Maple syrup urine disease (MSUD) is due to biallelic variants in one of the three genes: BCKDHA, BCKDHB, and DBT. Branched-chain alpha-ketoacid dehydrogenase complex deficiency and elevated leucine, valine, isoleucine and alloisoleucine in body fluids are the results. We report hyperleucinosis during intercurrent illnesses in six patients with MSUD post liver transplantation. Patient charts were retrospectively reviewed. Data was entered into an Excel Database. Literature was reviewed. Six patients with MSUD were included who had post liver transplantation hyperleucinosis during an intercurrent illness. Five had encephalopathy. One received hemodialysis for the management of hyperleucinosis. All patients had unrestricted diet. Additionally, there were five patients (one patient included into the current study) reported in the literature. We suggested management considerations for the follow-up of patients with MSUD post liver transplantation after the first episode of unexplained encephalopathy or signs of acute hyperleucinosis during intercurrent illness due to our clinical experience: 1) Healthy: Unrestricted diet and monitoring of leucine levels; 2) Illness: a) home illness management: increased carbohydrate intake b) illness management at hospital: intravenous dextrose, intravenous lipid and daily plasma amino acid monitoring. We report hyperleucinosis and/or encephalopathy as a rare event post liver transplantation in MSUD as a multicenter case series. Hyperleucinosis and/or encephalopathy may occur in both related and unrelated donor liver transplantation. Based on the long-term follow-up of those patients, these suggested management considerations may be revised as per the patients' needs.

枫糖浆尿病（MSUD）是由于BCKDHA，BCKDHB和DBT这三个基因之一的双等位基因变异引起的。结果是支链α-酮酸脱氢酶复合物缺乏和体液中亮氨酸，缬氨酸，异亮氨酸和别异亮氨酸升高。我们报告了肝移植后六名MSUD患者在并发疾病期间出现高白细胞增多症。回顾性检查患者病历。数据已输入到Excel数据库中。文献进行了审查。纳入了6例MSUD患者，他们在并发疾病期间发生肝移植后高白细胞增多症。五人患有脑病。其中一名接受血液透析治疗高白蛋白血症。所有患者饮食均不受限制。此外，文献中报道了五例患者（本研究包括一名患者）。由于我们的临床经验，我们建议管理上的考虑因素，用于在首次原因不明的脑病发作或并发疾病期间出现急性高白蛋白病征象后，对肝移植后MSUD患者进行随访：1）健康：饮食无限制并监测亮氨酸水平；2）疾病：a）家庭疾病管理：增加碳水化合物的摄入b）医院疾病管理：静脉葡萄糖，静脉血脂和每日血浆氨基酸监测。我们报告多发性硬化症中MSUD肝移植后罕见的事件是白细胞增生和/或脑病。相关和不相关的供体肝移植均可能发生白细胞增生和/或脑病。根据对这些患者的长期随访，