Abstract:
A 19-year-old woman with known maple syrup urine disease presented to hospital with metabolic crisis in the setting of influenza type A infection and intractable vomiting, rapidly progressing to acute cerebral oedema manifesting as refractory seizures and decreased level of consciousness needing emergency intubation and mechanical ventilation, continuous veno-venous haemodiafiltration and thiopentone coma. A computed tomography scan and magnetic resonance imaging of the brain demonstrated classic signs of cerebral oedema secondary to a metabolic crisis from the metabolic disorder. Her management posed multiple challenges to all teams involved due to lack of familiarity and experience in managing this clinical scenario in the adult intensive care setting.
摘要:
一名19岁女性,已知患有枫糖浆尿病,因甲型流感感染和顽固性呕吐而出现代谢危机,迅速发展为急性脑水肿,表现为难治性癫痫发作和意识水平下降,需要紧急插管和机械通气,连续静脉-静脉血液透析滤过和硫喷戊酮昏迷。大脑的计算机断层扫描扫描和磁共振成像显示了由代谢紊乱引起的代谢危机继发的脑水肿的经典迹象。由于缺乏在成人重症监护环境中管理这种临床方案的熟悉程度和经验,她的管理给所有相关团队带来了多重挑战。