Article: Sochetanie retsidiviruyushchego srednego otita i adenotomii v rannem detskom vozraste kak diagnosticheskii marker mukopolisakharidoza II tipa (sindroma Khantera).
2022 Volume 87, Issue 4, Page(s) 19–22
Abstract: 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 ... ...
Title translation | The combination of recurrent otitis media and adenotomy in early childhood as diagnostic marker of mucopolysaccharidosis type II (Hunter syndrome). |
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Abstract | 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. |
MeSH term(s) | Child ; Child, Preschool ; Glycosaminoglycans ; Humans ; Mucopolysaccharidosis II/diagnosis ; Mucopolysaccharidosis II/surgery ; Otitis Media |
Chemical Substances | Glycosaminoglycans |
Language | Russian |
Publishing date | 2022-09-16 |
Publishing country | Russia (Federation) |
Document type | Case Reports ; Journal Article |
ZDB-ID | 124096-1 |
ISSN | 0042-4668 |
ISSN | 0042-4668 |
DOI | 10.17116/otorino20228704119 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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