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The management of paediatric acute liver failure: results from an international multi-centre survey across Europe

  • Emma C Alexander,
  • Mikkel Malham,
  • Dominic Lenz,
  • Serge Grazioli,
  • Marianne Hørby Jørgensen,
  • Akash Deep

摘要

Background

Paediatric acute liver failure (PALF) is a rare condition with high morbidity and mortality. The liver transplant era, and improvements in medical management, have transformed outcomes. However, differences in aetiology, management, experience and resources are reported across single-centre case series. This study aimed to survey current practices in the critical care of children with PALF across Europe.

Methods

Cross-sectional survey of PICUs in European countries was created and distributed by the Liver Failure Working Group of the European Society of Paediatric and Neonatal Intensive Care (ESPNIC) and ERN Rare-Liver. Participants were intensivists and hepatologists across PICUs in Europe. The survey was distributed using the EUSurvey platform April-June 2023.

Results

25 centres from 16 countries participated (response rate 66% of 38 relevant centres). Most centres (56%, n = 14) managed 1–6 cases of PALF per year. Therapeutic strategies varied across the participating centres. Overall, 80% (n = 20) routinely prescribed antibiotics. Invasive intracranial pressure monitoring was practiced by 20% (n = 5) of centres; cerebral perfusion pressure was targeted by 68% of centres (n = 17). All centres used renal replacement therapy (88% for hyperammonaemia, 50% hepatic encephalopathy); 64% (n = 16) made use of plasma exchange. Most centres used the King’s College Hospital Criteria (68%, n = 17) for listing.

Conclusions

This survey across Europe highlighted varied aetiologies in different settings, and variations in key aspects of management, for patients with PALF. International collaboration is needed to standardise guidelines to improve equity of access to treatment, and to foster research collaborations to improve outcomes, for this high-risk patient group.