pulmo

Пульмонология

PULMONOLOGIYA

0869-01892541-9617

Scientific and Practical Journal “PULMONOLOGIYA” LLC

10.18093/0869-0189-2017-27-6-705-731

pulmo-938

Research Article

КЛИНИЧЕСКИЕ РЕКОМЕНДАЦИИ

CLINICAL GUIDELINES

Клинические рекомендации по диагностике первичной цилиарной дискинезии

Clinical guidelines for diagnosis of primary ciliary dyskinesia

редакционная

статья

Editotial

Article

2017

23022018

276705731

2018

редакционная с.

editotial a.

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/938

Диагноз первичной цилиарной дискинезии (ПЦД) обычно подтверждается с помощью стандартных, но сложных и дорогостоящих исследований. Однако в большинстве случаев диагностика остается трудной, несмотря на множество сложных тестов, при этом «золотой стандарт» диагностики ПЦД отсутствует. В связи с этим Рабочей группой при поддержке Европейского респираторного общества разработаны и опубликованы клинические рекомендации, в которых содержатся доказательные данные о диагностике ПЦД, особенно с учетом новых сведений и необходимости точной диагностики для больных, включенных в рандомизированные клинические исследования. В клинических рекомендациях освещены отдельные вопросы клинического ведения лиц с ПЦД, содержатся систематический обзор литературы и оценка доказательств по системе Градации рекомендаций, анализа, разработки и оценки (Grading of Recommendations, Assessment, Development and Evaluation – GRADE). Обсуждаются клинические проявления заболевания, особенности измерения назального оксида азота; анализируются частота и паттерн биения ресничек с использованием высокоскоростного видеомикроскопического анализа, трансмиссионной электронной микроскопии, генотипирования и иммунофлюоресцентного анализа. Для разработки алгоритма диагностики ПЦД, предназначенного для надежного подтверждения либо исключения диагноза, использован модифицированный метод Дельфи. В клинических рекомендациях предложен набор критериев качества диагностических тестов для ПЦД для будущих исследований. Дополнительные материалы представлены на сайте erj.ersjournals.com

The diagnosis of primary ciliary dyskinesia is often confirmed with standard, albeit complex and expensive, tests. In many cases, however, the diagnosis remains difficult despite the array of sophisticated diagnostic tests. There is no “gold standard” reference test. Hence, a Task Force supported by the European Respiratory Society has developed this guideline to provide evidence-based recommendations on diagnostic testing, especially in light of new developments in such tests, and the need for robust diagnoses of patients who might enter randomised controlled trials of treatments. The guideline is based on pre-defined questions relevant for clinical care, a systematic review of the literature, and assessment of the evidence using the GRADE (Grading of Recommendations, Assessment, Development and Evaluation) approach. It focuses on clinical presentation, nasal nitric oxide, analysis of ciliary beat frequency and pattern by high-speed video-microscopy analysis, transmission electron microscopy, genotyping and immunofluorescence. It then used a modified Delphi survey to develop an algorithm for the use of diagnostic tests to definitively confirm and exclude the diagnosis of primary ciliary dyskinesia; and to provide advice when the diagnosis was not conclusive. Finally, this guideline proposes a set of quality criteria for future research on the validity of diagnostic methods for primary ciliary dyskinesia.

This article has supplementary material available from erj.ersjournals.com

первичная цилиарная дискинезиядиагностиканазальный оксид азотачастота биения ресничеквысокоскоростной видеомикроскопический анализтрансмиссионная электронная микроскопиягенотипирование

primary ciliary dyskinesiadiagnosisnasal nitric oxideciliary beat frequencyhigh-speed video-microscopytransmission electron microscopygenotyping

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