[Liver transplantation in a patient with hemolytic syndrome in the course of fulminant Wilson's disease]. / Transplantacja watroby u chorej z zespolem hemolitycznym w przebiegu piorunujacej postaci choroby Wilsona.

The case report of a 20 year old woman with fulminant liver failure and hemolytic syndrome was described. Huge amounts of copper excreted in the urine (3555 mg/12 h without, and 5180 mg/12 h after d-penicillamine provocation, respectively) confirmed the diagnosis of fulminant Wilson's disease. Because the patient's general condition worsened rapidly (hemolysis, diathesis hemorrhagic, ascites, encephalopathy increased during 3 days of clinical observation) orthotopic liver transplantation was performed. After the transplantation, ischemic type biliary lesion (ITBL) II stage was diagnosed. The woman is still being treated with Prograf and Urso-Falk. The patient returned to her normal life, continues to work and was married. Two years after OLT she gave birth to a healthy boy. The liver function tests are normal with the exception of GGTP and FALK activities elevation. Copper and ceruloplasmin level, as well as copper excretion in the urine are within the normal values.