Cirrhosis of the liver in children

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

It should be noted that fibrosis is not synonymous with cirrhosis. In the case of fibrosis, as a rule, the functional state of the liver is not impaired, and the only clinical symptom is the violation of portal blood flow with the formation of portal hypertension. Often, fibrosis is detected by chance. The formation of regeneration nodes without fibrosis (for example, with partial nodal transformation of the liver) is also not considered cirrhosis.

ICD-10 codes

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

Epidemiology of liver cirrhosis

In pediatric practice, the incidence of cirrhosis has not been established. Disease of cirrhosis of the liver is 1.2% of all causes of death in the United States. From chronic liver diseases and cirrhosis, 35,000 people die every year.

[1], [2], [3], [4], [5], [6], [7], [8], [9], [10],

Causes of liver cirrhosis in children?

The causes of cirrhosis in children are diverse. First of all, these 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 of the liver is formed in the case of untimely treatment of these diseases.

What causes cirrhosis in children?

[11], [12]

Symptoms of liver cirrhosis in children

Symptoms of liver cirrhosis in children include jaundice, itchy skin of varying severity (as the synthetic function of the liver is affected, itching decreases due to reduced synthesis of bile acids), hepatosplenomegaly, increased vascular pattern in the abdomen and chest, general symptoms (anorexia, weight loss, weakness and a decrease in muscle mass). In severe cases, there is a pronounced venous network on the abdomen in the form of a "jellyfish head". Gastrointestinal bleeding can occur from varicose veins of the esophagus or rectum. Often there are telangiectasias, palmar erythema, changes in nails ("drumsticks"), peripheral neuropathy and hepatic encephalopathy.

Symptoms of liver cirrhosis in children

Classification of cirrhosis of the liver

Morphologically, there are 3 forms of cirrhosis - small-node, coarse-nodular and mixed. From the clinical point of view, cirrhosis should be classified according to the etiologic factor.

[13], [14], [15], [16], [17], [18], [19], [20]

Diagnosis of liver cirrhosis in children

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

At physical examination it is necessary to evaluate the physical development of the child, the severity of jaundice, the presence of an enhanced vascular pattern on the chest and abdomen, extrahepatic symptoms (telangiectasia, palmar erythema, "drumsticks", peripheral neuropathy, etc.), edematous syndrome. It is necessary to measure the size of the liver and spleen, abdominal circumference (with ascites), evaluate the color of the stool and urine.

Diagnosis of liver cirrhosis in children

[21], [22], [23], [24], [25], [26], [27], [28],

Treatment of cirrhosis in children

The basis for the treatment of liver cirrhosis is the prevention and correction of complications of liver cirrhosis. The diet is high-calorie, containing branched amino acids. Medication provides for correction of complications of liver cirrhosis.

The key to the treatment of ascites is the restriction of sodium in the diet, which is often difficult to achieve in children. The second component is the provision of a sufficient amount of potassium. When prescribing diuretics, the drug of choice is considered to be spironolactone, administered at a dose of 2-3 mg / (kilogram). In case of inefficiency, furosemide is used at a dosage of 1-Zmg / (kilogram). The appointment of diuretics requires daily monitoring of diuresis, body weight, abdominal circumference and the content of electrolytes in the blood. The risk of diuretic treatment is the risk of collapse with too rapid loss of fluid, hyponatremia of dilution due to insufficient secretion of antidiuretic hormone, provocation of portosystemic encephalopathy due to water-electrolyte and circulatory disorders.

How is cirrhosis treated in children?