Symptoms of liver cirrhosis in children

Clinical symptoms of liver cirrhosis 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.

Complications of liver cirrhosis

Complications of cirrhosis include portal hypertension, spontaneous bacterial peritonitis, hepatic encephalopathy, hepatorenal and hepatopulmonary syndromes, hepato- and cholangiocarcinoma.

Portal hypertension is an increase in pressure in the portal vein, leading to an increase in the pressure gradient between the portal and inferior vena cava. Increased resistance to portal blood flow leads to the formation of portosystemic collaterals. The formation of ascites is associated with an increase in pressure in the intrahepatic lymphatic vessels and fluid leakage through the liver capsule into the abdominal cavity. An additional factor is the disruption of the catabolism of hormones and other biologically active substances in the liver, provoking serum concentrations of renin, aldosterone, angiotensin, vasopressin, leading to sodium and water retention.

Spontaneous bacterial peritonitis is the most common infectious complication of cirrhosis. In this case, mortality in adults reaches 61-78%. The development of spontaneous bacterial peritonitis is initiated by microbial contamination of ascitic fluid. The main source of seeding of the abdominal cavity is the microflora of the large intestine, penetrating into the ascitic fluid due to increased permeability of the intestinal wall. Rarer causes are hematogenous spread of infection against the background of persistent bacteremia, infection during paracentesis or imposition of a peritoneovenous shunt. The increased tendency of patients with liver cirrhosis to develop bacterial complications is due to a decrease in the nonspecific resistance of the body. In the pathogenesis of spontaneous bacterial peritonitis, an important role is played by ascitic fluid as a medium for contact of nonspecific resistance factors with microorganisms. It is assumed that with a large volume of ascitic fluid, the possibility of contact between polymorphonuclear leukocytes and bacterial cells decreases.

Hepatic encephalopathy in liver cirrhosis is the most severe and prognostically unfavorable complication. Endogenous neurotoxins and amino acid imbalance, accumulating as a result of hepatocellular insufficiency, lead to edema and functional disorders of astroglia. These changes increase the permeability of the blood-brain barrier, change the activity of ion channels, disrupt the processes of neurotransmission and the supply of neurons with macroergic compounds.

The most significant neurotoxin is ammonia, the increase in the concentration of which is associated with a decrease in the synthesis of urea (the ornithine cycle of ammonia inactivation) and glutamine in the liver. Ammonia in non-ionized form penetrates the blood-brain barrier, exerting a neurotoxic effect.

Amino acid imbalance in liver failure - an increase in the content of aromatic amino acids (phenylalanine, tyrosine, etc.) in the blood and a decrease in the concentration of amino acids with a branched side chain. Excessive intake of aromatic amino acids into the brain is accompanied by the synthesis of false transmitters, structurally similar to norepinephrine and dopamine.

Hepatic encephalopathy includes various neuropsychiatric disorders, the correct assessment of which in pediatric practice, especially during the first year of life, is difficult. The most objective diagnostic criterion is considered to be the results of electroencephalography. Depending on the stage of hepatic encephalopathy, a slowdown in the activity of the a-rhythm of varying severity and the appearance of 5- and 9-activity are detected. In older children, psychometric testing can be performed to detect disorders characteristic of stages I and II of hepatic encephalopathy. The number connection test and the number-symbol test are aimed at determining the speed of cognitive activity. The line test and the dotted figure tracing test allow us to determine the speed and accuracy of fine motor skills.

Determining the concentration of ammonia in the blood has diagnostic value. In most patients, the concentration of ammonia is elevated, but normal ammonia levels cannot serve as a basis for excluding the diagnosis of hepatic encephalopathy.

The most informative diagnostic methods are magnetic resonance spectroscopy and evoked potentials of the brain. Magnetic resonance spectroscopy reveals an increase in the intensity of the T signal, basal ganglia and white matter of the brain, as well as a decrease in the myoinositol/creatine ratio, an increase in the glutamine peak in the gray and white matter of the brain. The severity of these changes correlates with the severity of hepatic encephalopathy. The sensitivity of this method approaches 100%.

The method of evoked potentials of the brain has lower sensitivity (about 80%). The main changes concern the slowing of interpeak latencies.

Hepatorenal syndrome is a progressive renal failure that develops against the background of liver cirrhosis and occurs with portal hypertension. Clinical and laboratory symptoms include oliguria, increased serum creatinine, and decreased glomerular filtration.

Hepatopulmonary syndrome includes an increase in the alveolar-arterial gradient and dilation of the intrapulmonary vessels, characterized by dyspnea, clubbing of the fingers, and hypoxemia. Pulmonary hypertension, decreased vital capacity, and hepatic hydrothorax may develop.

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