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Difference between revisions of "Wilson's disease"
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'''Wilson disease's''' is a rare autosomal recessive genetic disease characterized by abnormal copper transportation. | '''Wilson disease's''' is a rare autosomal recessive genetic disease characterized by abnormal copper transportation. Its' presentation may be atypical. In the context of [[pathology]], it is typically seen as a [[liver biopsy]]. | ||
==General== | ==General== | ||
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==Microscopic== | ==Microscopic== | ||
Features: | Features: | ||
*Nothing specific - known as the great mimicker of liver pathology. | *Nothing specific - known as the great mimicker of [[liver pathology]]. | ||
*[[Steatosis of the liver|Steatosis]]. | *[[Steatosis of the liver|Steatosis]]. | ||
*Portal fibrosis. | *Portal fibrosis. | ||
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*Copper staining is a non-specific finding seen in many liver diseases; it is associated with impaired bile secretion.<ref name=pmid2464523>{{cite journal |author=Miyamura H, Nakanuma Y, Kono N |title=Survey of copper granules in liver biopsy specimens from various liver abnormalities other than Wilson's disease and biliary diseases |journal=Gastroenterol. Jpn. |volume=23 |issue=6 |pages=633–8 |year=1988 |month=December |pmid=2464523 |doi= |url=}}</ref> | *Copper staining is a non-specific finding seen in many liver diseases; it is associated with impaired bile secretion.<ref name=pmid2464523>{{cite journal |author=Miyamura H, Nakanuma Y, Kono N |title=Survey of copper granules in liver biopsy specimens from various liver abnormalities other than Wilson's disease and biliary diseases |journal=Gastroenterol. Jpn. |volume=23 |issue=6 |pages=633–8 |year=1988 |month=December |pmid=2464523 |doi= |url=}}</ref> | ||
Image | ===Image=== | ||
*[http://openi.nlm.nih.gov/legacy/detailedresult.php?img=3375662_yjbm_85_2_249_g03&req=4 Orecin staining in Wilson's diseae (nih.gov)]. | *[http://openi.nlm.nih.gov/legacy/detailedresult.php?img=3375662_yjbm_85_2_249_g03&req=4 Orecin staining in Wilson's diseae (nih.gov)]. | ||