treatment of hepatic encephalopathy in pediatrics
Ensure a quiet environment and minimize routine tracheal suctioning for intubated patients. Elevate the head of the bed to 30° and address potential contributors to altered mental status, such as sepsis, electrolyte abnormalities, hypoglycemia, and sedative medications. Implement precautions for patient agitation, like using bed rail pads.
Consider limiting protein intake to 1 g/kg/day to reduce ammonia production, though the efficacy of specialized enteral formulas is uncertain.
In cases of hyperammonemia-associated progressive hepatic encephalopathy, initiate lactulose at 0.5 mL/kg per dose, up to 30 mL per dose, given every two hours. Adjust the dose to achieve two to three soft stools daily. Lactulose is not recommended for patients without hyperammonemia. If lactulose is inadequate, consider rifaximin as a second-line treatment for bowel decontamination.
Continuous renal replacement therapy has been used successfully to remove ammonia and/or manage fluid balance in adults and children with ALF. In children, observe a significant improvement in survival with a reduction in ammonia within 48 hours of therapy initiation, making this modality a consideration if other treatments are unsuccessful.
Ref:
- Squires JE, Alonso EM, Ibrahim SH, Kasper V, Kehar M, Martinez M, Squires RH. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Position Paper on the Diagnosis and Management of Pediatric Acute Liver Failure. J Pediatr Gastroenterol Nutr. 2022 Jan 1;74(1):138-158. doi: 10.1097/MPG.0000000000003268. PMID: 34347674.
- Debray D, Yousef N, Durand P. New management options for end-stage chronic liver disease and acute liver failure: potential for pediatric patients. Paediatr Drugs. 2006;8(1):1-13. doi: 10.2165/00148581-200608010-00001. PMID: 16494508.
- Cardoso FS, Gottfried M, Tujios S, Olson JC, Karvellas CJ; US Acute Liver Failure Study Group. Continuous renal replacement therapy is associated with reduced serum ammonia levels and mortality in acute liver failure. Hepatology. 2018 Feb;67(2):711-720. doi: 10.1002/hep.29488. Epub 2017 Dec 26. PMID: 28859230; PMCID: PMC5832542.
- Deep A, Stewart CE, Dhawan A, Douiri A. Effect of Continuous Renal Replacement Therapy on Outcome in Pediatric Acute Liver Failure. Crit Care Med. 2016 Oct;44(10):1910-9. doi: 10.1097/CCM.0000000000001826. PMID: 27347761.
Because of the life-threatening and complex nature of this condition, management must be carried out in an intensive care unit at a liver transplant center. Treatment of acute liver failure is supportive; the definitive lifesaving therapy is liver transplantation. Supportive measures are listed in this Table . Efforts are made to treat metabolic derangements, avoid hypoglycemia, support respiration, minimize hepatic encephalopathy, and support renal function.
Ref: Nelson Essentials of pediatrics 2023 9th EDition.
