Primary ciliary dyskinesia. Clinical observation

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 b...

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Autores principales:	T. B. Pavlova, V. M. Shinkaryova
Formato:	article
Lenguaje:	RU
Publicado:	Scientific Сentre for Family Health and Human Reproduction Problems 2016
Materias:	primary ciliary dyskinesia kartagener syndrome clinical observation Science Q
Acceso en línea:	https://doaj.org/article/9ff7aecd708c447a8bb84704dc877268
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spelling	oai:doaj.org-article:9ff7aecd708c447a8bb84704dc8772682021-11-23T06:14:33ZPrimary ciliary dyskinesia. Clinical observation2541-94202587-959610.12737/21492https://doaj.org/article/9ff7aecd708c447a8bb84704dc8772682016-02-01T00:00:00Zhttps://www.actabiomedica.ru/jour/article/view/159https://doaj.org/toc/2541-9420https://doaj.org/toc/2587-9596We 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.T. B. PavlovaV. M. ShinkaryovaScientific Сentre for Family Health and Human Reproduction Problemsarticleprimary ciliary dyskinesiakartagener syndromeclinical observationScienceQRUActa Biomedica Scientifica, Vol 1, Iss 1, Pp 75-77 (2016)
institution	DOAJ
collection	DOAJ
language	RU
topic	primary ciliary dyskinesia kartagener syndrome clinical observation Science Q
spellingShingle	primary ciliary dyskinesia kartagener syndrome clinical observation Science Q T. B. Pavlova V. M. Shinkaryova Primary ciliary dyskinesia. Clinical observation
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.
format	article
author	T. B. Pavlova V. M. Shinkaryova
author_facet	T. B. Pavlova V. M. Shinkaryova
author_sort	T. B. Pavlova
title	Primary ciliary dyskinesia. Clinical observation
title_short	Primary ciliary dyskinesia. Clinical observation
title_full	Primary ciliary dyskinesia. Clinical observation
title_fullStr	Primary ciliary dyskinesia. Clinical observation
title_full_unstemmed	Primary ciliary dyskinesia. Clinical observation
title_sort	primary ciliary dyskinesia. clinical observation
publisher	Scientific Сentre for Family Health and Human Reproduction Problems
publishDate	2016
url	https://doaj.org/article/9ff7aecd708c447a8bb84704dc877268
work_keys_str_mv	AT tbpavlova primaryciliarydyskinesiaclinicalobservation AT vmshinkaryova primaryciliarydyskinesiaclinicalobservation
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Primary ciliary dyskinesia. Clinical observation

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