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Multidisciplinary management of children with acute liver failure - Report on 104 children treated in single center
Piotr Kaliciński1, Ryszard Grenda2, Marek Szymczak1
1Department of Pediatric Surgery and Organ Transplantation, The Children's Memorial Health Institute, Warsaw, Poland.
Insights
Pediatric acute liver failure (PALF) is a critical condition requiring expert care. Timely liver transplantation (LT) significantly improves survival rates for children with PALF, despite complex peritransplant challenges.
Area of Science:
- Pediatric Hepatology
- Transplant Surgery
- Intensive Care Medicine
Background:
- Pediatric acute liver failure (PALF) presents a significant challenge in pediatric hepatology, intensive care, and liver transplantation.
- This study reviews clinical patterns, diagnostics, and therapeutics for children with PALF at high risk of mortality without transplantation.
Purpose of the Study:
- To analyze the clinical characteristics and outcomes of children with PALF undergoing liver transplantation.
- To evaluate the effectiveness of diagnostic and therapeutic strategies in managing PALF patients.
Main Methods:
- Retrospective analysis of 104 children with PALF evaluated for liver transplantation (LT) between 1990 and 2022.
- 81 children underwent LT, with detailed data on pre-transplant complications and post-transplant outcomes.
Main Results:
- Of 104 children, 81 (78%) received LT. 15 died awaiting transplant, and 8 recovered spontaneously.
- Pre-transplant complications included circulatory failure (43.3%), mechanical ventilation (63.5%), acute kidney injury (17.3%), and encephalopathy (57.7%).
- Post-transplant mortality was 24.7%, with 18.5% in the early period and 6.1% late mortality.
Conclusions:
- Managing children with PALF peritransplant requires a highly skilled multidisciplinary team.
- Despite advancements, survival for PALF patients post-LT remains lower than for elective cases.
- Prompt qualification and timely liver transplantation are crucial for improving survival in pediatric acute liver failure.
Background:
Pediatric acute liver failure (PALF) is one of the most demanding emergencies in hepatology, intensive care, and for transplant team. This report describes the clinical pattern, diagnostic and therapeutic modalities in children with ALF considered at risk of death without liver transplantation, basing on a long-term experience of the pediatric transplant center.
Materials And Methods:
Between 1990 and 2022, 104 children aged 7 days-17 years (median 8 years), with body weight 3.1 to 77 kg (median 32 kg), were qualified for LT due to ALF, and finally 81 (78%) of them were transplanted (9% of all 899 LT performed in children in the same period).
Results:
A total of 23 children were not transplanted: 15 (14.4%) died while awaiting transplantation. In 8 (7.7%) patients liver function recovered. Before transplantation 45 (43.3%) children developed circulatory failure, in 66 (63.5%) mechanical ventilation was necessary, 18 patients presented acute kidney injury (17.3%), and encephalopathy higher than stage I was present in 60 (57.7%) patients. In 63 children, various kidney/liver assist procedures were performed: CVVHD (continuous veno-venous hemodiafiltration in 22 (21.2%) patients, albumin dialysis (MARS; molecular adsorbent recirculating system) in 39 (37.5%) patients, therapeutic plasma exchange (TPE) in 13 (12.5%) patients. Twenty (24.7%) children died after LT including 15 (18.5%) in the early posttransplant period, and 5 (6.1%) in the late follow-up.
Conclusions:
Treatment of children with ALF in the peritransplant period is very difficult and require an experienced, multidisciplinary team. Despite continued advances in the care of children with ALF, patient survival remains lower than for elective indications for liver transplantation, and timely qualification and transplantation still are the most important factors of survival of these children.
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