Primary biliary cirrhosis overview: Difference between revisions
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==Historical Perspective== | ==Historical Perspective== | ||
Addison and Gull in 1851 described the clinical picture of progressive obstructive jaundice in the absence of mechanical obstruction of the large bile ducts. Ahrens ''et al'' in 1950 coined the term primary biliary cirrhosis for this disease. The association with anti mitochondrial antibodies was first reported in 1986.<ref name=Mitchison1986>{{cite journal |author=Mitchison HC, Bassendine MF, Hendrick A, ''et al.'' |title=Positive antimitochondrial antibody but normal alkaline phosphatase: is this primary biliary cirrhosis? |journal=Hepatology |volume=6 |issue=6 |pages=1279–84 |year=1986 |pmid=3793004 |doi=10.1002/hep.1840060609}}</ref> | Addison and Gull in 1851 described the clinical picture of progressive obstructive jaundice in the absence of mechanical obstruction of the large bile ducts. Ahrens ''et al'' in 1950 coined the term primary biliary cirrhosis for this disease. The association with anti mitochondrial antibodies was first reported in 1986.<ref name=Mitchison1986>{{cite journal |author=Mitchison HC, Bassendine MF, Hendrick A, ''et al.'' |title=Positive antimitochondrial antibody but normal alkaline phosphatase: is this primary biliary cirrhosis? |journal=Hepatology |volume=6 |issue=6 |pages=1279–84 |year=1986 |pmid=3793004 |doi=10.1002/hep.1840060609}}</ref> | ||
==Epidemiology and Demographics== | |||
The female:male ratio is at least 9:1. In some areas of the US and UK the prevalence is estimated to be as high as 1 in 4000. This is much more common than in South America or Africa, which may be due to better recognition in the US and UK. First-degree relatives may have as much as a 500 times increase in prevalence, but there is debate if this risk is greater in the same generation relatives or the one that follows. | |||
==References== | ==References== | ||
Revision as of 19:39, 16 April 2013
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Differentiating Primary Biliary Cirrhosis from other Diseases |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Primary biliary cirrhosis is an autoimmune disease of the liver marked by the slow progressive destruction of the small bile ducts (bile canaliculi) within the liver. When these ducts are damaged bile builds up in the liver (cholestasis) and over time damages the tissue. This can lead to scarring, fibrosis, cirrhosis, and ultimately liver failure.
Historical Perspective
Addison and Gull in 1851 described the clinical picture of progressive obstructive jaundice in the absence of mechanical obstruction of the large bile ducts. Ahrens et al in 1950 coined the term primary biliary cirrhosis for this disease. The association with anti mitochondrial antibodies was first reported in 1986.[1]
Epidemiology and Demographics
The female:male ratio is at least 9:1. In some areas of the US and UK the prevalence is estimated to be as high as 1 in 4000. This is much more common than in South America or Africa, which may be due to better recognition in the US and UK. First-degree relatives may have as much as a 500 times increase in prevalence, but there is debate if this risk is greater in the same generation relatives or the one that follows.
References
- ↑ Mitchison HC, Bassendine MF, Hendrick A; et al. (1986). "Positive antimitochondrial antibody but normal alkaline phosphatase: is this primary biliary cirrhosis?". Hepatology. 6 (6): 1279–84. doi:10.1002/hep.1840060609. PMID 3793004.