How is cirrhosis of the liver in children treated?

Indications for consultation with other specialists

The detection of liver cirrhosis is an indication for consultation with a surgeon and a neurologist.

Indications for hospitalization

Indications for hospitalization are the development of life-threatening complications, the need for parenteral administration of drugs, and liver transplantation.

Treatment of liver cirrhosis

The goal of treatment is the prevention and correction of complications of liver cirrhosis.

Non-drug treatment of liver cirrhosis

The diet is high in calories and contains branched-chain amino acids.

Drug treatment of liver cirrhosis

Drug treatment involves correction of complications of liver cirrhosis.

Portal hypertension. 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.

The development of ascites is accompanied by hypoalbuminemia, which reduces oncotic pressure and causes the ineffectiveness of diuretic treatment. To correct hypoalbuminemia, albumin solutions are used at a rate of 1 g / (kg x day). Ascites is considered refractory if it is impossible to control fluid accumulation against the background of using maximum doses of diuretics in combination with albumin infusion. In this case, paracentesis and fluid removal are indicated.

In portal hypertension, the pressure gradient between the portal and inferior vena cava increases, leading to the formation of portosystemic collaterals. The basis of pharmacological treatment of varicose veins due to portal hypertension is a decrease in portal blood flow and / or hepatic resistance, which helps to reduce portal pressure. Vasoconstrictors (vasopressin, non-selective beta-blockers) are used, which reduce visceral blood flow, portal blood flow and portal pressure. The drug of choice is propranolol (obzidan) at a dose of 1-2 mg / (kg x day) under the control of blood pressure and pulse. In the absence of side effects, this drug can be used for a year or longer. The use of vasodilators (nitroglycerin, etc.) is also justified, but in pediatric practice, such drugs are used sparingly.

It is possible to use histamine H2-receptor blockers (ranitidine, famotidine, etc.), which reduce the acidity of gastric contents, but these drugs do not prevent varicose veins.

Recent studies of the effectiveness of sclerotherapy for the prevention of bleeding have shown that the method has no significant advantages over pharmacotherapy and is accompanied by a higher mortality rate. The use of sclerotherapy is justified in case of contraindications to other methods of treatment.

Stopping acute bleeding involves stopping feeding, installing a nasogastric tube, reducing the volume of fluid to 2/3 of the physiological requirement, and administering hemostatic drugs. If conservative treatment is ineffective, sclerotherapy is performed.

Spontaneous bacterial peritonitis. The drug of choice for the treatment of spontaneous bacterial peritonitis is considered to be an antibiotic from the group of cephalosporins of the third generation - cefotaxime, which has minimal hepatotoxicity. Antibiotic therapy is considered ineffective in the absence of a clinical effect within 3 days after the start of treatment, a significant number of neutrophils in the ascitic fluid, the presence of microflora resistant to this antibiotic according to the results of sowing. In the future, the choice of the drug is carried out taking into account the sensitivity of the sown microflora. At present, much attention is paid to the prevention of spontaneous bacterial peritonitis in patients with liver cirrhosis.

Hepatic encephalopathy. Treatment of this disease, especially severe forms, is associated with significant difficulties. In adult practice, mortality is 25-80% depending on the severity. An important component of treatment is a diet that provides for protein restriction and sufficient energy value (140-150 kcal/kg per day). Currently used drugs are aimed at reducing the degree of hyperammonemia. The most well-known and widely used drug is lactulose (Duphalac).

Hepatorenal syndrome. Treatment of hepatorenal syndrome includes limiting table salt in the diet. In case of severe hyponatremia, a decrease in the volume of fluid consumed is indicated. Among the drugs that have participated in scientific studies, but have not yet been approved by treatment standards, it is worth noting ornipressin (analog of vasopressin), which has a vasoconstrictor effect, eliminating the hyperdynamic type of blood circulation, increasing glomerular filtration and sodium excretion. Another drug, aprotinin (inhibitor of the kallikrein-kinin system), causes vasoconstriction of internal organs with an increase in renal blood flow.

The following treatment methods have been found to be ineffective: hemodialysis, peritoneal dialysis, administration of plasma substitutes, paracentesis, and the use of systemic vasoactive drugs.

Hepatopulmonary syndrome. The first signs of this syndrome are considered an indication for liver transplantation as soon as possible.

Surgical treatment of liver cirrhosis

A radical method of treatment is liver transplantation. The optimal timing of the operation is determined taking into account the risk of developing life-threatening complications, for the assessment of which the Child-Pugh classification is widely used in older children and adults.

There is a high risk of developing life-threatening complications of liver cirrhosis, therefore, indications for liver transplantation are patients classified in groups B and C according to the Child-Pugh scale. In group A, the risk of developing complications is minimal: the patient can receive conservative treatment until the transition to stage B or C.

In pediatric practice, an objective assessment of complications of liver cirrhosis is of the utmost importance; the severity of the condition can significantly affect survival after surgery and the likelihood of developing postoperative complications. In this regard, determining the optimal timing of liver transplantation is especially important. One of the indicators in the Child-Pugh scale for assessing liver function is the severity of encephalopathy, which is difficult to determine in young children. Taking this factor into account, other scales have been proposed that include a wider range of laboratory parameters and allow assessing the severity of the patient's condition in the first year of life to determine the optimal timing of liver transplantation:

• presence of ascites, +15 points;
• cholesterol content <100 mg/dL or <2.5 mmol/L, +15 points;
• indirect bilirubin content 3-6 mg/dl or 51-103 μmol/l, + 11 points;
• total bilirubin content >6 mg/dl or >103 µmol/l, +13 points;
• prothrombin index <50%, +10 points.

Using this scale, the risk of mortality within 6 months is determined by the sum of points. If the sum of points is more than 40, there is a high risk of death (more than 75%) due to the development of complications of liver cirrhosis. If the sum is 29-39, the risk is 75%, if the sum of points is less than 28, then the probability of complications is minimal and mortality within 6 months is less than 25%.

Forecast

The prognosis is unfavorable without liver transplantation. The survival rate of children after this operation is more than 90%.

Prevention

Vaccination against viral hepatitis A and B.

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