid
es a progressive improvement of patient respiratory function, nutritio
nal status and quality of life, meanwhile not increasing the risk of l
ung infection due to immunosuppression.