TY - JOUR
T1 - Nasal Polyposis in mucopolysaccharidosis type II
AU - Kabekkodu, Sushmitha
AU - Sreedharan, Suja
AU - Gupta, Kirti
AU - Murali, Nirupama
N1 - Funding Information:
The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Publisher Copyright:
© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.
Copyright:
Copyright 2021 Elsevier B.V., All rights reserved.
PY - 2021/3/5
Y1 - 2021/3/5
N2 - 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 â € 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.
AB - 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 â € 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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U2 - 10.1136/bcr-2020-238778
DO - 10.1136/bcr-2020-238778
M3 - Article
AN - SCOPUS:85102209216
SN - 1757-790X
VL - 14
JO - BMJ Case Reports
JF - BMJ Case Reports
IS - 3
M1 - e238778
ER -