pulmoПульмонологияPULMONOLOGIYA0869-01892541-9617Scientific and Practical Journal “PULMONOLOGIYA” LLCpulmo-2846Research ArticleОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯORIGINAL STUDIESУльтраструктурные изменения ресничек при первичной цилиарной дискинезииUltrastructural ciliar lesions in primary ciliar dyskinesiaЭбсенМ.EbsenM.

Бохум

МоргенротК.MorgenrotK.

Бохум

Рурский университетGermany200130122001041923Copyright © Эбсен М., Моргенрот К., 20012001Эбсен М., Моргенрот К.Ebsen M., Morgenrot K.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.pulmonology.ru/pulm/article/view/2846

Первичная цилиарная дискинезия (ПЦЦ) обычно характеризуется ультраструктурными изменениями ресничек. Нами было проведено электронно-микроскопическое исследование ресничек эпителия 99 пациентов с 1990 по 1999 гг. У 23 пациентов была выявлена ПЦД (13 мужчин и 10 женщин). Ультраструктурные изменения чаще были связаны со строением внутренних и наружных денеиновых выступов и радиальных спиц. Нарушение расположения трубочек или потеря центральных трубочек отмечались крайне редко. Данные о частоте и характеристика ультраструктурных изменений ресничек окажут помощь в постановке диагноза и дифференциальной диагностике первичных и вторичных нарушений.

Generally primary ciliar dyskinesia (PCD) is characterized by ultrastructural lesions of cilia. We performed electronic microscopic investigation of epithelium cilia in 99 patients in 1990–1999 years. PCD was found in 23 patients (13 males and 10 females). Ultrastructural lesions were provided more often by structure of internal and external denein ledges and radial spokes. A disorder of tubule’s disposition or losses of central tubule were noted extremely rarely. Information about frequency of ultrastructural ciliar lesions and their characterization will be useful for detection and differential diagnosis of primary and secondary disturbances.

ReferencesBush A., Cole P., Hariri M.et al. Primary ciliary dyskinesia: diagnosis and standards of care. Eur. Respir. J . 1998; 12: 982—988.Bush A., Cole P., Hariri M.et al. Primary ciliary dyskinesia: diagnosis and standards of care. Eur. Respir. J . 1998; 12: 982—988.Dornbi V.H., Walt H. Primäre ziliare Dyskinesie, ’’immotile-cilia syndrome” und Kartagener - Syndrom: diagnostische Kriterien. Schweiz. Med. Wschr. 1996; 126: 421—433.Dornbi V.H., Walt H. Primäre ziliare Dyskinesie, ’’immotile-cilia syndrome” und Kartagener - Syndrom: diagnostische Kriterien. Schweiz. Med. Wschr. 1996; 126: 421—433.Wisseman C.L., S im e l D.L., Spock A., Shelburne J.D. The prevalence of abnormal cilia in normal pediatric lungs. Arch. Pathol. Lab. Med. 1981; 105: 552—555.Wisseman C.L., S im e l D.L., Spock A., Shelburne J.D. The prevalence of abnormal cilia in normal pediatric lungs. Arch. Pathol. Lab. Med. 1981; 105: 552—555.Rossman CM ., Lee R.M.K.W., Forrest J.B., Newhouse M.T. Nasal ciliary ultrastructure and function in patients with primary ciliary dyskinesia compared with that in normal subjects and in subjects with various respiratory diseases. Am. Rev. Respir. Dis. 1984; 129: 161—167.Rossman CM ., Lee R.M.K.W., Forrest J.B., Newhouse M.T. Nasal ciliary ultrastructure and function in patients with primary ciliary dyskinesia compared with that in normal subjects and in subjects with various respiratory diseases. Am. Rev. Respir. Dis. 1984; 129: 161—167.Smallman L.A., Gregory J. Ultrastructural abnormalities of cilia in the human respiratory tract. Hum. Pathol. 1986; 17: 848—855.Smallman L.A., Gregory J. Ultrastructural abnormalities of cilia in the human respiratory tract. Hum. Pathol. 1986; 17: 848—855.Hattory Y., Watanabe Y., O ku d aM . Ultrastructural abnormalities of the cilia in the human nasal epithelia. Nippon Jibi-Inkoka Gakkai Kaiho 1991; 94: 942—962.Hattory Y., Watanabe Y., O ku d aM . Ultrastructural abnormalities of the cilia in the human nasal epithelia. Nippon Jibi-Inkoka Gakkai Kaiho 1991; 94: 942—962.Jorissen M., Cassiman J.J. Relevance of the ciliary ultrastructure in primary ciliary dyskinesia: A review. Am. J. Rhinol. 1991; 5: 91—101.Jorissen M., Cassiman J.J. Relevance of the ciliary ultrastructure in primary ciliary dyskinesia: A review. Am. J. Rhinol. 1991; 5: 91—101.Lurie M., Rennert G., Goldenberg S. et al. Ciliary ultrastructure in primary ciliary dyskinesia and other chronic respiratory conditions: the relevance of microtubular abnormalities. Ultrastruct. Pathol. 1992; 16: 547—553.Lurie M., Rennert G., Goldenberg S. et al. Ciliary ultrastructure in primary ciliary dyskinesia and other chronic respiratory conditions: the relevance of microtubular abnormalities. Ultrastruct. Pathol. 1992; 16: 547—553.R u tla n d J., Dewar A., Сох Т., Cole P.J. Nasal brushing for the study of ciliary ultrastructure. J. Clin. Pathol. 1982; 35: 356—359.R u tla n d J., Dewar A., Сох Т., Cole P.J. Nasal brushing for the study of ciliary ultrastructure. J. Clin. Pathol. 1982; 35: 356—359.Green C.G. Letter to the editor. Pediatr. Pulmonol. 1990; 9: 131.Green C.G. Letter to the editor. Pediatr. Pulmonol. 1990; 9: 131.Trevisani L., Sartori S., Pazzi P. et al. Acquired ciliary defects in bronchial epithelium of patients with chronic bronchitis. Respi-bronchial 1990; 57: 6—13.Trevisani L., Sartori S., Pazzi P. et al. Acquired ciliary defects in bronchial epithelium of patients with chronic bronchitis. Respi-bronchial 1990; 57: 6—13.Verra F., Escudier E., Lebargy F. et at. Ciliary abnormalities in bronchial epithelium of smokers, ex-smokers and nonsmokers. Am. ration 1J. Respir. Crit. Care Med. 1995; 151: 630—634.Verra F., Escudier E., Lebargy F. et at. Ciliary abnormalities in bronchial epithelium of smokers, ex-smokers and nonsmokers. Am. ration 1J. Respir. Crit. Care Med. 1995; 151: 630—634.

The authors declare that there are no conflicts of interest present.