1: Arch Dis Child 1994 Oct;71(4):349-52

Liver transplantation for hepatic cirrhosis in cystic fibrosis

Noble-Jamieson G, Valente J, Barnes ND, Friend PJ, Jamieson NV, Rasmussen A,
Calne RY
Department of Paediatrics and Surgery, Addenbrooke's NHS Trust, Cambridge.

Abstract
Five children with cystic fibrosis complicated by hepatic cirrhosis received
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 aspergillus. Liver transplantation
was well tolerated and there was no increase in respiratory or other early
postoperative complications. Four of the children were fully well from 14 to 35
months after transplantation; the most recently transplanted had problems from a
biliary stricture. In spite of the need for immunosuppression there was no
increase in infection and respiratory function improved or remained stable. Once
the children were stabilised after transplantation their nutrition and general
health were greatly improved