LIVER-TRANSPLANTATION IN A CYSTIC-FIBROSI S ADOLESCENT

Background. - Orthotopic liver transplantation (OLT) is an effective t reatment for patients with cystic fibrosis end stage liver disease, es pecially those with only mild pulmonary involvement. Long-term follow- rep in such transplanted patients is still lacking. Case report. - A 1 5-year-old girl with cystic fibrosis received an OLT because of severe decompensated cirrhosis. She had been colonized by Pseudomonas aerugi nosa for 3 years and had pancreatic insufficiency; she also had mild g lucose intolerance. Postoperatively she developed diabetes mellitus re quiring insulin therapy for 9 months. Oral ciclosporin was poorly abso rbed so that she was given a new emulsion of ciclosporin (Neoral(R)) t hat was better absorbed. A rapid pubertal catch-up was obtained but th e patient remained colonized by Pseudomonas aeruginosa Conclusion. - T his 3-year post-operative follow-up confirms that OLT can represent a good alternative in those patients with severe liver disease and mild pulmonary involvement.