Cirrhosis of the liver in children

Cirrhosis of the liver in children may occur in the neonatal period due to liver damage in the antenatal period (fetal hepatitis). The cause of cirrhosis in newborn can be transferred mother viral infection (serum hepatitis, cytomegalovirus, rubella, herpes infection), in which the virus is transmitted transplacentally to the foetus. Marked liver damage in the newborn and when listeriosis, syphilis, toxoplasmosis mother. The severity of cirrhotic liver changes at the birth of a child depends on the duration of infection in the period of intrauterine development. Cirrhosis may develop in connection with congenital zaradenie (atresia) biliary tract.
The main symptoms of liver cirrhosis in newborns are enlarged liver and spleen, jaundice, slightly coloured stools, presence of bile pigments in the urine, gemorragii (various sizes bruises and hemorrhagic lesions on the face, chest and torso), bleeding from the rest of the umbilical cord, melena (see). Expressed for the development of venous network in the area of the abdominal wall.
In newborns clinically difficult to differentiate cirrhosis of the liver associated with the transferred fetal hepatitis, and biliary cirrhosis of the liver, caused by biliary atresia, which haemorrhagic syndrome can be detected only in the terminal stage. In difficult diagnostic cases apply laparoscopy combined with fine-needle biopsy of the liver or trial laparotomy.
The treatment is ineffective. Prescribe vitamins - B1 and B2-by 1-2 mg per year of life per day in 3 reception inside, B6-10-20 mg per day in two reception inside B12 - 5 g on 1 kg of weight per day intramuscularly. Steroids (prednisone) at the rate of 1 mg/kg of body weight per day for 3-4 weeks. Magnesium sulfate - 25% solution of 1 teaspoon 3 times a day. The prognosis is unfavorable.
Cirrhosis of the liver in children of older age is often a consequence of infectious hepatitis or intoxication by chemicals or drugs (dichloroethane, typos and others), as well as stagnation in connection with cardiovascular disorders. Has much in common with the passage of cirrhosis of the liver in adults. Some kind of flows cirrhosis of the liver, with hereditary diseases of metabolism, accompanied by the accumulation of various substances in the liver cells, for example glycogen when glycogenic disease, as well as by the growth of connective tissue due to portal hypertension.

Cirrhosis of the liver in children occur less often than in adults, but some of them in a timely manner is not recognized. Cirrhosis occurs more often in boys of school age. In respect of some forms of liver cirrhosis (congenital angiomatosis, family hemolytic anemia, gepatolentikuliarnaya degeneration) there is a family predisposition. Congenital character is the syndrome Krouvila - Baumgarten. Cirrhosis of the liver in children are widespread everywhere, but the most common in Asia and the Middle East. High morbidity and mortality in children during the first two years of life gives infantile cirrhosis (syndrome of Hay), found in South-East Asia. In the Central Asian republics of the USSR there are cases heliotropic cirrhosis (heliotropic dystrophy) after consumption of bread grain, contaminated with seeds of heliotrope.
The main forms of cirrhosis of the liver in children are the same as in adults - postnecrotic, portal and biliary; usually occur mixed forms. The most common cause of cirrhosis of the liver in children is epidemic hepatitis, including Busselton form. The babies may the rapid development of liver cirrhosis of viral etiology. Described cirrhosis of the liver, resulting in infancy on the ground rapidly developing fatty infiltration of the liver, and after visceral form of congenital toxoplasmosis.
The clinical picture of cirrhosis of the liver in children are usually characterized by an initial latent period. The disease begins with vague complaints of illness. In the early period marked weakness, drowsiness, fatigue, loss of appetite, headache, nausea, chilling, subfebrile temperature, dryness, and pale skin, nosebleeds. Appear recurrent jaundice (not always), dull pain in the liver area, flatulence, diarrhea or constipation. Already in the early period of the liver increased, hardened. Splenomegaly can be observed at different stages of cirrhosis. On the skin occur star telangiectasias, xanthelasma and areas of depigmentation. Sometimes joins skin itch. Further, there are retarded physical development, often depletion, hair loss, increased hemorrhagic syndrome.
Sooner or later, there are signs of portal hypertension and violations of the portal circulation (splenomegaly, varicose veins of the esophagus and stomach, giving a sudden profuse bleeding, extended subcutaneous venous network on the stomach). In end-stage cirrhosis of the liver in children along with increased bleeding, neurological signs (insomnia, delirium, tremor hands, neuritis) prevail ascites and General edema. Cardiosclerosis develops mainly in older children with severe right ventricular failure.

For cirrhosis of the liver in children as in adults, characterized by the decrease in total protein, albumin and increased globulins, especially ' -fractions of blood serum enzymes (transaminases, aldolase). Thymol, bromsulfaleinovy sample is positive, the synthesis of hippuric acid is reduced, the prothrombin time is lengthened. Often identified urobilinemia, high concentrations of bile acids and amino acids in the urine, hypochromic anemia. At jaundice increased amount of bilirubin and serum enzymes. The development of skin collaterals in the early stages can be identified by using infrared rays. Varicose veins of the esophagus is found on the x-ray. Especially valuable laparoscopy and needle biopsy of the liver, which allows not only to confirm the presence of cirrhosis of the liver in children, but also to establish the degree of the process activity considering clinical and laboratory data.
For cirrhosis of the liver in children often long, with periods of deterioration and improvement. Except for rare cases of recovery, the disease usually progresses, sometimes quickly. There are 3 stages in the course of cirrhosis of the liver in children: compensated; subcompensated with expanded clinical picture with preserved liver function terminal (ascitic, dystrophic) with signs of liver failure. Sick children die from profuse esophago-gastric bleeding, secondary diseases or gradually developing liver failure. Secondary infection accelerate the coming of the end. Hepatalgia (liver failure) and bleeding is the most threatening complications of cirrhosis of the liver in children.
The differential diagnosis of cirrhosis of the liver in children should be borne in mind chronic hepatitis, hepatocholecystitis, congestive liver, tuberculosis, leukemia, hemolytic anemia, reticuloendotheliosis, biliary atresia, system red lupus, toxoplasmosis, cytomegaly, glycogenic disease, lstinas, galactosemia, and other
Forecast of cirrhosis of the liver in children is serious. However, latent and erased forms, the process may end up with a cure.
Treatment. Apply hypochloride diet restriction of fat and curd cheese, vitamins C and complex, including B6, B12 and especially B15 (pangamic acid), mineral water, lipokain, methionine, plasma glucose, prednisolone, antibiotics, gipotiazid, aldactone, lasix, and other Favorit
If indicated, careful destruction of ascitic fluid.
In portal hypertension shown surgical methods, especially in the I - II stage of cirrhosis.
Prevention is the timely and proper treatment of diseases (epidemic hepatitis), leading to the development of cirrhosis. You dispensary observation and treatment of children with suspected liver cirrhosis.