Abstract
The article presents the most modern positions of healthcare delivery for children with primary ciliary dyskinesia. Symptoms of this pathology in clinical practice vary that is conditioned by genetic heterogeneity of the disease. The most common disease manifestation in children is frequent inflammatory diseases of the upper and lower respiratory tract. They are recorded in most patients, especially in young children, and the diagnosis is often determined untimely due to a low awareness of specialists about this nosology. Differential diagnostic approach is described in detail, peculiarities of treatment and management of children with this nosology are specified. The material is based on clinical guidelines developed and approved by the professional association «Union of Pediatricians of Russia».
Highlights
В статье представлены самые современные позиции оказания медицинской помощи детям с первичной цилиарной дискинезией
The article presents the most modern positions of healthcare delivery for children with primary ciliary dyskinesia. Symptoms of this pathology in clinical practice vary that is conditioned by genetic heterogeneity of the disease
The most common disease manifestation in children is frequent inflammatory diseases of the upper and lower respiratory tract. They are recorded in most patients, especially in young children, and the diagnosis is often determined untimely due to a low awareness of specialists about this nosology
Summary
Наиболее распространенное проявление болезни у детей — частые воспалительные заболевания верхних и нижних дыхательных путей, которые регистрируются у большинства пациентов, особенно у детей раннего возраста, а при невысокой осведомленности специалистов о данной нозологии диагноз зачастую устанавливается несвоевременно. КРАТКАЯ ИНФОРМАЦИЯ Определение Первичная цилиарная дискинезия (ПЦД) — редкое генетически детерминированное заболевание, при котором поражаются подвижные структуры клеток (реснички и жгутики). (For citation: Baranov Alexander А., Namazova-Baranova Leyla S., Vishneva Elena А., Selimzyanova Liliya R., Simonova Olga I., Sereda Elena V., Rozinova Nadezhda N., Tzygina Elena N., Katosova Lubov K., Lazareva Anna V., Gorinova Yulia V., Kustova Olga V. Что исключает воз- фаллопиевых труб у женщин также имеют аномальное можность проведения больших когортных и рандомизи- строение, хотя при этом случаи женского бесплодия рованных контролируемых исследований, поэтому для у больных с ПЦД единичны, однако нередко наблюдается создания протоколов диагностики и терапии используют- внематочная беременность [2].
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