Primary sclerosing cholangitis (PSC) is a chronic, cholestatic liver disease characterised by fibrosing inflammation of the extra and intahepatic bile ducts. Although PSC is not always associated with progrssion leading to death, many patients
have
an
unrelenting downhill course progressing to cirhosis and liver failure. Because no therapy had been shown to alter the natural history of this disease, liver transplatation may had been a therapeutic option for patients with end-stage PSC. It has
become
the third most common indication of orthotopic liver trans plantation in adult patients in USA.
In Korea, on February 26, 1994, a forty five year-old male with PSC was treated with liver transplantation at Seoul National University Hospital. He was diagnosed as PSC at outside hospital by needle biopsy of the liver. He visited our hospital
for
persistent jaundice and underwent cholecystectomy with T-tube choledocholithotomy, 3 years ago. At that time, PSC and secondary biliary cirrhosis was confirmed by wedge biopsy of the liver and ERCP. Thereafter, he experienced recurrent variceal
bleeding
and cholangitic symptoms. On February 25, he was arrived at our ER with massive hemtemesis, melena and altered consciousness. On February 26, 1994 the orthotopic liver transplantation was performed. His diseased liver was removed and replaced
with
the
cadaveric donor liver from a brain dead patient. Postoperatively, he was managed using standard protocols. Immunosuppression was based on cyclosporine, storoid and azathioprine. No technical complication was occurred. He was developed some
complications
in the postoperative period, such as, acute rejection, autoimmune hemolytic anemia, pulmonary edema and pneumonia. He was discharged as normal liver function state and followed at our OPD.
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