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What causes cirrhosis of the liver in children?
Last reviewed: 06.07.2025
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The cause of cirrhosis in children are various diseases of the hepatobiliary system:
- viral and autoimmune hepatitis;
- toxic liver damage;
- biliary atresia;
- Alagille syndrome and non-syndromic form of intrahepatic bile duct hypoplasia;
- metabolic disorders; alpha1-antitrypsin deficiency, hemochromatosis, glycogen storage disease type IV, Niemann-Pick disease. Gaucher disease, progressive familial intrahepatic cholestasis type III, porphyria, cystic fibrosis. In Wilson's disease, tyrosinemia, fructosemia, galactosemia, liver cirrhosis develops in case of untimely treatment of these diseases;
- disorders of the synthesis or excretion of bile acids (Byler's disease and syndrome);
- primary biliary cirrhosis;
- primary sclerosing cholangitis;
- venous outflow disorders: Budd-Chiari syndrome, veno-occlusive disease, right ventricular heart failure;
- alcoholic liver disease;
- cryptogenic cirrhosis;
- other causes (sarcoidosis, parasitic liver lesions, hypervitaminosis A, etc.).
Pathogenesis of liver cirrhosis
The development of liver cirrhosis is based on necrosis of hepatocytes, leading to the collapse of liver lobules, diffuse formation of fibrous septa and occurrence of regeneration nodes. The leading place in the pathogenesis of liver cirrhosis belongs to collagen types I and III, deposited in the liver lobules (Disse space), whereas normally these types of collagen are found in the portal tracts and around the central veins. Endothelial cells of the sinusoids lose vascularization and become impermeable to proteins (albumin, blood clotting factors, etc.). Collagen synthesis in the Disse space is carried out by Ito cells, specializing in the accumulation of lipids. Under the influence of inflammatory cytokines, these cells become similar to myofibroblasts and begin to produce collagen. The lumen of the sinusoids decreases, which contributes to an increase in vascular resistance. Hepatocytes limited by fibrous septa regenerate, forming spherical lobules, significantly disrupting blood flow. Bile ducts are obliterated, disrupting the outflow of bile. As a result of these changes, collaterals are formed, connecting the portal vein with the terminal sections of the hepatic veins, bypassing the liver parenchyma.