RESUMO
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.
Assuntos
Fibrose Cística/terapia , Transplante de Fígado , Adolescente , Constituição Corporal , Fibrose Cística/complicações , Feminino , Humanos , Cirrose Hepática/complicações , Cirrose Hepática/cirurgia , Período Pós-OperatórioRESUMO
We report the case of a 12 year-old boy with Wilson's disease, presenting with decompensated liver cirrhosis. Medical treatment failed to prevent fulminant liver failure in less than one month. Emergency liver transplantation was successfully performed. This report led us to review the prognosis of Wilson's disease with liver failure according to the present results of liver transplantation.