End-stage liver disease in infants and children presents a number of medical and psychosocial challenges to the clinical care team. While many of these complications are similar to those in adults with end-stage liver disease, infants and children with end-stage liver disease have unique characteristics requiring special attention by healthcare providers. A wide variety of liver diseases may progress to cirrhosis, a final common histologic pathway characterized by diffuse fibrosis and conversion of the normal liver architecture into structurally abnormal nodules (Fig. 420-1). However, there is often a poor correlation between histology and the clinical status of the patient. Some patients with cirrhosis are essentially asymptomatic (compensated cirrhosis), whereas others have all of the sequelae of chronic liver disease (decompensated cirrhosis) discussed below.
Cirrhosis with architectural alteration resulting from fibrosis and nodular hepatocellular regeneration (Masson trichrome, 2×). (Reproduced with permission from Fauci AS, Braunwald E, Kasper DL et al: Harrison’s Principles of Internal Medicine, 17th ed. New York: McGraw-Hill Companies; 2008.)
The etiology of end-stage liver disease in children varies with age of presentation (Table 420-1). Critical considerations for optimizing the clinical care and outcomes of children with end-stage liver disease due to a chronic liver condition include attending to (1) nutritional status focusing on the challenges of adequate intake of calories, protein, vitamins, and nutrient status; (2) completion of childhood immunizations whenever possible; (3) management of the potential complications of cirrhosis including variceal bleeding, ascites, spontaneous bacterial peritonitis (SBP), and hepatic encephalopathy; (4) assessment for involvement of extrahepatic organs including the kidney, lungs, brain, and bones; and (5) addressing the unique psychosocial needs of the child with a chronic medical condition, which are distinct from the adult experience. To address these challenges and optimize the pediatric patient’s long-term clinical outcomes, the care of infants, children, and adolescents with end-stage liver disease should ideally be conducted at a pediatric institution with an integrated multidisciplinary approach. The goals of surveillance and follow-up of the child with end-stage liver disease are to maintain stability and prevent complications as long as possible, to detect the earliest signs of decompensation in order to ameliorate some of these chronic complications, to address the multidimensional needs of this distinct patient population, and to expedite referral to a pediatric liver transplant program as soon as needed.
Table 420-1MOST COMMON CAUSES OF END-STAGE LIVER DISEASE IN CHILDREN |Favorite Table|Download (.pdf) Table 420-1MOST COMMON CAUSES OF END-STAGE LIVER DISEASE IN CHILDREN
|Infants ||Older Children and Adolescents |
Genetic causes of intrahepatic cholestasis syndromes
Parental nutrition–associated cholestatic liver disease
Metabolic liver conditions
Glycogen storage disease
Autoimmune liver diseases
Metabolic liver conditions