PRIMARY CILIARY DYSKINESIA. CLINICAL OBSERVATION

Tatyana Pavlova; Vera Shinkareva

doi:doi:10.12737/21492

PRIMARY CILIARY DYSKINESIA. CLINICAL OBSERVATION

Published в Acta biomedica scientifica · Volume 1, Issue 1, 2016 · Pages 75–77 · Rubrics: Case report

DOI 10.12737/21492

Received: 15.09.2016 Accepted: 15.09.2016 Published: 15.09.2016 Language of publication: RUS

Authors

Tatyana Pavlova · Vera Shinkareva

ABSTRACT

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.

KEYWORDS

primary ciliary dyskinesia Kartagener syndrome clinical observation

Information References

Authors:

Type:

Article

Pages:

from 75 to 77

Status:

Published

Language:

Russian

Keywords:

primary ciliary dyskinesia, Kartagener syndrome, clinical observation

References

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2. Bobrovnichiy VI, Bezler ZA (2011). Primary ciliary dyskinesia [Pervichnaya ciliarnaya diskineziya]. Medicinskiy zhurnal, 2, 152-156.

3. Kaganov SY, Rozinova NN, Kerimov MB (1991). Clinical variants of kids’ Kartagener syndrome [Klinicheskiye varianti sindroma Kartagenera u detey]. Pulmonogiya, 2, 20-25.

4. Rozinova NN (2003). Primary ciliary dyskinesia in children [Pervichnaya ciliarnaya diskineziya u detey]. Voprosi sovremennoy pediatrii, 6, 28-32.

5. Meeks M, Bush A (2000). Primary ciliary dyskinesia. Pediatric pulmonology, 29, 307-316.

6. Philpott CM, McKiernan DC (2008). Bronchiectasis and sino-nasal disease: a review. J. Laryngol. Otol., 122 (1), 11-15.

7. Steinkamp G, Seithe H, Nusslein T (2004). Therapy of primary ciliary dyskinesia. Pneumologie, 58 (3), 179-187.

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