Budd-Chiari syndrome pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mazia Fatima, MBBS [2]
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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.