{"id":12628,"date":"2024-10-11T18:21:47","date_gmt":"2024-10-11T16:21:47","guid":{"rendered":"https:\/\/valintermed.com\/?p=12628"},"modified":"2024-10-11T18:21:47","modified_gmt":"2024-10-11T16:21:47","slug":"progressiruyuschiy-semeynyy-vnutripechenochnyy-holestaz-tip-3","status":"publish","type":"post","link":"https:\/\/valintermed.com\/en\/medlibrary\/progressive-family-intraepic-hole-pelvis-tip-3\/","title":{"rendered":"Progressive familial intrahepatic cholestasis, type 3"},"content":{"rendered":"
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Progressive familial intrahepatic cholestasis (PFIC), type 3, is a hereditary disease characterized by impaired bile excretion from the liver, which leads to retention of bile acids in the hepatocyte and, as a result, to liver tissue damage. This disease is autoimmune in nature and most often occurs in children, but can also manifest itself in adulthood. The main mechanism of pathogenesis is the insufficiency of a specific transport mechanism that is responsible for the transport of bile acids across liver cell membranes. This can lead to various clinical manifestations, including pruritus, jaundice, cholestasis and, ultimately, to liver cirrhosis and liver failure.<\/p>\n

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