Budd-Chiari syndrome pathophysiology: Difference between revisions
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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. Obstruction in the venous drainage from liver results in venous congestion causing hepatomegaly.Patients develop postsinusoidal 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.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. | [[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]] 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 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 two hepatic veins leads to Budd-Chiari syndrome. Single hepatic vein occlusion is usually 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 drainage from liver results in venous congestion causing hepatomegaly.Patients develop postsinusoidal portal hypertension. | *[[Obstruction]] in the [[Venous|venous drainage]] from [[liver]] results in [[Venous|venous congestion]] causing [[hepatomegaly]]. [[Patients]] develop postsinusoidal [[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. | *[[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]]. | ||
*Hepatocellular necrosis results from increased sinusoidal pressure. | *[[Necrosis|Hepatocellular necrosis]] results from increased [[Sinusoidal|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. | *Budd-Chiari is commonly associated with [[Atrophy|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]]. | ||
==Genetics== | ==Genetics== | ||
*Genes associated with increased expression in the pathogenesis of Budd-Chiari syndrome include:<ref name="pmid16162682">{{cite journal |vauthors=Paradis V, Bièche I, Dargère D, Cazals-Hatem D, Laurendeau I, Saada V, Belghiti J, Bezeaud A, Vidaud M, Bedossa P, Valla DC |title=Quantitative gene expression in Budd-Chiari syndrome: a molecular approach to the pathogenesis of the disease |journal=Gut |volume=54 |issue=12 |pages=1776–81 |year=2005 |pmid=16162682 |pmc=1774794 |doi=10.1136/gut.2005.065144 |url=}}</ref><ref name="pmid24799994">{{cite journal |vauthors=Dragoteanu M, Balea IA, Piglesan CD |title=Nuclear medicine dynamic investigations in the diagnosis of Budd-Chiari syndrome |journal=World J Hepatol |volume=6 |issue=4 |pages=251–62 |year=2014 |pmid=24799994 |pmc=4009481 |doi=10.4254/wjh.v6.i4.251 |url=}}</ref> | *[[Genes]] associated with increased [[expression]] in the [[pathogenesis]] of Budd-Chiari syndrome include:<ref name="pmid16162682">{{cite journal |vauthors=Paradis V, Bièche I, Dargère D, Cazals-Hatem D, Laurendeau I, Saada V, Belghiti J, Bezeaud A, Vidaud M, Bedossa P, Valla DC |title=Quantitative gene expression in Budd-Chiari syndrome: a molecular approach to the pathogenesis of the disease |journal=Gut |volume=54 |issue=12 |pages=1776–81 |year=2005 |pmid=16162682 |pmc=1774794 |doi=10.1136/gut.2005.065144 |url=}}</ref><ref name="pmid24799994">{{cite journal |vauthors=Dragoteanu M, Balea IA, Piglesan CD |title=Nuclear medicine dynamic investigations in the diagnosis of Budd-Chiari syndrome |journal=World J Hepatol |volume=6 |issue=4 |pages=251–62 |year=2014 |pmid=24799994 |pmc=4009481 |doi=10.4254/wjh.v6.i4.251 |url=}}</ref> | ||
**Matrix metalloproteinase 7 | **[[Matrix metalloproteinases|Matrix metalloproteinase 7]] | ||
**Superior cervical ganglion 10 (SCG10) | **[[Superior cervical ganglion|Superior cervical ganglion 10]] (SCG10) | ||
**Proliferating cell nuclear antigen gene | **Proliferating cell nuclear antigen gene | ||
**C-MYC oncogene | **[[Oncogenes|C-MYC oncogene]] | ||
**Tumor protein p53 gene | **[[P53 gene|Tumor protein p53 gene]] | ||
*Thrombospondin-1 gene expression is decreased in Budd-Chiari syndrome. | *[[Thrombospondin|Thrombospondin-1 gene]] expression is decreased in Budd-Chiari syndrome. | ||
==Gross Pathology== | ==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.<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> | ||
*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 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> | ||
==References== | ==References== | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
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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 develop postsinusoidal 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 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 postsinusoidal 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.