Acute-on-chronic liver failure in children

Abstract

Although various complex definitions of acute-on-chronic liver failure (ACLF) have been suggested in relation to adult patients, there is currently no universal definition of the syndrome in pediatric patients. In simplified terms, ACLF is characterized by the acute deterioration of the liver functions due to the effects of a precipitating factor on the basis of a chronic liver disease. Acute events and underlying liver diseases are very different in children from those seen in adults. Moreover, acute events and underlying chronic liver diseases vary among geographical regions, although it seems that the most common such diseases and acute events are autoimmune hepatitis, Wilson’s disease, and their flares. ACLF is associated with a poor prognosis. While no scoring systems have been developed to predict the prognosis for children with ACLF, modified versions of the Asian Pacific Association for the Study of the liver’s acute-on-chronic liver failure scoring system and the Chronic Liver Failure-Sequential Organ Failure Assessment criteria can be used in children until specific and validated scoring systems are available. Aside from liver transplantation, there is no proven treatment for ACLF. Thus, the early recognition of ACLF prior to the development of extrahepatic organ failure is important.

Keywords: Liver failure; Prognosis; Prevalence; Clinics; Histopathology; Scoring systems; Treatment

Core Tip: Acute-on-chronic liver failure (ACLF) remains poorly defined in pediatric patients. ACLF is associated with acute deterioration in patients with chronic liver disease or cirrhosis due to an underlying precipitating event. In the limited number of pediatric studies conducted to date, the underlying chronic diseases and acute precipitating events have been found to vary among geographical regions, while high rates of short-term mortality have also been reported. This review focuses on ACLF in children.