Budd-Chiari syndrome pathophysiology: Difference between revisions
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{{Budd-Chiari syndrome}} | {{Budd-Chiari syndrome}} | ||
{{CMG}}; {{AE}} | {{CMG}}; {{AE}}{{Mazia}} | ||
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== Overview== | == Overview== | ||
[[Occlusion]] of at least two [[hepatic veins]] leads to Budd-Chiari syndrome. Single [[hepatic vein]] [[occlusion]] is usually [[Silent mutation|silent]]. [[Obstruction]] in the [[Venous|venous drainage]] from [[liver]] results in [[Venous|venous congestion]] causing [[hepatomegaly]]. [[Patients]] develop [[Sinusoidal|postsinusoidal]] [[portal hypertension]]. [[Stasis]] of [[blood]] and [[congestion]] cause [[Hypoxic|hypoxic damage]] | [[Occlusion]] of at least two [[hepatic veins]] leads to Budd-Chiari syndrome. Single [[hepatic vein]] [[occlusion]] is usually [[Silent mutation|silent]]. [[Obstruction]] in the [[Venous|venous drainage]] from [[liver]] results in [[Venous|venous congestion]] causing [[hepatomegaly]]. [[Patients]] may develop [[Sinusoidal|postsinusoidal]] [[portal hypertension]]. [[Stasis]] of [[blood]] and [[congestion]] cause [[Hypoxic|hypoxic damage]] in the [[hepatocytes]], affecting [[liver function]]. This can result in centrilobular [[fibrosis]], nodular regenerative [[hyperplasia]] and ultimately [[cirrhosis]]. [[Hepatocellular Disease|Hepatocellular]] [[necrosis]] results from increased [[Sinusoid (blood vessel)|sinusoidal]] [[pressure]]. Budd-Chiari syndrome is commonly associated with [[atrophy]] of peripheral regions and [[Caudate lobe of liver|enlargement of the caudate lobe]] because [[blood]] is directly shunted through it into the [[Inferior vena cavae|inferior vena cava]]. [[Genes]] associated with increased [[expression]] in the [[pathogenesis]] of Budd-Chiari syndrome include [[Matrix metalloproteinase|matrix metalloproteinase 7]], [[Superior cervical ganglion|superior cervical ganglion 10 (SCG10)]], proliferating [[cell]] [[nuclear]] [[antigen]] [[gene]], [[c-myc]] [[Oncogenes|oncogene]], [[P53 gene|tumor protein p53 gene]], [[Thrombospondin|thrombospondin-1]] [[gene]]. | ||
==Pathogenesis== | ==Pathogenesis== | ||
*[[Occlusion]] of at least [[Hepatic veins|two hepatic veins]] leads to Budd-Chiari syndrome. [[Hepatic veins|Single hepatic vein]] [[occlusion]] is usually [[Silent mutations|silent]].<ref name="pmid26668741">{{cite journal |vauthors=Martens P, Nevens F |title=Budd-Chiari syndrome |journal=United European Gastroenterol J |volume=3 |issue=6 |pages=489–500 |year=2015 |pmid=26668741 |pmc=4669515 |doi=10.1177/2050640615582293 |url=}}</ref> | *[[Occlusion]] of at least [[Hepatic veins|two hepatic veins]] leads to Budd-Chiari syndrome. [[Hepatic veins|Single hepatic vein]] [[occlusion]] is usually [[Silent mutations|silent]].<ref name="pmid26668741">{{cite journal |vauthors=Martens P, Nevens F |title=Budd-Chiari syndrome |journal=United European Gastroenterol J |volume=3 |issue=6 |pages=489–500 |year=2015 |pmid=26668741 |pmc=4669515 |doi=10.1177/2050640615582293 |url=}}</ref> | ||
*[[Obstruction]] in the [[Venous|venous drainage]] from [[liver]] results in [[Venous|venous congestion]] causing [[hepatomegaly]]. [[Patients]] develop | *[[Obstruction]] in the [[Venous|venous drainage]] from [[liver]] results in [[Venous|venous congestion]] causing [[hepatomegaly]]. [[Patients]] develop post-[[Sinusoid (blood vessel)|sinusoidal]] [[portal hypertension]]. | ||
