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Kartagener’s syndrome: a case report

BACKGROUND: Kartagener’s syndrome is a subset of primary ciliary dyskinesia, an autosomal recessive inherited disorder characterized by the clinical triad of chronic sinusitis, bronchiectasis, and situs inversus. Abnormal ciliary structure or function leading to impaired ciliary motility is the main...

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Autores principales: Tadesse, Abilo, Alemu, Hailemariam, Silamsaw, Mezgebu, Gebrewold, Yonathan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5761107/
https://www.ncbi.nlm.nih.gov/pubmed/29316973
http://dx.doi.org/10.1186/s13256-017-1538-2
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author Tadesse, Abilo
Alemu, Hailemariam
Silamsaw, Mezgebu
Gebrewold, Yonathan
author_facet Tadesse, Abilo
Alemu, Hailemariam
Silamsaw, Mezgebu
Gebrewold, Yonathan
author_sort Tadesse, Abilo
collection PubMed
description BACKGROUND: Kartagener’s syndrome is a subset of primary ciliary dyskinesia, an autosomal recessive inherited disorder characterized by the clinical triad of chronic sinusitis, bronchiectasis, and situs inversus. Abnormal ciliary structure or function leading to impaired ciliary motility is the main pathophysiologic problem in Kartagener’s syndrome. CASE PRESENTATION: A 24-year-old man from Gondar town, North-West Ethiopia, presented to University of Gondar Hospital with recurrent episodes of nasal congestion with itching and paranasal discomfort, and productive cough for more than a decade. Clinical and imaging findings revealed chronic sinusitis, bronchiectasis, dextrocardia, and situs inversus. He was treated with orally administered antibiotics, mucolytic, and chest physiotherapy. He was symptomatically better with the above therapy, and started on a long-term low-dose prophylactic antibiotic. CONCLUSIONS: Patients with Kartagener’s syndrome exist in Ethiopia as cases of chronic recurrent sinopulmonary infections. As there is no easy, reliable non-invasive diagnostic test for Kartagener’s syndrome and the correct diagnosis is often delayed by years, it may cause chronic respiratory problems with reduced quality of life. Genetic counseling and fertility issues should be addressed once Kartagener’s syndrome is diagnosed.
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spelling pubmed-57611072018-01-16 Kartagener’s syndrome: a case report Tadesse, Abilo Alemu, Hailemariam Silamsaw, Mezgebu Gebrewold, Yonathan J Med Case Rep Case Report BACKGROUND: Kartagener’s syndrome is a subset of primary ciliary dyskinesia, an autosomal recessive inherited disorder characterized by the clinical triad of chronic sinusitis, bronchiectasis, and situs inversus. Abnormal ciliary structure or function leading to impaired ciliary motility is the main pathophysiologic problem in Kartagener’s syndrome. CASE PRESENTATION: A 24-year-old man from Gondar town, North-West Ethiopia, presented to University of Gondar Hospital with recurrent episodes of nasal congestion with itching and paranasal discomfort, and productive cough for more than a decade. Clinical and imaging findings revealed chronic sinusitis, bronchiectasis, dextrocardia, and situs inversus. He was treated with orally administered antibiotics, mucolytic, and chest physiotherapy. He was symptomatically better with the above therapy, and started on a long-term low-dose prophylactic antibiotic. CONCLUSIONS: Patients with Kartagener’s syndrome exist in Ethiopia as cases of chronic recurrent sinopulmonary infections. As there is no easy, reliable non-invasive diagnostic test for Kartagener’s syndrome and the correct diagnosis is often delayed by years, it may cause chronic respiratory problems with reduced quality of life. Genetic counseling and fertility issues should be addressed once Kartagener’s syndrome is diagnosed. BioMed Central 2018-01-10 /pmc/articles/PMC5761107/ /pubmed/29316973 http://dx.doi.org/10.1186/s13256-017-1538-2 Text en © The Author(s). 2018 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Tadesse, Abilo
Alemu, Hailemariam
Silamsaw, Mezgebu
Gebrewold, Yonathan
Kartagener’s syndrome: a case report
title Kartagener’s syndrome: a case report
title_full Kartagener’s syndrome: a case report
title_fullStr Kartagener’s syndrome: a case report
title_full_unstemmed Kartagener’s syndrome: a case report
title_short Kartagener’s syndrome: a case report
title_sort kartagener’s syndrome: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5761107/
https://www.ncbi.nlm.nih.gov/pubmed/29316973
http://dx.doi.org/10.1186/s13256-017-1538-2
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