Nasal Polyposis in mucopolysaccharidosis type II

被引:0
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作者
Kabekkodu, Sushmitha [1 ]
Sreedharan, Suja [2 ]
Gupta, Kirti [3 ]
Murali, Nirupama [4 ]
机构
[1] Manipal Acad Higher Educt, Kasturba Med Coll Mangalore, Otorhinolaryngol, Mangalore, Karnataka, India
[2] Manipal Acad Highe Educ, Kasturba Med Coll Mangalore, Otorhinolaryngol, Mangalore, Karnataka, India
[3] Post Grad Inst Med Educ & Res, Histopathol, Chandigarh, India
[4] Manipal Acad Higher Educ, Kasturba Med Coll Mangalore, Pathol, Mangalore, Karnataka, India
关键词
ear; nose and throat; nasal polyps; pathology; otolaryngology; ENT; genetics;
D O I
10.1136/bcr-2020-238778
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Mucopolysaccharidosis (MPS) type II is a rare multisystem disorder resulting from the accumulation of breakdown products of glycosaminoglycans in the body tissues. Many patients with this disease undergo ENT (ear, nose and throat) surgeries such as adenotonsillectomy and tympanocentesis at a very early age, much before the diagnosis of MPS. Nasal polyposis is a rare occurrence, with only one case of MPS II with polyposis reported in the literature. We present a patient who presented with recurrent nasal polyposis from the age of 2 years. Hale's colloidal iron was used to stain these 'nasal polyps', which revealed that they are, in fact, mucopolysaccharide-laden sinonasal mucosa prolapsing into the nasal cavities. We believe this is the first time that this stain has been used to stain nasal polyps in MPS. In addition to the histopathological peculiarities of these nasal masses, we also discuss the natural history of nasal polyposis in MPS II.
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页数:4
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