Author/Authors :
Pashaei, MR Dr. Daryanis Clinic of Gastroenterology and Hepatology, ايران , Ajdarkosh, H iran university of medical sciences - Firouzgar Hospital - Gastrointestinal and Liver Disease Research Center, تهران, ايران , Daryani, NE tehran university of medical sciences tums - Imam Khomeini Hospital - Gastroenterology Division, تهران, ايران , Habibollahi, P tehran university of medical sciences tums - Imam Khomeini Hospital - Gastroenterology Division, تهران, ايران , Beigmohammadi, MT tehran university of medical sciences tums - Imam Khomeini Hospital - Anesthesiology and Intensive Care Unit, تهران, ايران , Pashaei, Mohammad Reza Dr. Daryanis Clinic of Gastroenterology and Hepatology, ايران
Abstract :
Wilson s disease, caused by a deficient cellular copper export system, is transmitted as an autosomal recessive inherited disorder and results in copper accumulation in liver and other organs, particularly in brain. Acute hepatic failure and severe Coombs negative hemolysis may occur in the course of the disease which has a poor prognosis and most patients do not survive the crisis. Only liver transplantation has been recommended as an effective medical intervention. Herein, we presented a 25-year-old woman with impaired consciousness, acute hepatic failure and hemolysis who was treated with plasmapheresis and albumin replacement. Beside improvement in medical condition, serum copper and hemolysis decreased significantly and renal function was preserved. We concluded that plasmapheresis may be a life saving intervention during fulminant hepatic failure of Wilson s disease.
Keywords :
Wilson s disease , Plasmapheresis , Acute Hepatic Failure , Hemolysis
