Intractable Rare Dis Res. 2012;1(1):13-17. (DOI: 10.5582/irdr.2012.v1.1.13)

Primary sclerosing cholangitis as an intractable disease.

Tamura S, Sugawara Y, Kokudo N


Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown origin which eventually results in liver cirrhosis. The disease is reported to be more common among the Western population than in the Asian population. Asian experience remains limited. Diagnosis and treatment standards in the Far East have largely followed those in the West, including liver transplantation. Unlike in the West, however, recent reports from Japan have presented with a higher recurrence rate of PSC after liver transplantation, suggesting the intractable nature of the disease even after the replacement of the entire affected organ.

KEYWORDS: Primary sclerosing cholangitis (PSC), living donor liver transplantation (LDLT), deceased donor liver transplantation (DDLT), Japan

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