Cirrhosis of the liver in children

Liver cirrhosis in children is an anatomical concept that implies reorganization of the organ structure due to the development of fibrosis and regeneration nodules. Disorganization of the lobules and vascular triads of the liver leads to portal hypertension, the development of extra- and intrahepatic portocaval anastomoses, and a deficiency in the blood supply to the nodules. From a clinical point of view, cirrhosis is a chronic diffuse liver lesion with the proliferation of non-functional connective tissue. Biliary cirrhosis is cirrhosis that develops as a result of chronic cholestasis.

It should be noted that fibrosis is not a synonym for cirrhosis. In the case of fibrosis, the functional state of the liver is usually not impaired, and the only clinical symptom is a disruption of portal blood flow with the formation of portal hypertension. Fibrosis is often discovered by chance. The formation of regeneration nodes without fibrosis (for example, with partial nodular transformation of the liver) is also not considered cirrhosis.

ICD-10 codes

• K74. Liver cirrhosis and fibrosis.
• K74.6. Other and unspecified cirrhosis of liver.
• K74.4. Secondary biliary cirrhosis.
• K74.5. Biliary cirrhosis, unspecified.

Epidemiology of liver cirrhosis

The incidence of liver cirrhosis in pediatric patients has not been established. Liver cirrhosis accounts for 1.2% of all causes of death in the United States. Chronic liver disease and cirrhosis cause 35,000 deaths annually.

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Causes of liver cirrhosis in children?

The causes of cirrhosis in children are varied. First of all, these 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.

What causes liver cirrhosis in children?

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Symptoms of liver cirrhosis in children

Symptoms of liver cirrhosis in children include jaundice, skin itching of varying severity (as the liver's synthetic function deteriorates, itching decreases due to decreased synthesis of bile acids), hepatosplenomegaly, increased vascular pattern in the abdomen and chest, and general symptoms (anorexia, weight loss, weakness, and decreased muscle mass). In severe cases, a pronounced venous network forms on the abdomen in the form of a "caput medusa." Gastrointestinal bleeding from varicose veins of the esophagus or rectum may occur. Telangiectasias, palmar erythema, nail changes ("clubbing"), peripheral neuropathy, and hepatic encephalopathy are common.

Symptoms of liver cirrhosis in children

Classification of liver cirrhosis

Morphologically, there are 3 forms of cirrhosis - small-nodular, large-nodular and mixed. From a clinical point of view, cirrhosis should be classified by the etiologic factor.

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Diagnosis of liver cirrhosis in children

When collecting anamnesis, it is necessary to establish the timing of the onset of the first clinical signs and patterns of disease development, the presence of cases of hepatobiliary system pathology in the family history.

During a physical examination, it is necessary to assess the child's physical development, the severity of jaundice, the presence of increased vascular pattern on the chest and abdomen, extrahepatic symptoms (telangiectasia, palmar erythema, "drumsticks", peripheral neuropathy, etc.), edema syndrome. It is necessary to measure the size of the liver and spleen, abdominal circumference (in case of ascites), assess the color of stool and urine.

Diagnosis of liver cirrhosis in children

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Treatment of liver cirrhosis in children

The basis of liver cirrhosis treatment is prevention and correction of liver cirrhosis complications. The diet is high-calorie, containing branched amino acids. Drug treatment involves correction of liver cirrhosis complications.

The key element of ascites treatment is considered to be sodium restriction in the diet, which is often difficult to achieve in children. The second component is to ensure sufficient potassium. When prescribing diuretics, the drug of choice is considered to be spironolactone, prescribed at a dose of 2-3 mg / (kg x day). In case of inefficiency, furosemide is used at a dose of 1-3 mg / (kg x day). The prescription of diuretics requires daily monitoring of diuresis, body weight, abdominal circumference and blood electrolyte levels. The danger of treatment with diuretics is the risk of collapse with too sharp a loss of fluid, dilutional hyponatremia due to insufficient secretion of antidiuretic hormone, provocation of portosystemic encephalopathy due to water-electrolyte and circulatory disorders.

How is liver cirrhosis treated in children?