Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Mar 27, 2015; 7(3): 392-405
Published online Mar 27, 2015. doi: 10.4254/wjh.v7.i3.392
Cirrhosis in children and adolescents: An overview
Raquel Borges Pinto, Ana Claudia Reis Schneider, Themis Reverbel da Silveira
Raquel Borges Pinto, Pediatric Gastroenterology Unit, Hospital da Criança Conceição, Porto Alegre 91340 480, Rio Grande do Sul, Brazil
Ana Claudia Reis Schneider, Themis Reverbel da Silveira, Post-Graduate Program in Gastroenterology and Hepatology, Universidade Federal do Rio Grande do Sul, Porto Alegre RS 90040-060, Brazil
Author contributions: All the authors made substantial contributions to conception and design of the article, drafted the article and gave final approval of the version to be published.
Supported by FIPE-HCPA (Research Incentive Fund - Hospital de Clínicas de Porto Alegre).
Conflict-of-interest: The authors declare that there are no conflicts of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Raquel Borges Pinto, MD, PhD, Pediatric Gastroenterology Unit, Hospital da Criança Conceição, Tomaz Gonzaga 900, Porto Alegre 91340 480, Rio Grande do Sul, Brazil.
Telephone: +55-51-99868415 Fax: +55-51-33572313
Received: September 9, 2014
Peer-review started: September 9, 2014
First decision: November 14, 2014
Revised: December 10, 2014
Accepted: January 9, 2015
Article in press: January 9, 2015
Published online: March 27, 2015

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.

Keywords: Cirrhosis, Liver diseases, Nutrition, Pediatric patients, Portal hypertension

Core tip: The investigation and management of pediatric cirrhosis presents several challenges. The etiology of the condition may vary according to patient age. In many cases, cirrhosis is a predictable consequence of the progression of several chronic liver diseases, such as biliary atresia, although it may also be detected when splenomegaly is discovered on routine examination, or during the investigation of conditions such as hypersplenism, anemia, thrombocytopenia, leukopenia, petechiae and/or ecchymosis. The present article will discuss the diagnostic and treatment aspects of cirrhosis in children and adolescents.