摘要
Several conditions, especially chronic liver diseases, can lead to cirrhosis in children and adolescents. Most cases in clinical practice are caused by similar etiologies. In infants, cirrhosis is most often caused by biliary atresia and genetic-metabolic diseases, while in older children, it tends to result from autoimmune hepatitis,Wilson's disease, alpha-1-antitrypsin deficiency and primary sclerosing cholangitis. The symptoms of cirrhosis in children and adolescents are similar to those of adults. However, in pediatric patients, the first sign of cirrhosis is often poor weight gain. The complications of pediatric cirrhosis are similar to those observed in adult patients, and include gastrointestinal bleeding caused by gastroesophageal varices, ascites and spontaneous bacterial peritonitis. In pediatric patients, special attention should be paid to the nutritional alterations caused by cirrhosis, since children and adolescents have higher nutritional requirements for growth and development. Children and adolescents with chronic cholestasis are at risk for several nutritional deficiencies. Malnutrition can have severe consequences for both pre- and post-liver transplant patients. The treatment of cirrhosis-induced portal hypertension in children and adolescents is mostly based on methods developed for adults. The present article will review the diagnostic and differential diagnostic aspects of end-stage liver disease in children, as well as the major treatment options for this condition.
Several conditions, especially chronic liver diseases,can lead to cirrhosis in children and adolescents. Mostcases in clinical practice are caused by similar etiologies.In infants, cirrhosis is most often caused by biliaryatresia and genetic-metabolic diseases, while in olderchildren, it tends to result from autoimmune hepatitis,Wilson's disease, alpha-1-antitrypsin deficiency andprimary sclerosingcholangitis. The symptoms of cirrhosisin children and adolescents are similar to those ofadults. However, in pediatric patients, the first sign ofcirrhosis is often poor weight gain. The complications ofpediatric cirrhosis are similar to those observed in adultpatients, and include gastrointestinal bleeding causedby gastroesophageal varices, ascites and spontaneousbacterial peritonitis. In pediatric patients, specialattention should be paid to the nutritional alterationscaused by cirrhosis, since children and adolescentshave higher nutritional requirements for growth anddevelopment. Children and adolescents with chroniccholestasis are at risk for several nutritional deficiencies.Malnutrition can have severe consequences for bothpre- and post-liver transplant patients. The treatmentof cirrhosis-induced portal hypertension in children andadolescents is mostly based on methods developed foradults. The present article will review the diagnostic anddifferential diagnostic aspects of end-stage liver diseasein children, as well as the major treatment options forthis condition.
基金
FIPE-HCPA(Research Incentive Fund-Hospital de Clinicas de Porto Alegre)