Kartagener syndrome, current data on a classical disease. Case report.

Introduction: This article addresses the general aspects (pathophysiology, embryology, clinical presentation and prognosis) of the Kartagener syndrome (KS). Case presentation: 26-year-old male patient, with a history of complicated sinusitis with cerebral abscess and secondary epilepsy, who consulte...

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Main Authors: Sandra Viviana Gómez-Correa, Iván David Ruiz-Ángel, Luis Carlos Salazar-Díaz
Format: Article
Language:English
Published: Universidad Nacional de Colombia 2018-07-01
Series:Case Reports
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Online Access:https://revistas.unal.edu.co/index.php/care/article/view/69756
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author Sandra Viviana Gómez-Correa
Iván David Ruiz-Ángel
Luis Carlos Salazar-Díaz
author_facet Sandra Viviana Gómez-Correa
Iván David Ruiz-Ángel
Luis Carlos Salazar-Díaz
author_sort Sandra Viviana Gómez-Correa
collection DOAJ
description Introduction: This article addresses the general aspects (pathophysiology, embryology, clinical presentation and prognosis) of the Kartagener syndrome (KS). Case presentation: 26-year-old male patient, with a history of complicated sinusitis with cerebral abscess and secondary epilepsy, who consulted to the Hospital Universitario Nacional de Colombia due to headache, fever and mucus expectoration. The presence of situs inversus, chronic sinusitis and bronchiectasis suggested a diagnosis of primary ciliary dyskinesia and KS. Discussion: Differential diagnoses of KS should be framed in its possible causal relationship with primary ciliary dyskinesia and other diagnoses associated with secondary ciliary dysfunction, such as cystic fibrosis, immunodeficiency and anatomical-functional conditions with rhinosenopulmonary involvement. Clinical suspicion of KS occurs when the heart is auscultated on the right and the liver is palpated on the left. Confirmation is achieved through imaging methods that prove visceral heterotaxia, indirect methods related to scan of ciliary malfunction (nasal nitric oxide, video microscopy) and ciliary biopsy that demonstrates the defect of the ciliary ultrastructure. Conclusions: Respiratory infectious involvement in patients with KS is explained by the alteration of the cilia, which leads both to the malposition of some organs and to the structural and functional alteration of others.
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spelling doaj-art-2a1aa02d0c2345fd9642501c10a90fd22025-08-20T01:52:44ZengUniversidad Nacional de ColombiaCase Reports2462-85222018-07-014210.15446/cr.v4n2.6975649170Kartagener syndrome, current data on a classical disease. Case report.Sandra Viviana Gómez-CorreaIván David Ruiz-ÁngelLuis Carlos Salazar-DíazIntroduction: This article addresses the general aspects (pathophysiology, embryology, clinical presentation and prognosis) of the Kartagener syndrome (KS). Case presentation: 26-year-old male patient, with a history of complicated sinusitis with cerebral abscess and secondary epilepsy, who consulted to the Hospital Universitario Nacional de Colombia due to headache, fever and mucus expectoration. The presence of situs inversus, chronic sinusitis and bronchiectasis suggested a diagnosis of primary ciliary dyskinesia and KS. Discussion: Differential diagnoses of KS should be framed in its possible causal relationship with primary ciliary dyskinesia and other diagnoses associated with secondary ciliary dysfunction, such as cystic fibrosis, immunodeficiency and anatomical-functional conditions with rhinosenopulmonary involvement. Clinical suspicion of KS occurs when the heart is auscultated on the right and the liver is palpated on the left. Confirmation is achieved through imaging methods that prove visceral heterotaxia, indirect methods related to scan of ciliary malfunction (nasal nitric oxide, video microscopy) and ciliary biopsy that demonstrates the defect of the ciliary ultrastructure. Conclusions: Respiratory infectious involvement in patients with KS is explained by the alteration of the cilia, which leads both to the malposition of some organs and to the structural and functional alteration of others.https://revistas.unal.edu.co/index.php/care/article/view/69756Kartagener SyndromePrimary Ciliary DyskinesiaCiliaembryologySitus Inversus.
spellingShingle Sandra Viviana Gómez-Correa
Iván David Ruiz-Ángel
Luis Carlos Salazar-Díaz
Kartagener syndrome, current data on a classical disease. Case report.
Case Reports
Kartagener Syndrome
Primary Ciliary Dyskinesia
Cilia
embryology
Situs Inversus.
title Kartagener syndrome, current data on a classical disease. Case report.
title_full Kartagener syndrome, current data on a classical disease. Case report.
title_fullStr Kartagener syndrome, current data on a classical disease. Case report.
title_full_unstemmed Kartagener syndrome, current data on a classical disease. Case report.
title_short Kartagener syndrome, current data on a classical disease. Case report.
title_sort kartagener syndrome current data on a classical disease case report
topic Kartagener Syndrome
Primary Ciliary Dyskinesia
Cilia
embryology
Situs Inversus.
url https://revistas.unal.edu.co/index.php/care/article/view/69756
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AT ivandavidruizangel kartagenersyndromecurrentdataonaclassicaldiseasecasereport
AT luiscarlossalazardiaz kartagenersyndromecurrentdataonaclassicaldiseasecasereport