End-stage liver disease in children

Abstract

The treatment of children with end-stage liver disease involves the coordinated management of nutritional deficiencies, ascites, pruritus, encephalopathy, and portal hypertension. The implementation of management strategies depends upon a parent or guardian to administer the plan in the context of a child at different stages of developmental, physiologic, emotional, and physical maturity. Fat-soluble vitamins (A, D, E, and K) and micronutrient levels should be monitored routinely and supplemented if deficient. In some patients, supplemental nutrition to provide additional energy and protein is needed to ensure optimal growth and development. Ascites often respond to spironolactone and sodium restriction, but may require the addition of a loop diuretic or even abdominal paracentesis. Pruritus significantly impairs the quality of life of patients and is typically treated with ursodeoxycholic acid, rifampin, or an antihistamine. Partial biliary diversion, or liver transplant in some instances, is necessary for patients with self-mutilating pruritus that results from intrahepatic cholestasis. Hepatic encephalopathy is poorly defined in infants and small children. Elevated serum ammonia serves as a surrogate marker for encephalopathy, which is treated with dietary protein restriction and lactulose. The usefulness of medical prophylaxis for esophageal varices has been noted in adults, though such studies have not been performed in children. If variceal bleeding becomes problematic, treatment with endoscopic variceal banding or sclerotherapy is indicated. A surgical shunt to reduce portal pressure is needed in some cases. Orthotopic liver transplant ultimately may be necessary to overcome the unrelenting consequences of end-stage liver disease.

Evaluation of the pediatric patient for liver transplantation: 2014 practice guideline by the american association for the study of liver diseases, american society of transplantation and the north american society for pediatric gastroenterology, hepatolo

“…Complications associated with endstage liver disease include ascites, pruritus, portal hypertension, malnutrition, vitamin deficiencies, and delayed growth and development . In cirrhosis patients, accumulation of ascites is a result of portal hypertension, vasodilatation, and hyperaldosteronism .…”

Evaluation of the Pediatric Patient for Liver Transplantation

“…Complications associated with end‐stage liver disease include ascites, pruritus, portal hypertension, malnutrition, vitamin deficiencies, and delayed growth and development (10). In patients with cirrhosis, accumulation of ascites is a result of portal hypertension, vasodilatation, and hyperaldosteronism (11).…”

Profile of hepatic encephalopathy in children with cirrhosis and response to lactulose

“…According to one classification HE in children is defined as: stage I, inconsolable crying; stage II, inconsolable crying and inattention to task; stage III, somnolence, stupor, combativeness; stage IV, comatose, arousal with painful stimuli (IVa) or no response (IVb). [15] However, in our cohort we used West-Haven criteria for assessment of HE as it is still widely used for HE. Serum ammonia remains the surrogate marker for encephalopathy, but altered ammonia metabolism is only a component of the complex nature of this clinical entity.…”