Symptoms of liver cirrhosis in children

Clinical symptoms of cirrhosis of the liver 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 reduction 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.

Complications of liver cirrhosis

The complications of cirrhosis include portal hypertension, spontaneous bacterial peritonitis, hepatic encephalopathy, hepatorenal and hepatic-pulmonary syndromes, hepato- and cholangiocarcinoma.

Portal hypertension is an increase in pressure in the portal portal, 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 increased pressure in the intrahepatic lymphatic vessels and sweating of fluid through the capsule of the liver into the abdominal cavity. An additional factor is 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 frequently developing infectious complication of cirrhosis. In this case, mortality in adults reaches 61-78%. The development of spontaneous bacterial peritonitis initiates microbial contamination of ascites fluid. The main source of colonization of the abdominal cavity is the microflora of the large intestine, which penetrates into the ascitic fluid due to increased permeability of the intestinal wall. Rareer causes are hematogenous spread of the infection against persistent bacteremia, infection with paracentesis or peritoneovenovenous shunt application. Increased tendency of patients with cirrhosis of the liver to the development of bacterial complications is due to a decrease in nonspecific resistance of the organism. In the pathogenesis of spontaneous bacterial peritonitis, an important role is played by ascitic fluid as a medium for contacting non-specific resistance factors with microorganisms. It is assumed that with a large volume of ascitic fluid, the possibility of contact of polymorphonuclear leukocytes with bacterial cells decreases.

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

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

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

Hepatic encephalopathy includes a variety of neuropsychic disorders, a correct assessment of which in children's practice, especially during the first year of life, is difficult. The most objective criterion of diagnosis is the results of electroencephalography. Depending on the stage of hepatic encephalopathy, the activity of a-rhythm of varying severity is slowing down and the appearance of 5- and 9-activity. At an older age, psychometric testing is possible to detect abnormalities characteristic of stages I and II of hepatic encephalopathy. The test of the connection of numbers and the test "number-symbol" are aimed at determining the speed of cognitive activity. The test of the line and the test of dotting of the dotted figures allow to determine the speed and accuracy of fine motor skills.

Diagnostic value is the determination of the concentration of ammonia in the blood. In most patients, the concentration of ammonia is increased, but the normal ammonia content can not 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. Magneto-resonance spectroscopy indicates 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 less sensitivity (about 80%). The main changes concern the slowing of interpic latencies.

Hepatorenal syndrome is a progressive renal failure that develops against the background of cirrhosis of the liver and proceeds with portal hypertension. Clinical and laboratory symptoms include oliguria, an increase in serum creatinine and a decrease in glomerular filtration.

Hepatopulmonary syndrome includes an increase in the alveolar-arterial gradient and enlargement of intrapulmonary vessels, characterized by shortness of breath, deformation of the fingers in the form of "drumsticks" and hypoxemia. Pulmonary hypertension, a decrease in the vital capacity of the lungs, a hepatic hydrothorax can begin.

[1], [2], [3], [4]