Wilson's disease: Difference between revisions

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*[[Steatosis of the liver|Steatosis]].

*Portal fibrosis.

A. [[File:1 Wilson 1 680x512px.tif|Inflamed & relatively unaffected segments (40X).]]

B. [[File:2 Wilson 1 680x512px.tif|Bile ductular proliferation with interface hepatitis [inflammation of periportal hepatocytes] (200X).]]

C. [[File:3 Wilson 1 680x512px.tif|Enlarged hepatocytes, some with feathery degeneration, others with steatosis. Nuclei show nucleoli (400X).]]

D. [[File:4 Wilson 1 680x512px.tif|Trichrome shows periportal & sinusoidal fibrosis. No bridging was seen. (100X).]]

Wilson’s disease, pre-cirrhotic. A. Inflamed & relatively unaffected segments. B. Bile ductular proliferation with interface hepatitis [inflammation of periportal hepatocytes]. C. Enlarged hepatocytes, some with feathery degeneration, others with steatosis. Nuclei show nucleoli. D. Trichrome shows periportal & sinusoidal fibrosis.

[[File:4 38555059990939 sl 1.png|Wilson’s disease with cirrhosis]]

[[File:4 38555059990939 sl 2.png|Wilson’s disease with cirrhosis]]

[[File:4 38555059990939 sl 3.png|Wilson’s disease with cirrhosis]]

[[File:4 38555059990939 sl 4.png|Wilson’s disease with cirrhosis]]

[[File:4 38555059990939 sl 5.png|Wilson’s disease with cirrhosis]]

Wilson’s disease with cirrhosis in a 22 year old woman. A. Nodules show steatosis without definite triads. B. Trichrome shows minute fibrous bound nodules in this case. C. Reticulin shows extensive regeneration (2-3 nuclei thick cords with lack of direction) with nodule formation. D. Bridge with extensive proliferated bile ductules and acute and chronic inflammatory cells. E. Ballooning degeneration with Mallory bodies.

==Stains==

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==Additional testing==

*Mass spectrometry - ~~determine portion~~ of copper.

Copper quantification:

*Mass spectrometry.<ref name=pmid24731025>{{Cite journal | last1 = Hahn | first1 = SH. | title = Population screening for Wilson's disease. | journal = Ann N Y Acad Sci | volume = 1315 | issue = | pages = 64-9 | month = May | year = 2014 | doi = 10.1111/nyas.12423 | PMID = 24731025 }}</ref>

*Atomic absorption spectroscopy.

**>250 microg of copper/g of liver suggest Wilson's disease; below does not exclude it.<ref name=pmid16234011>{{Cite journal | last1 = Ferenci | first1 = P. | last2 = Steindl-Munda | first2 = P. | last3 = Vogel | first3 = W. | last4 = Jessner | first4 = W. | last5 = Gschwantler | first5 = M. | last6 = Stauber | first6 = R. | last7 = Datz | first7 = C. | last8 = Hackl | first8 = F. | last9 = Wrba | first9 = F. | title = Diagnostic value of quantitative hepatic copper determination in patients with Wilson's Disease. | journal = Clin Gastroenterol Hepatol | volume = 3 | issue = 8 | pages = 811-8 | month = Aug | year = 2005 | doi = | PMID = 16234011 }}</ref>

[[Sensitivity]]:<ref>{{Cite journal | last1 = Liggi | first1 = M. | last2 = Mais | first2 = C. | last3 = Demurtas | first3 = M. | last4 = Sorbello | first4 = O. | last5 = Demelia | first5 = E. | last6 = Civolani | first6 = A. | last7 = Demelia | first7 = L. | title = Uneven distribution of hepatic copper concentration and diagnostic value of double-sample biopsy in Wilson's disease. | journal = Scand J Gastroenterol | volume = 48 | issue = 12 | pages = 1452-8 | month = Dec | year = 2013 | doi = 10.3109/00365521.2013.845904 | PMID = 24164422 }}</ref>

*250 microg of copper/g of liver - 80-86% (value dependent on sampling).

*50 microg of copper/g of liver - 97%.

