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Recurrent pyogenic cholangitis (RPC), also known as Hong Kong disease, Oriental cholangitis, and Oriental infestational cholangitis, is a chronic infection characterized by recurrent bouts of bacterial cholangitis with primary hepatolithiasis. [1] [2] It is exclusive to people who live or have lived in southeast Asia. [3] [4] [5]
Reynolds' pentad is a collection of signs and symptoms consistent with obstructive ascending cholangitis, a serious infection of the biliary tract.It is a combination of Charcot's triad (right upper quadrant pain, jaundice, and fever) with shock (low blood pressure, tachycardia) and an altered mental status. [1]
Acute cholangitis carries a significant risk of death, the leading cause being irreversible shock with multiple organ failure (a possible complication of severe infections). [7] Improvements in diagnosis and treatment have led to a reduction in mortality: before 1980, the mortality rate was greater than 50%, but after 1980 it was 10–30%. [7]
Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune disease of the liver. [1] [2] [3] It results from a slow, progressive destruction of the small bile ducts of the liver, causing bile and other toxins to build up in the liver, a condition called cholestasis.
Diagnosis of PSC requires the exclusion of all secondary causes of sclerosing cholangitis; else, if a known aetiology can be uncovered, SSC is diagnosed. Its clinical and cholangiographic features may mimic PSC, yet its natural history may be more favorable if recognition is prompt and appropriate therapy is introduced.
The exact cause of primary sclerosing cholangitis is unknown, and its pathogenesis is improperly understood. [1] Although PSC is thought to be caused by autoimmune disease, it does not demonstrate a clear response to immunosuppressants. Thus, many experts believe it to be a complex, multifactorial (including immune-mediated) disorder and ...
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