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Kazakova V.A.

National Medical Research Center for Otorhinolaryngology;
Society of Specialists in Pediatric Otorhinolaryngology and Head and Neck Surgery

Polyakov D.P.

National Medical Research Center for Otorhinolaryngology;
Society of Specialists in Pediatric Otorhinolaryngology and Head and Neck Surgery;
Pirogov Russian National Research Medical University

Yunusov A.S.

National Medical Research Center for Otorhinolaryngology;
Society of Specialists in Pediatric Otorhinolaryngology and Head and Neck Surgery;
Pirogov Russian National Research Medical University

Kiyan T.A.

N.P. Bochkov Medical and Genetic Scientific Center;
Research Clinical Institute of Childhood

Sinonasal pathology in patients with primary ciliary dyskinesia

Authors:

Kazakova V.A., Polyakov D.P., Yunusov A.S., Kiyan T.A.

More about the authors

Journal: Russian Rhinology. 2025;33(3): 223‑232

DOI: 10.17116/rosrino202533031223

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Table of contents

SINONASAL PATHOLOGY IN PATIENTS WITH PRIMARY CILIARY DYSKINESIA
SINONASAL PATHOLOGY IN PATIENTS WITH PRIMARY CILIARY DYSKINESIA

To cite this article:

Kazakova VA, Polyakov DP, Yunusov AS, Kiyan TA. Sinonasal pathology in patients with primary ciliary dyskinesia. Russian Rhinology. 2025;33(3):223‑232. (In Russ.)
https://doi.org/10.17116/rosrino202533031223

Подписка 2026 Российская ринология (Russian Rhinology)
 
МСФ на ЯМ
Использование инфографики авторами научных работ
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Abstract:

Primary ciliary dyskinesia (PCD) is a little-studied polygenic pathology from the group of ciliopathies, characterized mainly by the failure of the entire respiratory tract and requiring a multidisciplinary approach. Often the first symptoms of the disease are related to pathological changes in the nasal cavity and paranasal sinuses (PNSs). The most common manifestation of the upper respiratory tract (URT) pathology in PCD is chronic rhinosinusitis (CRS) with polyps and without them. There is a direct relationship between the condition of the PNSs and lungs due to the downstream contamination with the pathogenic microflora, that has an influence on the course and prognosis of the disease in general. The low degree of awareness of otorhinolaryngologists about this genetic nosology leads to a lack of appropriate suspicion and adequate diagnostic search.

OBJECTIVE

To analyze and generalize results of studies on the pathology of the nasal cavity and PNSs in children and adults suffering from PCD.

MATERIAL AND METHODS

The review includes publications from PubMed database and eLIBRARY.RU scientific electronic library for the period from 2007 to 2024, which provide relevant information on clinical picture, diagnosis and treatment of sinonasal pathology in patients with PCD.

RESULTS AND CONCLUSION

The review of available literature on sinonasal pathology in patients suffering from PCD has shown that the available information is insufficient and of low confidence, based on short-term and often single-arm studies with small samples. According to the majority of scientists, CRS is the main manifestation of URT failure in patients with PCD, thus PNSs are becoming a reservoir of bacterial flora. The results of bacteriological study of the nasal cavity and PNSs discharge have demonstrated dependence on the biomaterial sampling method and direct relationship with the microbiological status of the lower respiratory tract (LRT). Characteristic radiographic signs of changes in the PNSs in PCD are described. A combination of endoscopic surgery of the PNSs with adjuvant therapy has shown good results in the treatment of severe forms of CRS in patients with PCD. According to a number of scientific studies, early diagnosis and treatment of CRSs in patients with PCD correlate with lower prevalence of Pseudomonas aeruginosa, reducing the probability of the PNSs colonization, that further prevents pulmonary dysfunction and bronchiectasis formation, predetermining a relatively favorable outcome.

Keywords:

primary ciliary dyskinesia
Kartagener syndrome
chronic rhinosinusitis
chronic rhinosinusitis with nasal polyps
cystic fibrosis

Authors:

Kazakova V.A.

National Medical Research Center for Otorhinolaryngology;
Society of Specialists in Pediatric Otorhinolaryngology and Head and Neck Surgery

Polyakov D.P.

National Medical Research Center for Otorhinolaryngology;
Society of Specialists in Pediatric Otorhinolaryngology and Head and Neck Surgery;
Pirogov Russian National Research Medical University

Yunusov A.S.

National Medical Research Center for Otorhinolaryngology;
Society of Specialists in Pediatric Otorhinolaryngology and Head and Neck Surgery;
Pirogov Russian National Research Medical University

Kiyan T.A.

N.P. Bochkov Medical and Genetic Scientific Center;
Research Clinical Institute of Childhood

Received:

06.04.2024

Accepted:

05.09.2024

Close metadata

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