原发性硬化性胆管炎
医学
肝移植
胃肠病学
内科学
恶性肿瘤
广谱
发病机制
疾病
移植
组合化学
化学
作者
Raquel T. Yokoda,Elizabeth J. Carey
标识
DOI:10.14309/ajg.0000000000000268
摘要
Cholestatic liver diseases encompass a broad spectrum of pathologies, with the core injury occurring at the level of cholangiocytes and progressing to hepatic fibrosis and liver dysfunction. Primary biliary cholangitis and primary sclerosing cholangitis are the most significant progressive cholangiopathies in adults. Although rare, they commonly evolve to liver failure and need for liver transplantation. Despite recent advances in the basic knowledge of these cholangiopathies, the pathogenesis is still elusive. Targeted treatments to prevent disease progression and to preclude malignancy are not yet available. This review will address the general clinical features of both diseases, analyze their commonalities and differences, and provide a state-of-the art overview of the currently available therapeutics.
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