Cirrhosis of the Liver Biliary Congenital

Congenital biliary liver cirrhosis: causes, symptoms and treatment

Congenital biliary cirrhosis, also known as hepatic biliary atresia syndrome, is a rare genetic disease that affects the liver in newborns and infants. This condition is characterized by the inability of the bile duct to exit the liver, which leads to the gradual destruction of liver tissue and the development of cirrhosis.

The causes of congenital biliary cirrhosis are not yet fully understood. However, research indicates a possible interaction between genetic and environmental factors. Some children may have genetic mutations that affect the normal development of bile ducts in the liver. As a result, congenital anomalies are formed that prevent the normal outflow of bile.

Symptoms of congenital biliary cirrhosis can appear at an early age in a child. One of the most characteristic signs is jaundice, which manifests itself as yellowing of the skin and whites of the eyes. Other symptoms include enlarged liver and spleen, pallor, lack of appetite, failure to gain weight, dark urine and light-colored stool. Some children may have other problems, such as bone damage, developmental delays, and digestive problems.

Diagnosis of congenital biliary cirrhosis includes a clinical examination, blood tests, and educational methods such as ultrasound examination of the liver and bile ducts. A liver biopsy may be required to definitively confirm the diagnosis.

Treatment of congenital biliary cirrhosis often requires surgery. One of the most effective methods is tangential portal angiography followed by cholangio-portalenterostomy (CP-CPE), also known as the Kasai procedure. This procedure restores bile flow by bypassing damaged bile ducts in the liver. However, if the Kasai procedure does not significantly improve the liver, a liver transplant may be required.

After surgery, children with congenital biliary cirrhosis require ongoing medical support and care. Regular visits to your doctor and following dietary and medication recommendations can help monitor your liver condition and prevent progression of the disease.

In conclusion, biliary congenital cirrhosis is a rare genetic disease that affects newborns and infants. It causes a disruption in the outflow of bile from the liver and leads to the gradual destruction of liver tissue and the development of cirrhosis. Early diagnosis and surgical intervention, such as the Kasai procedure, may be key to providing treatment and improving the prognosis for children suffering from this condition. However, it is important to remember that congenital biliary cirrhosis requires long-term and complex care, and regular medical supervision is necessary to maintain liver health and the overall well-being of the patient.

Congenital biliary liver cirrhosis or infantile jaundice is a polyetiological disease of young children, in which there is a significant decrease in the mass of non-functional tissue of healthy liver parenchyma of a diffuse nature due to intraorgan fibrosis and necrosis. The pathogenesis of cirrhosis is based on long-term, systematic toxic damage to the liver, predominantly of infectious and hereditary origin. Damage to parenchymal organs by viruses, parasitic factors and drug abuse, mainly of an alcoholic nature. Against the background of severe hemo- and glycoprotein losses (angina, viral infections, hemorrhagic vasculitis, visceral leishmaniasis), thrombosis, vitamin deficiencies A, E, B (40–60%), the course of the disease is severe, extremely rarely reaching the stage of cirrhosis, but often relapsing [1 , 2, 3].

If signs of liver failure are detected in a child, such as yellow discoloration of the skin, mucous membranes and sclera (jaundice), you should immediately consult a doctor to exclude cardiovascular, gastrointestinal and liver pathologies. The doctor may prescribe an ultrasound examination of the abdominal cavity, which will show an increase in the thickness of the liver parenchyma, fluid accumulation, or other abnormalities. Among the diseases accompanied by jaundice syndrome, the leading place is occupied by viral and alcoholic hepatitis, cirrhosis, echinococcosis, neoplasms of the hematopoietic and digestive organs, ectopic transplacental embolism, hemolysis, some immunodeficiency diseases, allergic, mechanical, mesenchymal liver dysfunction, chronic autoimmune hepatitis (CAIG), poisoning and intoxication of various etiologies, Wilson-Konovalov disease, leptospirosis.