How is cirrhosis treated in children?

Indications for consultation of other specialists

The detection of liver cirrhosis serves as an indication for the consultation of a surgeon and a neurologist.

Indications for hospitalization

Indication for hospitalization is the development of life-threatening complications, the need for parenteral drug administration, and liver transplantation.

Treatment of cirrhosis of the liver

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

Non-drug treatment of liver cirrhosis

The diet is high-calorie, containing branched amino acids.

Medicamentous treatment of cirrhosis of the liver

Medication provides for correction of complications of liver cirrhosis.

Portal hypertension. 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 the case of inefficiency, furosemide is used at a dose 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.

The development of ascites is accompanied by hypoalbuminemia. Reducing oncotic pressure and causing inefficiency of diuretic treatment. To correct hypoalbuminemia, albumin solutions are used at a rate of 1 g / (kg x day). Ascites are considered refractory if it is not possible to control the accumulation of fluid against the backdrop of applying the maximum doses of diuretics in combination with albumin infusion. In this case, the paracentesis is carried out and the liquid is removed.

With 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 the reduction of portal blood flow and / or hepatic resistance, which contributes to the reduction of portal pressure. Vasoconstrictors (vasopressin, nonselective beta-blockers) that reduce visceral blood flow, portal blood flow and portal pressure are used. The drug of choice is propranolol (obzidan) in a dose of 1-2 mg / (kghsut) 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, these drugs are used in a limited way.

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

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

The relief of acute bleeding involves the abolition of feeding, the installation of a nasogastric tube, a decrease in the volume of fluid to 2/3 of the physiological requirement, and the introduction of haemostatic drugs. If the conservative treatment is ineffective, sclerotherapy is performed.

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

Hepatic encephalopathy. Treatment of this disease, especially severe forms, involves significant difficulties. In adult practice, the mortality rate is 25-80%, depending on the degree of severity. An important component of the treatment is a diet that provides 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 famous and widely used drug is lactulose (dyufalac).

Hepatorenal syndrome. Treatment of hepatorenal syndrome includes restriction of table salt in a diet. With pronounced hyponatremia, a decrease in the volume of fluid used is indicated. Among drugs that participated in scientific research but not yet approved by the standards of treatment, it should be noted ornipressin (an analogue of vasopressin), which has a vasoconstrictive effect, eliminating the hyperdynamic type of circulation, increasing glomerular filtration and sodium excretion. Another drug - aprotinin (an inhibitor of the kallikrein-kinin system), causes narrowing of the vessels of the internal organs with an increase in renal blood flow.

The following treatment methods are recognized as ineffective: hemodialysis, peritoneal dialysis, introduction of plasma substitutes, paracentesis and the use of vasoactive drugs of systemic action.

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 evaluation of which Child-Pugh classification is widely used in older children and adults.

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

In children's practice, an objective assessment of complications of liver cirrhosis is of paramount importance; The severity of the condition can significantly affect survival after surgery and the likelihood of postoperative complications. In this regard, determining the optimal timing of liver transplantation is especially important. One of the indicators in the Child-Pugh scale in assessing liver function is the degree of severity of encephalopathy, the definition of which is difficult in young children. Given this factor, other scales have been proposed that include a wider range of laboratory indicators and allow one to assess the severity of the condition of the patient in the first year of life in order 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;
• the content of indirect bilirubin is 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 sum of the points is determined by the risk of lethality within 6 months. With a score of more than 40, there is a high risk of death (over 75%) due to the development of complications of liver cirrhosis. With a total of 29-39, the risk is 75%, if the score is less than 28, the probability of complications is minimal and the lethality within 6 months is less than 25%.

Forecast

Prognosis unfavorable without liver transplantation. Survival of children after this operation is more than 90%.

Prevention

Vaccination against viral hepatitis A and B.

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