Difference between revisions of "Medical liver disease"

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==Primary sclerosing cholangitis==
==Primary sclerosing cholangitis==
*Abbreviated ''PSC''.
*Abbreviated ''PSC''.
===General===
*Strongly associated with [[ulcerative colitis]]; 75-90% of PSC patients have [[inflammatory bowel disease]] (IBD).<ref name=emed_psc_over>Khurana V, Singh T. Primary sclerosing cholangitis. eMedicine.com. URL: [http://emedicine.medscape.com/article/187724-overview http://emedicine.medscape.com/article/187724-overview]. Accessed on: 29 November 2009.</ref>
*Strongly associated with [[ulcerative colitis]]; 75-90% of PSC patients have [[inflammatory bowel disease]] (IBD).<ref name=emed_psc_over>Khurana V, Singh T. Primary sclerosing cholangitis. eMedicine.com. URL: [http://emedicine.medscape.com/article/187724-overview http://emedicine.medscape.com/article/187724-overview]. Accessed on: 29 November 2009.</ref>
*Risk for [[cholangiocarcinoma]].<ref name=pmid19668124>{{Cite journal  | last1 = Jesudian | first1 = AB. | last2 = Jacobson | first2 = IM. | title = Screening and diagnosis of cholangiocarcinoma in patients with primary sclerosing cholangitis. | journal = Rev Gastroenterol Disord | volume = 9 | issue = 2 | pages = E41-7 | month =  | year = 2009 | doi =  | PMID = 19668124 }}</ref>
*Risk for [[cholangiocarcinoma]].<ref name=pmid19668124>{{Cite journal  | last1 = Jesudian | first1 = AB. | last2 = Jacobson | first2 = IM. | title = Screening and diagnosis of cholangiocarcinoma in patients with primary sclerosing cholangitis. | journal = Rev Gastroenterol Disord | volume = 9 | issue = 2 | pages = E41-7 | month =  | year = 2009 | doi =  | PMID = 19668124 }}</ref>


===Diagnosis===
====Diagnosis====
*Diagnosed radiologically.
*'''Diagnosed radiologically'''.
*Liver biopsy is rarely useful diagnostically<ref name=emed_psc>Khurana V, Singh T. Primary sclerosing cholangitis. eMedicine.com. URL: [http://emedicine.medscape.com/article/187724-diagnosis http://emedicine.medscape.com/article/187724-diagnosis]. Accessed on: 29 November 2009.</ref> - as the disease may be patchy.
*Liver biopsy is rarely useful diagnostically<ref name=emed_psc>Khurana V, Singh T. Primary sclerosing cholangitis. eMedicine.com. URL: [http://emedicine.medscape.com/article/187724-diagnosis http://emedicine.medscape.com/article/187724-diagnosis]. Accessed on: 29 November 2009.</ref> - as the disease may be patchy.
**The utility of the biopsy is staging.
**The utility of the biopsy is staging.


===Treatment===
====Treatment====
*None very good.
*None very good.
*May be indication for transplant.
*May be indication for transplant.


===Histopathology===
===Microscopic===
Features:
*Classic: "onion-skinning" - cells layer around the bile ducts; "onion skin" present in approx. 40% of cases.<ref name=medscape_psc>Steele et al. URL: [http://www.medscape.com/viewarticle/552500_6 http://www.medscape.com/viewarticle/552500_6]. Accessed on: 29 November 2009.</ref>
*Classic: "onion-skinning" - cells layer around the bile ducts; "onion skin" present in approx. 40% of cases.<ref name=medscape_psc>Steele et al. URL: [http://www.medscape.com/viewarticle/552500_6 http://www.medscape.com/viewarticle/552500_6]. Accessed on: 29 November 2009.</ref>
**Not pathognomonic for PSC<ref name=medscape_psc/> - but not too much else looks like this on microscopy (ergo good fellowship exam question).
**Not pathognomonic for PSC<ref name=medscape_psc/> - but not too much else looks like this on microscopy (ergo good fellowship exam question).
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Micrographs:
Micrographs:
*[http://www.pathconsultddx.com/pathCon/largeImage?pii=S1559-8675%2806%2970838-5&figureId=fig1 Onion-skinning (pathconsultddx.com)]
*[http://www.pathconsultddx.com/pathCon/largeImage?pii=S1559-8675%2806%2970838-5&figureId=fig1 Onion-skinning (pathconsultddx.com)]
Notes:
*PSC often has minimal inflammation.<ref>STC. 9 December 2010.</ref>


====Staging====
====Staging====
48,544

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