PDF(Sci-hub)
Abstract：
Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome is a rare entity that is characterised by its onset in healthy children at 2-4 years of age. It is a complex syndrome that includes, among other symptoms, rapid weight gain with hyperphagia, hypothalamic dysfunction, central hypoventilation, and autonomic dysregulation. The case is presented of a 10-year-old boy with a diagnosis of ROHHAD syndrome undergoing insertion of a port-a-cath under general anaesthesia, who developed complications during the anaesthetic procedure related to his illness. The peri-operative management of these patients represents a challenge for the anaesthetist, given the involvement of multiple systems and the frequent respiratory comorbidities associated with them. A summary is presented of some of the implications and anaesthetic considerations that must be taken into account in the management of these patients.
摘要：
速发性肥胖伴下丘脑功能障碍,通气不足,自主神经失调（ROHHAD）综合征是一种罕见的实体，其特征是在2-4岁的健康儿童中发作。这是一种复杂的综合症，包括，在其他症状中，体重迅速增加，伴有饮食过多，下丘脑功能障碍,中枢通气不足,和自主神经失调.该病例是一个10岁的男孩，诊断为ROHHAD综合征，正在全身麻醉下插入血管口，他在麻醉过程中出现了与他的疾病相关的并发症。这些患者的围手术期管理对麻醉师来说是一个挑战，考虑到多个系统的参与以及与之相关的频繁的呼吸道合并症。总结了在这些患者的管理中必须考虑的一些含义和麻醉考虑因素。
