【摘要】 目的:分析黏多糖贮积症(mucopolysaccharidosis, MPS)Ⅰ型和Ⅱ型患儿临床表现的特点，以提高耳鼻喉科医生对该病的认识。方法:回顾性分析55例MPSⅠ型和Ⅱ型患儿的相关临床资料，探讨MPS在耳鼻喉科的临床表现。结果:40例(72.72%)的MPS患者在病程中均至少出现了一种耳鼻喉科症状，其中95%的患者在确诊MPS前出现耳鼻喉科症状；上气道阻塞为最常见的耳鼻喉科症状(34例，85.00%),反复上呼吸道感染次之(23例，57.50%),最后为听力下降(11例，27.50%);26例患者至少接受过1次外科手术，其中15例(57.69%)接受过耳鼻喉科手术，这些患者均在确诊前接受耳鼻喉科手术。最常见的耳鼻喉科手术是腺样体切除术。结论:MPS患者早期临床表现不典型，但耳鼻喉科症状出现早期且普遍，提高耳鼻喉科医生对该病的认识对MPS预后有着积极的意义。更多还原

【Abstract】 Objective: To analyze the characteristics of otorhinolaryngological clinical manifestations in children with Mucopolysaccharide(MPS) type Ⅰ and type II in order to improve the knowledge of otorhinolaryngologists about this disease. Methods: Clinical data related to 55 children with MPS type Ⅰ and type II were retrospectively analyzed to investigate the clinical manifestations of MPS in ENT. Results: All 40 patients(72.72%) with MPS had at least one ENT symptom during the course of the disease, with 95% of them having an ENT symptom prior to the diagnosis of MPS; upper airway obstruction was the most common ENT symptom(34, 85.00%), followed by recurrent upper respiratory tract infections(23, 57.50%), and lastly, hearing loss(11, 27.50%); all 26 patients had undergone at least one surgical procedure, of which 15(57.69%) had undergone ENT surgery, and all of these patients underwent ENT surgery before diagnosis. The most common ENT surgery was adenoidectomy. Conclusion: Early clinical manifestations of MPS patients are atypical, but the early and prevalent appearance of otolaryngologic symptoms and increased awareness of the disease among otolaryngologists has a positive impact on the prognosis of MPS.更多还原