Shinhar Shai Y, Zablocki Hedy, Madgy David N
Department of Pediatric Otolaryngology, Children's Hospital of Michigan, Detroit 48201, USA.
Arch Otolaryngol Head Neck Surg. 2004 Feb;130(2):233-7. doi: 10.1001/archotol.130.2.233.
Mucopolysaccharidoses (MPS), a group of disorders caused by a genetic disruption, create a special challenge for the otolaryngologist. With the rare types of MPS IV and MPS 1 S, a skilled practitioner is required to abate airway management complications. The erratic deposits of mucopolysaccharides throughout the trachea should be taken into account when decisions to stent the airway are made. Proper management requires to provide an airway that is custom-made to meet the patient's needs. This is a case-by-case presentation of 3 patients with MPS who presented to the Children's Hospital of Michigan with progressive respiratory embarrassment. Discussed are the various issues revolving around our ability to provide proper airway management, from intubation to tracheostomy tube placement.
黏多糖贮积症（MPS）是一组由基因破坏引起的疾病，给耳鼻喉科医生带来了特殊挑战。对于罕见类型的MPS IV和MPS 1 S，需要熟练的从业者来减少气道管理并发症。在决定是否对气道进行支架置入时，应考虑到气管内黏多糖的不规则沉积。正确的管理需要提供一个定制的气道以满足患者的需求。本文逐案介绍了3例患有MPS的患者，他们因进行性呼吸窘迫就诊于密歇根儿童医院。讨论了围绕我们提供适当气道管理能力的各种问题，从插管到气管造口管置入。