*[[Stasis]] of [[blood]] and [[congestion]] cause [[hypoxic]] damage of [[hepatocytes]], affecting the [[liver]] [[Function (biology)|function]].This can result in centrilobular [[fibrosis]], [[nodular regenerative hyperplasia]] and ultimately [[cirrhosis]]. | *[[Stasis]] of [[blood]] and [[congestion]] cause [[hypoxic]] damage of [[hepatocytes]], affecting the [[liver]] [[Function (biology)|function]].This can result in centrilobular [[fibrosis]], [[nodular regenerative hyperplasia]] and ultimately [[cirrhosis]]. | ||
*[[Necrosis|Hepatocellular necrosis]] results from increased [[Sinusoidal|sinusoidal pressure]]. | *[[Necrosis|Hepatocellular necrosis]] results from increased [[Sinusoidal|sinusoidal pressure]]. | ||
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**[[Matrix metalloproteinases|Matrix metalloproteinase 7]] | **[[Matrix metalloproteinases|Matrix metalloproteinase 7]] | ||
**[[Superior cervical ganglion|Superior cervical ganglion 10]] (SCG10) | **[[Superior cervical ganglion|Superior cervical ganglion 10]] (SCG10) | ||
**Proliferating cell nuclear antigen gene | **Proliferating [[cell]] [[nuclear]] [[antigen]] [[gene]] | ||
**[[Oncogenes|C-MYC oncogene]] | **[[Oncogenes|C-MYC oncogene]] | ||
**[[P53 gene|Tumor protein p53 gene]] | **[[P53 gene|Tumor protein p53 gene]] | ||
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==Gross Pathology== | ==Gross Pathology== | ||
*[[Stasis]] of [[blood]] and [[congestion]] cause [[Hypoxic|hypoxic damage]] of [[hepatocytes]], affecting the [[Liver function test|liver function]].This can result in [[Fibrosis|centrilobular fibrosis]], [[nodular regenerative hyperplasia]] and ultimately [[cirrhosis]].<ref name="pmid26668741">{{cite journal |vauthors=Martens P, Nevens F |title=Budd-Chiari syndrome |journal=United European Gastroenterol J |volume=3 |issue=6 |pages=489–500 |year=2015 |pmid=26668741 |pmc=4669515 |doi=10.1177/2050640615582293 |url=}}</ref> | *[[Stasis]] of [[blood]] and [[congestion]] cause [[Hypoxic|hypoxic damage]] of [[hepatocytes]], affecting the [[Liver function test|liver function]].This can result in [[Fibrosis|centrilobular fibrosis]], [[nodular regenerative hyperplasia]] and ultimately [[cirrhosis]].<ref name="pmid26668741">{{cite journal |vauthors=Martens P, Nevens F |title=Budd-Chiari syndrome |journal=United European Gastroenterol J |volume=3 |issue=6 |pages=489–500 |year=2015 |pmid=26668741 |pmc=4669515 |doi=10.1177/2050640615582293 |url=}}</ref> | ||
*[[Necrosis|Hepatocellular necrosis]] results from increased sinusoidal [[pressure]]. | *[[Necrosis|Hepatocellular necrosis]] results from increased [[sinusoidal]] [[pressure]]. | ||
==Microscopic Pathology== | ==Microscopic Pathology== | ||
*Budd-Chiari is commonly associated with [[atrophy]] of peripheral regions and enlargement of the [[caudate lobe]] because [[blood]] is directly shunted through it into the [[Inferior vena cavae|inferior vena cava]].<ref name="pmid26668741">{{cite journal |vauthors=Martens P, Nevens F |title=Budd-Chiari syndrome |journal=United European Gastroenterol J |volume=3 |issue=6 |pages=489–500 |year=2015 |pmid=26668741 |pmc=4669515 |doi=10.1177/2050640615582293 |url=}}</ref> | *Budd-Chiari is commonly associated with [[atrophy]] of peripheral regions and enlargement of the [[caudate lobe]] because [[blood]] is directly shunted through it into the [[Inferior vena cavae|inferior vena cava]].<ref name="pmid26668741">{{cite journal |vauthors=Martens P, Nevens F |title=Budd-Chiari syndrome |journal=United European Gastroenterol J |volume=3 |issue=6 |pages=489–500 |year=2015 |pmid=26668741 |pmc=4669515 |doi=10.1177/2050640615582293 |url=}}</ref> | ||
