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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...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2018
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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. |
format | Online Article Text |
id | pubmed-5761107 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
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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