Understanding pediatric acute liver failure (PALF) is of utmost importance in identifying at-risk patients with possible underlying conditions such as Alagille syndrome (ALGS) and initiating proper measures, according to a study recently published in Critical Care Nursing Clinics of North America.
“Timely referral for patients with PALF to a pediatric transplant center is one of the most important goals of care,” the authors wrote.
This review describes the case of a 5-year-old female who presented to the emergency room in a comatose state. She had no relevant prior medical history other than a recent viral infection. Laboratory tests detected liver failure, and the patient was transferred to the pediatric intensive care unit (PICU).
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She rapidly progressed to multisystem organ failure, requiring high-volume plasmapheresis, invasive ventilation, and administration of inotropes. The healthcare team prepared for an emergency liver transplant while still not fully identifying the etiology and mechanisms involved in this rapidly progressing, fulminant clinical picture.
The study aims to highlight that even though PALF is relatively uncommon, quickly recognizing it may determine the chance of survival for these patients. At least 40% of the PALF cases occur without a clear causal agent, and 30% will either require a liver transplant or will die.
Regardless, there are no clear guidelines to help identify which children are at risk of presenting with PALF, which subsequently compromises the prognosis since a transplantation surgery is not easily available in many centers, and transferring the patient under unstable conditions is challenging.
“It is important for nurses to consider this potential diagnosis for patients who present to the PICU with a variety of pediatric disorders and to carefully engage in nursing assessment to identify subtle changes following a system-based evaluation approach,” the study concluded.
About 500 liver transplants occur each year in the United States, most of them due to biliary atresia and nonalcoholic fatty liver disease in children younger than 2 years old, and adolescents, respectively. ALGS is another known entity that often requires this surgery as the preferred therapeutic approach.
Although these rates are alarming, children tend to recover faster from liver failure than adults and may even regain normal liver function upon appropriate treatment administration, highlighting the importance of awareness on this topic for the entire medical team.
Haut C, Flynn L. Acute liver failure in children. Crit Care Nurs Clin North Am. Published online July 20, 2022. doi:10.1016/j.cnc.2022.04.002