Department of Paediatric Child and Health, Aga Khan University Hospital, Karachi, Pakistan.
J Pak Med Assoc. 2024 Sep;74(9):1707-1710. doi: 10.47391/JPMA.10331.
Paediatric acute liver failure (PALF) is a rare yet severe condition that is associated with high mortality. Apart from liver transplant, no specific therapy exists, particularly in developing countries. Evidence suggests that removal of damage-associated molecular patterns, cytokines, toxins, and other metabolites that accumulate due to impaired liver function can enhance natural recovery. Plasmapheresis can be used to remove these products; however, there is limited evidence to support this approach. This case series discusses three critically ill patients with acute liver failure who underwent plasmapheresis. The patients included a seven-year-old boy (Case 1), a 17-year-old boy (Case 2), and a 16-monthold boy (Case 3). Two patients showed significant improvement in bilirubin level, coagulation profile, inotropes requirement, and Glasgow coma scale score. Unfortunately, one patient with PALF, complicated with multi-organ dysfunction, died due to refractory shock on the fourth day of hospitalisation. Our findings illustrate that early use of therapeutic plasmapheresis in PALF can lead to improvement in clinical outcome. It may serve as a bridging therapy for liver transplant and for the spontaneous regeneration of the patient's liver.
儿科急性肝衰竭（PALF）是一种罕见但严重的疾病，其死亡率很高。除肝移植外，目前尚无特定的治疗方法，特别是在发展中国家。有证据表明，清除由于肝功能受损而积聚的损伤相关分子模式、细胞因子、毒素和其他代谢物可以促进自然恢复。可以使用血浆置换来清除这些产物；然而，支持这种方法的证据有限。本病例系列讨论了 3 例接受血浆置换的急性肝衰竭重症患者。患者包括一名 7 岁男孩（病例 1）、一名 17 岁男孩（病例 2）和一名 16 个月大的男孩（病例 3）。两名患者的胆红素水平、凝血谱、正性肌力药需求和格拉斯哥昏迷评分均有显著改善。不幸的是，1 例并发多器官功能障碍的 PALF 患者因入院第 4 天难治性休克而死亡。我们的发现表明，在 PALF 中早期使用治疗性血浆置换可改善临床结局。它可以作为肝移植和患者肝脏自然再生的桥接治疗。
