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小儿肝衰竭的连续性肾脏替代治疗和治疗性血浆置换。

Continuous renal replacement therapy and therapeutic plasma exchange in pediatric liver failure.

机构信息

Division of Nephrology, Department of Pediatrics, Seattle Children's Hospital, University of Washington, Seattle, WA, USA.

Seattle Children's Research Institute, Seattle, WA, USA.

出版信息

Eur J Pediatr. 2024 Aug;183(8):3289-3297. doi: 10.1007/s00431-024-05587-3. Epub 2024 May 8.

Abstract

Patients with acute liver failure (ALF) and acute on chronic liver failure (ACLF) have significant morbidity and mortality. They require extracorporeal blood purification modalities like continuous renal replacement therapy (CRRT) and therapeutic plasma exchange (TPE) as a bridge to recovery or liver transplantation. Limited data are available on the outcomes of patients treated with these therapies. This is a retrospective single-center study of 23 patients from 2015 to 2022 with ALF/ACLF who underwent CRRT and TPE. We aimed to describe the clinical characteristics and outcomes of these patients. Median (IQR) age was 0.93 years (0.57, 9.88), range 16 days to 20 years. Ten (43%) had ALF and 13 (57%) ACLF. Most (n = 19, 82%) started CRRT for hyperammonemia and/or hepatic encephalopathy and all received TPE for refractory coagulopathy. CRRT was started at a median of 2 days from ICU admission, and TPE started on the same day in most. The liver transplant was done in 17 (74%), and 2 recovered native liver function. Four patients, all with ACLF, died prior to ICU discharge without a liver transplant. The median peak ammonia pre-CRRT was 131 µmol/L for the whole cohort. The mean (SD) drop in ammonia after 48 h of CRRT was 95.45 (43.72) µmol/L in those who survived and 69.50 (21.70) µmol/L in those who did not (p 0.26). Those who survived had 0 median co-morbidities compared to 2.5 in non-survivors (aOR (95% CI) for mortality risk of 2.5 (1.1-5.7), p 0.028).  Conclusion: In this cohort of 23 pediatric patients with ALF or ACLF who received CRRT and TPE, 83% survived with a liver transplant or recovered with their native liver. Survival was worse in those who had ACLF and those with co-morbid conditions. What is Known: •  Pediatric acute liver failure is associated with high mortality. •  Patients may require extracorporeal liver assist therapies (like CRRT, TPE, MARS, SPAD) to bridge them over to a transplant or recovery of native liver function. What is New: • Standard volume plasma exhange has not been evaluated against high volume plasma exchange for ALF. • The role, dose, and duration of therapeutic plasma exchange in patients with acute on chronic liver failure is not well described.

摘要

急性肝衰竭(ALF)和慢加急性肝衰竭(ACLF)患者的发病率和死亡率均较高。他们需要体外血液净化方式,如连续肾脏替代疗法(CRRT)和治疗性血浆置换(TPE),以作为恢复或肝移植的桥梁。目前关于接受这些治疗的患者结局的数据有限。这是一项回顾性单中心研究,纳入了 2015 年至 2022 年期间 23 名 ALF/ACLF 患者,这些患者接受了 CRRT 和 TPE 治疗。我们旨在描述这些患者的临床特征和结局。中位(IQR)年龄为 0.93 岁(0.57,9.88),范围为 16 天至 20 岁。10 名(43%)为 ALF,13 名(57%)为 ACLF。大多数(n=19,82%)因血氨升高和/或肝性脑病开始接受 CRRT,所有患者均因难治性凝血障碍接受 TPE。CRRT 中位开始于 ICU 入院后 2 天,大多数患者在同一天开始 TPE。17 名(74%)患者接受了肝移植,2 名患者恢复了自身肝功能。4 名患者(均为 ACLF)在未接受肝移植前于 ICU 出院时死亡。整个队列中,CRRT 前氨的峰值中位数为 131 μmol/L。存活者在 48 小时 CRRT 后氨的平均(SD)下降值为 95.45(43.72)μmol/L,而未存活者为 69.50(21.70)μmol/L(p=0.26)。存活者的中位合并症数量为 0 ,而非存活者为 2.5(优势比(95%CI)的死亡率风险为 2.5(1.1-5.7),p=0.028)。结论:在本研究中,23 名患有 ALF 或 ACLF 的儿科患者接受了 CRRT 和 TPE 治疗,83%的患者在接受肝移植后存活,或自身肝功能恢复。ACLF 和合并症患者的存活率更差。已知:•儿科急性肝衰竭与高死亡率相关。•患者可能需要体外肝脏辅助治疗(如 CRRT、TPE、MARS、SPAD),以使其过渡到肝移植或自身肝功能恢复。新内容:•尚未评估标准容量血浆置换与 ALF 的高容量血浆置换相比的效果。•急性加重期肝衰竭患者中治疗性血浆置换的作用、剂量和持续时间尚未明确。

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