Acute liver failure in children presents as a rare, complex, rapidly progressing, and life-threatening illness with a majority of the cases with unknown etiology.  A collaborative multidisciplinary approach involving transplant hepatologists and critical care specialists for the management of patients with Pediatric acute liver failure (PALF) will prove beneficial in improving the prognosis of this fatal disease. 

The use of extensive but systematic diagnostic evaluation is necessary to identify etiology and guide management. 

Management of PALF is challenging for physicians and intensivists but numerous recent advances in intensive care management of PALF have shown promising improvements in outcomes. However, the current management of PALF remains supportive and remains poorly defined due to its rarity and heterogeneity. 

Hyperammonemia - can be corrected by using lactulose and other antibiotics like rifaximin and neomycin OR Ammonia-lowering agents like LOLA and LOPA. Continuous renal replacement therapy can also be rendered.

Hepatic encephalopathy- should be managed with Supportive care in ICU with Minimal stimulation and avoid unnecessary interventions. Endotracheal intubation is recommended especially for stage 3 or 4 encephalopathies. CT/MRI head should be performed for any acute mental status changes.

Intracranial hypertension/cerebral edema - administer hypertonic saline (HTS) (3–30%) to maintain target serum Na level to 145 to 150 mmol/L to prevent ICP and CE. Mannitol 0.25–1.0 g/kg IV bolus can be repeated once or twice if serum osmolality < 320 mOsm/L. 

Cardiovascular instability - should be managed with Adequate fluid resuscitation with IV normal saline. Norepinephrine along with Vasopressin and its analogs should be used as a vasoconstrictor agent for volume-refractory instability.

Acute kidney injury/hepatorenal syndrome - should be prevented by maintaining fluid balance while avoiding excessive diuresis, minimizing the use of nephrotoxic medications or IV contrast, and maintaining renal perfusion pressure. Continuous renal replacement therapy is recommended.

Gastrointestinal bleed - should be managed with H2 blocker or proton pump inhibitors.

Infection/systemic inflammatory response syndrome

(SIRS) - Aggressive surveillance with cultures followed by empiric antibiotics administration should be done in presence of SIRS, worsening encephalopathy, refractory hypotension, or signs of infection

Nutrition, hypoglycemia - Enteral nutrition with high caloric density formula is recommended to avoid excess free water or Parenteral nutrition in patients who cannot be fed enterally. euglycemia should be maintained with Continuous glucose infusion at 10–15 mg/kg/min.

Electrolytes - should be monitored frequently to maintain acid-base balance. hyponatremia should be avoided to prevent cerebral edema.

This aggressive management approach to acute liver failure and related comorbidities will cause increased transplant-free survival and improved post-transplant outcomes, further reducing mortality and morbidity associated with this potentially fatal condition.

Source: Bhatt H, Rao GS. Management of Acute Liver Failure: A Pediatric Perspective. Curr Pediatr Rep,2018; 6: 246–257.