==See also==

@@ Line 22: / Line 22: @@
 *[[Steatosis of the liver|Steatosis]].
 *Portal fibrosis.
+A. [[File:1 Wilson 1 680x512px.tif|Inflamed & relatively unaffected segments  (40X).]]<br>
+B. [[File:2 Wilson 1 680x512px.tif|Bile ductular proliferation with interface hepatitis [inflammation of periportal hepatocytes]  (200X).]]<br>
+C. [[File:3 Wilson 1 680x512px.tif|Enlarged hepatocytes, some with feathery degeneration, others with steatosis. Nuclei show nucleoli (400X).]]<br>
+D. [[File:4 Wilson 1 680x512px.tif|Trichrome shows periportal & sinusoidal fibrosis. No bridging was seen. (100X).]]<br>
+Wilson’s disease, pre-cirrhotic. A. Inflamed & relatively unaffected segments. B. Bile ductular proliferation with interface hepatitis [inflammation of periportal hepatocytes]. C. Enlarged hepatocytes, some with feathery degeneration, others with steatosis. Nuclei show nucleoli. D. Trichrome shows periportal & sinusoidal fibrosis.
+[[File:4 38555059990939 sl 1.png|Wilson’s disease with cirrhosis]]
+[[File:4 38555059990939 sl 2.png|Wilson’s disease with cirrhosis]]
+[[File:4 38555059990939 sl 3.png|Wilson’s disease with cirrhosis]]
+[[File:4 38555059990939 sl 4.png|Wilson’s disease with cirrhosis]]
+[[File:4 38555059990939 sl 5.png|Wilson’s disease with cirrhosis]] <br>
+Wilson’s disease with cirrhosis in a 22 year old woman. A. Nodules show steatosis without definite triads.  B. Trichrome shows minute fibrous bound nodules in this case. C. Reticulin shows extensive regeneration (2-3 nuclei thick cords with lack of direction) with nodule formation. D. Bridge with extensive proliferated bile ductules and acute and chronic inflammatory cells. E. Ballooning degeneration with Mallory bodies.
 ==Stains==
@@ Line 34: / Line 47: @@
 ==Additional testing==
-*Mass spectrometry - determine portion of copper.
+Copper quantification:
+*Mass spectrometry.<ref name=pmid24731025>{{Cite journal  | last1 = Hahn | first1 = SH. | title = Population screening for Wilson's disease. | journal = Ann N Y Acad Sci | volume = 1315 | issue =  | pages = 64-9 | month = May | year = 2014 | doi = 10.1111/nyas.12423 | PMID = 24731025 }}</ref>
+*Atomic absorption spectroscopy.
+**>250 microg of copper/g of liver suggest Wilson's disease; below does not exclude it.<ref name=pmid16234011>{{Cite journal  | last1 = Ferenci | first1 = P. | last2 = Steindl-Munda | first2 = P. | last3 = Vogel | first3 = W. | last4 = Jessner | first4 = W. | last5 = Gschwantler | first5 = M. | last6 = Stauber | first6 = R. | last7 = Datz | first7 = C. | last8 = Hackl | first8 = F. | last9 = Wrba | first9 = F. | title = Diagnostic value of quantitative hepatic copper determination in patients with Wilson's Disease. | journal = Clin Gastroenterol Hepatol | volume = 3 | issue = 8 | pages = 811-8 | month = Aug | year = 2005 | doi =  | PMID = 16234011 }}</ref>
+[[Sensitivity]]:<ref>{{Cite journal  | last1 = Liggi | first1 = M. | last2 = Mais | first2 = C. | last3 = Demurtas | first3 = M. | last4 = Sorbello | first4 = O. | last5 = Demelia | first5 = E. | last6 = Civolani | first6 = A. | last7 = Demelia | first7 = L. | title = Uneven distribution of hepatic copper concentration and diagnostic value of double-sample biopsy in Wilson's disease. | journal = Scand J Gastroenterol | volume = 48 | issue = 12 | pages = 1452-8 | month = Dec | year = 2013 | doi = 10.3109/00365521.2013.845904 | PMID = 24164422 }}</ref>
+*250 microg of copper/g of liver - 80-86% (value dependent on sampling).
+*50 microg of copper/g of liver - 97%.
 ==See also==

Wilson's disease: Difference between revisions

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