Growth and Development in Chronic Liver Disease

Norberto Rodriguez-Baez; Karen I. Wayman; Kenneth L. Cox

doi:10.1542/neo.2-9-e211

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Objectives

After completing this article, readers should be able to:

Identify the two diseases emerging in the neonatal period that account for more than 50% of all pediatric liver transplantations completed each year.
List the factors that affect growth in infants who have chronic liver disease.
Delineate the factors affecting growth after liver transplantation.
Identify factors that are associated with poor developmental outcome among children who undergo liver transplantation.

Introduction

Liver transplantation currently is the definitive therapy for various forms of end-stage liver disease in children. Each year, 400 to 600 children are diagnosed with a liver disease that requires transplantation. Such disease may be the result of metabolic (eg, alpha-1-antitrypsin deficiency), structural (eg, biliary atresia), obstructive (eg, hepatocellular disease), infectious (eg, hepatitis), or toxic (eg, drug overdose) causes. All of these conditions can progress to cirrhosis and end-stage liver disease. The incidence of neonatal liver disease is estimated as 1 in 2,500 live births. Two diseases emerging in the neonatal period account for 80% of the pediatric diagnoses: biliary atresia (50%) and neonatal hepatitis (30%). Consequently, children 1 year of age and younger account for approximately 50% of all pediatric transplantations completed in the United States each year (United Network for Organ Sharing, 2001).

Managing chronic liver disease in infants and children is a challenge because of the multiple complications that can result from the damaged liver. Among the complications of end-stage cirrhotic liver disease are growth failure and developmental deficits.

Growth Failure and Liver Disease

Failure to grow is a serious consequence of chronic liver disease that increases morbidity and mortality. A number of factors affect linear growth in infancy, including nutritional status, underlying liver diagnosis and alterations in the growth hormone (GH)/insulin-like growth factor (IGF)/insulin-like growth factor binding proteins (IGFBP) axis. The prevalence of malnutrition associated with chronic liver disease varies according to the …