Liver transplantation for hepatic cirrhosis in cystic fibrosis

Arch Dis Child. 1994 Oct;71(4):349-52. doi: 10.1136/adc.71.4.349.


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.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / physiopathology
  • Female
  • Forced Expiratory Volume
  • Humans
  • Liver Cirrhosis / etiology
  • Liver Cirrhosis / surgery*
  • Liver Transplantation*
  • Male
  • Nutritional Status
  • Postoperative Period
  • Treatment Outcome
  • Vital Capacity