LIVER-TRANSPLANTATION FOR HEPATIC CIRRHOSIS IN CYSTIC-FIBROSIS

Five children with cystic fibrosis complicated by hepatic cirrhosis re ceived liver grafts. They all had portal hypertension with varices and three had variceal bleeding; respiratory function was only moderately impaired, but four were colonised with pseudomonas and one with asper gillus. Liver transplantation was well tolerated and there was no incr ease in respiratory or other early postoperative complications. Four o f the children were fully well from 14 to 35 months after transplantat ion; the most recently transplanted had problems from a biliary strict ure. In spite of the need for immunosuppression there was no increase in infection and respiratory function improved or remained stable. Onc e the children were stabilised after transplantation their nutrition a nd general health were greatly improved.