Otolaryngological findings in mucopolysaccharidosis

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dc.authoridBAYAR MULUK, NURAY/0000-0003-3602-9289
dc.contributor.authorCingi, Cemal
dc.contributor.authorMuluk, Nuray Bayar
dc.contributor.authorHanci, Deniz
dc.contributor.authorSahin, Ethem
dc.contributor.authorAcar, Mustafa
dc.date.accessioned2025-01-21T16:34:15Z
dc.date.available2025-01-21T16:34:15Z
dc.date.issued2014
dc.departmentKırıkkale Üniversitesi
dc.description.abstractIn this review paper, we reported otolaryngological problems in patients with mucopolysaccharidoses (MPSs). Mucopolysaccharidoses are a group of lysosomal storage diseases, each of which is produced by an inherited deficiency of an enzyme involved in the degradation of acid mucopolysaccharides, now called glycosaminoglycans (GAGs). The mucopolysaccharidoses consist of a group of 7 metabolic disorders, known as mucopolysaccharidoses types I-VII. In all groups, there are clinical and otolaryngological manifestations. In MPS patients, upper airway obstruction, obstructive sleep apnea, restriction of mouth opening, middle ear effusion, hearing and breathing problems, etc. are reported as common otolaryngological findings. Increasing awareness of MPS's among ENT doctors will be a life saving attempt for MPS suspected patients who admit an ENT doctor rather than a pediatrician. In MPS patients, tracheotomy may be difficult due to short neck. Due to mouth opening restriction, patients should be evaluated carefully before tonsillectomy and adenoidectomy operations. Airway problems must be evaluated before anesthesia. All ENT doctors should be noticed to be aware of these problems.
dc.description.sponsorshipContinuous Education and Scientific Research Association
dc.description.sponsorshipThe preparation of this paper, including design and planning, was supported by the Continuous Education and Scientific Research Association.
dc.identifier.doi10.2399/jmu.2014003001
dc.identifier.endpage129
dc.identifier.issn2149-7109
dc.identifier.issn2149-6498
dc.identifier.issue3
dc.identifier.startpage122
dc.identifier.trdizinid189164
dc.identifier.urihttps://doi.org/10.2399/jmu.2014003001
dc.identifier.urihttps://search.trdizin.gov.tr/tr/yayin/detay189164
dc.identifier.urihttps://hdl.handle.net/20.500.12587/23924
dc.identifier.volume4
dc.identifier.wosWOS:000219467600008
dc.identifier.wosqualityN/A
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakTR-Dizin
dc.language.isoen
dc.publisherDeomed Publ, Istanbul
dc.relation.ispartofEnt Updates
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanı
dc.rightsinfo:eu-repo/semantics/openAccess
dc.snmzKA_20241229
dc.subjectMucopolysaccharidoses (MPSs); lysosomal storage diseases; short neck; mouth opening restriction; airway problems
dc.titleOtolaryngological findings in mucopolysaccharidosis
dc.typeReview Article

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