Academic Journal

Первичная цилиарная дискинезия. Клиническое наблюдение

Bibliographic Details
Title: Первичная цилиарная дискинезия. Клиническое наблюдение
Source: Бюллетень Восточно-Сибирского научного центра Сибирского отделения Российской академии медицинских наук.
Publisher Information: Федеральное государственное бюджетное научное учреждение «Научный центр проблем здоровья семьи и репродукции человека», 2016.
Publication Year: 2016
Subject Terms: 03 medical and health sciences, 0302 clinical medicine, ПЕРВИЧНАЯ ЦИЛИАРНАЯ ДИСКИНЕЗИЯ,СИНДРОМ КАРТАГЕНЕРА,КЛИНИЧЕСКОЕ НАБЛЮДЕНИЕ,PRIMARY CILIARY DYSKINESIA,KARTAGENER SYNDROME,CLINICAL OBSERVATION, 3. Good health
Description: We give an example of our own clinical observation of primary ciliary dyskinesia showing difficulties of this disease diagnosis. The complexity of this clinical picture is in the absence of situs viscerum inversus of the patient with early symptoms of chronic bronchitis and chronic sinusitis. From birth patient was noticed to have wheezing during acute respiratory viral infection (4 episodes), first year -bilateral pneumonia, and later 3-4 pneumonias per year. Gastroesophageal reflux disease was diagnosed on third year of life, surgical treatment was used. Year 4 according to computerized tomography data middle lobe syndrome was detected. We performed middle lobectomy. Then we revealed morphologically-cylindrical bronchiectasis. Severe bronchial asthma and allergic rhinitis were diagnosed in 4,5 years. Despite the fact that baseline inhalation and antimicrobial therapy was done, general condition of the patient did not get better. On year 9 we did bronchoscopy with brush biopsy of bronchial mucosa. The result is sharp decrease of ciliary function. All in all, even though the patient had classical symptoms of primary ciliary dyskinesia and first symptoms showed during the first year of life, right diagnose was stated only when the patient turned nine. Primary ciliary dyskinesia is classified as rare (orphan) diseases, that is why awareness of primary care physicians against this pathology is low, resulting in high disability of patients.
В статье приведено собственное наблюдение первичной цилиарной дискинезии, иллюстрирующее трудности диагностики этого заболевания. Сложность данного клинического случая определялась отсутствием инверсии внутренних органов у пациента с ранними симптомами хронического бронхита и хронического синусита. Первичная цилиарная дискинезия относится к орфанным заболеваниям, поэтому осведомлённость врачей первичного звена в отношении этой патологии низкая, что способствует высокой инвалидизации пациентов.
Document Type: Article
File Description: text/html
Language: Russian
ISSN: 1811-0649
Access URL: http://cyberleninka.ru/article/n/pervichnaya-tsiliarnaya-diskineziya-klinicheskoe-nablyudenie
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Accession Number: edsair.od......2806..d9e4750c867991c77f03b43f5fb9ef01
Database: OpenAIRE
Description
ISSN:18110649