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[Liver transplantation in an adolescent with cystic fibrosis]
P Thevenot1, F Gottrand, E Tassin
1Service de pédiatrie, CHRU, hôpital Claude-Huriez, Lille, France.
Background:
Orthotopic liver transplantation (OLT) is an effective treatment for patients with cystic fibrosis end stage liver disease, especially those with only mild pulmonary involvement. Long-term follow-up in such transplanted patients is still lacking.
Case Report:
A 15-year-old girl with cystic fibrosis received an OLT because of severe decompensated cirrhosis. She had been colonized by Pseudomonas aeruginosa for 3 years and had pancreatic insufficiency; she also had mild glucose intolerance. Postoperatively she developed diabetes mellitus requiring insulin therapy for 9 months. Oral cyclosporin was poorly absorbed so that she was given a new emulsion of cyclosporin (Neoral) that was better absorbed. A rapid pubertal catch-up was obtained but the patient remained colonized by Pseudomonas aeruginosa.
Conclusion:
This 3-year postoperative follow-up confirms that OLT can represent a good alternative in those patients with severe liver disease and mild pulmonary involvement.