Latest revision as of 16:30, 30 November 2017
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mazia Fatima, MBBS [2]
Overview
Occlusion of at least two hepatic veins leads to Budd-Chiari syndrome. Single hepatic vein occlusion is usually silent. Obstruction in the venous drainage from liver results in venous congestion causing hepatomegaly. Patients may develop postsinusoidal portal hypertension. Stasis of blood and congestion cause hypoxic damage in the hepatocytes, affecting liver function. This can result in centrilobular fibrosis, nodular regenerative hyperplasia and ultimately cirrhosis. Hepatocellular necrosis results from increased sinusoidal pressure. Budd-Chiari syndrome is commonly associated with atrophy of peripheral regions and enlargement of the caudate lobe because blood is directly shunted through it into the inferior vena cava. Genes associated with increased expression in the pathogenesis of Budd-Chiari syndrome include matrix metalloproteinase 7, superior cervical ganglion 10 (SCG10), proliferating cell nuclear antigen gene, c-myc oncogene, tumor protein p53 gene, thrombospondin-1 gene.
Pathogenesis
- Occlusion of at least two hepatic veins leads to Budd-Chiari syndrome. Single hepatic vein occlusion is usually silent.[1]
- Obstruction in the venous drainage from liver results in venous congestion causing hepatomegaly. Patients develop post-sinusoidal portal hypertension.
- Stasis of blood and congestion cause hypoxic damage of hepatocytes, affecting the liver function.This can result in centrilobular fibrosis, nodular regenerative hyperplasia and ultimately cirrhosis.
- Hepatocellular necrosis results from increased sinusoidal pressure.
- Budd-Chiari is commonly associated with atrophy of peripheral regions and enlargement of the caudate lobe because blood is directly shunted through it into the inferior vena cava.
Genetics
- Genes associated with increased expression in the pathogenesis of Budd-Chiari syndrome include:[2][3]
- Matrix metalloproteinase 7
- Superior cervical ganglion 10 (SCG10)
- Proliferating cell nuclear antigen gene
- C-MYC oncogene
- Tumor protein p53 gene
- Thrombospondin-1 gene expression is decreased in Budd-Chiari syndrome.
Gross Pathology
- Stasis of blood and congestion cause hypoxic damage of hepatocytes, affecting the liver function.This can result in centrilobular fibrosis, nodular regenerative hyperplasia and ultimately cirrhosis.[1]
- Hepatocellular necrosis results from increased sinusoidal pressure.
Microscopic Pathology
- Budd-Chiari is commonly associated with atrophy of peripheral regions and enlargement of the caudate lobe because blood is directly shunted through it into the inferior vena cava.[1]
References
- ↑ 1.0 1.1 1.2 Martens P, Nevens F (2015). "Budd-Chiari syndrome". United European Gastroenterol J. 3 (6): 489–500. doi:10.1177/2050640615582293. PMC 4669515. PMID 26668741.
- ↑ Paradis V, Bièche I, Dargère D, Cazals-Hatem D, Laurendeau I, Saada V, Belghiti J, Bezeaud A, Vidaud M, Bedossa P, Valla DC (2005). "Quantitative gene expression in Budd-Chiari syndrome: a molecular approach to the pathogenesis of the disease". Gut. 54 (12): 1776–81. doi:10.1136/gut.2005.065144. PMC 1774794. PMID 16162682.
- ↑ Dragoteanu M, Balea IA, Piglesan CD (2014). "Nuclear medicine dynamic investigations in the diagnosis of Budd-Chiari syndrome". World J Hepatol. 6 (4): 251–62. doi:10.4254/wjh.v6.i4.251. PMC 4009481. PMID 24799994.