The combination of recurrent otitis media and adenotomy in early childhood as diagnostic marker of mucopolysaccharidosis type II (Hunter syndrome)

Kuznetsova N.E.

Tyumen State Medical University;
Regional Clinical Hospital No. 2

Kuznetsova T.B.

Tyumen State Medical University

The combination of recurrent otitis media and adenotomy in early childhood as diagnostic marker of mucopolysaccharidosis type II (Hunter syndrome)

Authors:

Kuznetsova N.E., Kuznetsova T.B.

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Journal: Russian Bulletin of Otorhinolaryngology. 2022;87(4): 19‑22

DOI: 10.17116/otorino20228704119

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To cite this article:

Kuznetsova NE, Kuznetsova TB. The combination of recurrent otitis media and adenotomy in early childhood as diagnostic marker of mucopolysaccharidosis type II (Hunter syndrome). Russian Bulletin of Otorhinolaryngology. 2022;87(4):19‑22. (In Russ.)
https://doi.org/10.17116/otorino20228704119

Подписка 2025 (Russian Bulletin of Otorhinolaryngology)

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Mucopolysaccharidoses are a group of rare lysosomal accumulation diseases caused by a deficiency of the lysosomal enzyme and the accumulation of mucopolysaccharides in various organs and tissues. Children with mucopolysaccharidosis type II (Hunter syndrome) develop multisystem dysfunction, including severe airway obstruction. At the same time, 34% of patients already at an early age (2-3 years) undergo surgical manipulations related to ENT organs (tonsillectomy, adenotomy). The article describes a clinical case of diagnosis of type II mucopolysaccharidosis by a pediatric otorhinolaryngologist. The main manifestations of the disease are discussed in detail, including the presence of indications for adenotomy at the age of 2 years, episodes of otitis media, which served as diagnostic markers for suspected orphan disease mucopolysaccharidosis type II. The leading role of the pediatric otorhinolaryngologist in the early diagnosis of the rare disease mucopolysaccharidosis type II is substantiated.

Keywords:

mucopolysaccharidosis type II (Hunter syndrome)

otorhinolaryngologist

ear

throat

nose diseases

early age

adenotomy

Authors:

Kuznetsova N.E.

Tyumen State Medical University;
Regional Clinical Hospital No. 2

Kuznetsova T.B.

Tyumen State Medical University

Received:

09.12.2021

Accepted:

11.02.2022

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