Liver cirrhosis in children

Abstract

Liver cirrhosis in children and adolescents is a consequence of a variety of chronic liver diseases, metabolic disorders, and genetic diseases. In infants, cirrhosis is most often due to biliary atresia and genetic-metabolic diseases, while in older children it usually occurs as a result of autoimmune hepatitis, Wilson’s disease, alpha-1 antitrypsin deficiency, and primary sclerosing cholangitis. Symptoms of cirrhosis in children and adolescents are not specific. In pediatric patients, the first sign of cirrhosis may more often be low weight gain, weakness, loss of appetite, low-grade fever, as well as more specific signs: transient jaundice, mild pruritus, epistaxis, heaviness in the right hypochondrium, changes in liver density on palpation, hyperbilirubinemia, hyperenzymemia, etc. Complications of pediatric cirrhosis are similar to those observed in adult patients and include gastrointestinal bleeding caused by esophageal varices, ascites, and spontaneous bacterial peritonitis. In pediatric practice, special attention should be paid to nutritional correction in liver cirrhosis since children have higher requirements for protein and micronutrients for growth and development. Treatment of cirrhosis-induced portal hypertension in children and adolescents is mainly based on methods developed for adults. The article deals with diagnostic and differential diagnostic aspects of the terminal stage of liver disease in children. The therapeutic management of patients with cirrhosis of the liver and acute renal failure is based on support of various organ functions. Hemodialysis/ plasmapheresis may serve in some patients as an interim therapy before liver transplantation. The indications for liver transplantation and problems arising after surgery are considered.