关键词: Dominant stricture balloon dilation biliary tract disease cholangioscopy endoscopy therapy liver transplantation

Mesh : Animals Bile Duct Neoplasms / etiology surgery Cholangiocarcinoma / etiology surgery Cholangiopancreatography, Endoscopic Retrograde / adverse effects instrumentation methods Cholangiopancreatography, Magnetic Resonance Cholangitis, Sclerosing / complications diagnostic imaging surgery Cholestasis / diagnostic imaging etiology surgery Humans Palliative Care Predictive Value of Tests Stents Treatment Outcome

来  源:   DOI:10.1080/17474124.2018.1483719

Abstract:
BACKGROUND: Primary sclerosing cholangitis (PSC) is a rare but clinically important cholestatic liver disease. Histopathologically and cholangiographically, PSC is characterized by intra- and/or extra-hepatic bile duct inflammation and fibro-obliteration, which ultimately leads to biliary cirrhosis and related sequelae, including development of hepatobiliary and colorectal carcinomata. PSC can be diagnosed at essentially any age and carries a median survival of 15-20 years, regardless of age at diagnosis, and is a foremost risk factor for cholangiocarcinoma. Given the chronic and progressive nature of PSC, its inherent association with both neoplastic and non-neoplastic biliary tract complications, and the lack of effective pharmacotherapies, alimentary and biliary tract endoscopy plays a major role in the care of patients with PSC. Areas covered: Here, we provide a narrative review on endoscopic management of PSC, including established and evolving applications to the diagnosis and treatment of both its benign and malignant complications. Expert commentary: Due to the rarity of PSC and the considerable patient-years required to rigorously study major endpoints, there remains a paucity of high-quality evidence regarding its management. As the advanced endoscopic repertoire expands, so has the interest in developing best practices in PSC, which we discuss herein.
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