Budd-Chiari syndrome pathophysiology: Difference between revisions
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==Pathogenesis== | ==Pathogenesis== | ||
*Occlusion of at least two hepatic veins leads to Budd-Chiari syndrome. Single hepatic vein occlusion is usually silent. | *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> | ||
*Obstruction in the venous drainage from liver results in venous congestion causing hepatomegaly.Patients develop postsinusoidal portal hypertension. | *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. | *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. | *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. | *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== | ==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> | ||
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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
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
Microscopic Pathology
References
- ↑ 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.