Characterization of otorhinolaryngologic manifestations in children with mucopolysaccharide storage disease type Ⅰ and type Ⅱ
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 a-bout 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 pa-tients 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.
mucopolysaccharidosclinical manifestationupper airway obstructionrecurrent respiratory in-fectionshearing loss
NETL
NSTL
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