Department of Paediatric and Adolescent Medicine.
Department of Surgical Gastroenterology and Transplantation.
J Pediatr Gastroenterol Nutr. 2021 Jun 1;72(6):815-819. doi: 10.1097/MPG.0000000000003108.
Paediatric acute liver failure (P-ALF) is a rare condition and is associated with a high mortality rate. Management of P-ALF aims to stabilise vital organ functions and to remove circulating toxins and provide vital plasma factors that are lacking. High-volume plasmapheresis (HVP) removes protein-bound substances and improves survival in adult ALF. It is unknown if this effect can be extrapolated to P-ALF. The aim of this study is to report the safety and feasibility of HVP in P-ALF.
Children with P-ALF were offered HVP if bilirubin was higher than 200 μmol/L or if the aetiology was toxic hepatitis. HVP was performed with fresh frozen plasma corresponding to 10% of the body weight on a minimum of 3 consecutive days. Diagnostics, biochemical and clinical data during HVP as well as outcome data after 3 months were collected from 2012 to 2019 and retrospectively analysed.
Sixteen children were treated by HVP and completed at least one series of three treatment sessions with HVP. The only complication seen was an increase in pH > 7.55 in three children within the first 12 hours and was corrected with hydrochloric acid. No bleeding or septic episodes were noted during HVP. Eight children survived without liver transplantation, two survived after successful grafting and a total of six children died. The liver injury unit score between survivors with their own liver and the rest, the two groups was significantly different (P = 0.005).
HVP with fresh frozen plasma is feasible and well tolerated in children with P-ALF. No serious adverse events and no procedure-related mortality were observed.
小儿暴发性肝衰竭（P-ALF）是一种罕见的疾病，其死亡率较高。P-ALF 的治疗旨在稳定重要器官的功能，清除循环毒素，并提供缺乏的重要血浆因子。大剂量血浆置换（HVP）可去除蛋白结合物质，提高成人 ALF 的生存率。尚不清楚这种效果是否可以外推到 P-ALF。本研究旨在报告 HVP 在 P-ALF 中的安全性和可行性。
如果胆红素高于 200μmol/L 或病因是中毒性肝炎，P-ALF 患儿可接受 HVP。HVP 采用与体重 10%对应的新鲜冷冻血浆进行，连续至少 3 天进行 3 次治疗。从 2012 年至 2019 年，收集 HVP 期间的诊断、生化和临床数据以及 3 个月后的预后数据，并进行回顾性分析。
16 名儿童接受 HVP 治疗，至少完成了 3 个连续治疗系列中的 1 个。仅观察到 3 名儿童在最初 12 小时内 pH 值增加超过 7.55，并用盐酸纠正。在 HVP 期间没有出血或脓毒症发作。8 名儿童在未进行肝移植的情况下存活，2 名儿童在成功移植后存活，共有 6 名儿童死亡。存活者（自身肝脏）与其余儿童之间的肝损伤单位评分有显著差异（P=0.005）。
在 P-ALF 儿童中，使用新鲜冷冻血浆的 HVP 是可行且耐受良好的。未观察到严重不良事件和与治疗相关的死亡率。
