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What causes cirrhosis in children?
Last reviewed: 19.10.2021
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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;
- Alazhil's syndrome and non-syndromic form of hypoplasia of intrahepatic bile ducts;
- metabolic disorders; deficiency of alpha1-antitrypsin, hemochromatosis, type IV glycogen disease, Niman-Pick disease. Gaucher disease, progressive familial intrahepatic cholestasis of type III, porphyria, cystic fibrosis. With Wilson's disease, tyrosinemia, fructoseemia, galactosemia, cirrhosis is formed in the case of untimely treatment of these diseases;
- impairment of the synthesis or excretion of bile acids (illness and Byler's syndrome);
- primary biliary cirrhosis;
- primary sclerosing cholangitis;
- violations of venous outflow: Badda-Chiari syndrome, veno-occlusive disease, right ventricular heart failure;
- alcoholic liver damage;
- cryptogenic cirrhosis;
- other causes (sarcoidosis, parasitic liver damage, hypervitaminosis A, etc.).
Pathogenesis of cirrhosis of the liver
At the heart of the development of liver cirrhosis is necrosis of hepatocytes. Leading to the collapse of the hepatic lobules, diffuse formation of fibrotic septa and the appearance of regeneration sites. The leading place in the pathogenesis of cirrhosis belongs to type I and III collagen, 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 sinusoids lose their vascularization and become impermeable to proteins (albumin, clotting factors, etc.). Synthesis of collagen in the Disse space is carried out by Ito cells, specializing in the accumulation of lipids. Under the action 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. Restricted by fibrotic septa hepatocytes regenerate, forming spherical lobes, significantly violating the blood flow. The bile ducts are obliterated, breaking the outflow of bile. Due to these changes, collaterals are formed, connecting the portal vein with the terminal sections of the hepatic veins, bypassing the hepatic parenchyma.