Chronic Liver Disease in Children

Abstract

Chronic liver disease encompasses a wide spectrum of disorders, including infectious, metabolic, genetic, drug-induced, idiopathic, structural, and autoimmune diseases. The clinical presentation and initial laboratory data in many of these diseases are similar, and a definitive diagnosis often relies on specialized laboratory investigation and histologic examination of liver tissue. The aim of this article is to: 1) Define chronic hepatitis and cirrhosis; 2) Review briefly the clinical presentation, pathophysiology, diagnosis, and management of the more common pediatric causes of chronic liver disease; 3) Review the complications associated with chronic liver disease and discuss their appropriate management; and 4) Review the indications for hepatic transplantation. Chronic Hepatitis Chronic hepatitis is defined as ongoing inflammation within the liver that is capable of progression to cirrhosis, liver failure, and death. The presence of continued hepatic inflammation, as confirmed by clinical manifestations and laboratory studies, for a period of greater than 10 weeks usually excludes a self-limited hepatitis and implies chronicity. However, to fulfill the international criteria for chronic disease strictly, evidence of continued hepatic inflammatory activity needs to be present for a period of at least 6 months. Most pediatricians, however, allow 3 months for an acute liver insult to clear before undertaking specialized laboratory investigations and invasive studies.