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A case of Kartagener's syndrome with combined aplasia of frontal and sphenoid sinuses and hypoplasia of maxillary and ethmoid sinuses

Kartagener's syndrome (KS), characterized by a triad of bronchiectasis, chronic sinusitis, and situs inversus, is a subset of an autosomal recessive hereditary disorder of primary ciliary dyskinesia. We report the case of a 35-year-old male who presented with a history of intermittent episodes...

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Autores principales: Sharma, Sonica, Durgaprasad, Bhamidipaty K., Vijayalakshmi, Payala
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8213105/
https://www.ncbi.nlm.nih.gov/pubmed/34194278
http://dx.doi.org/10.4103/jfcm.JFCM_304_20
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author Sharma, Sonica
Durgaprasad, Bhamidipaty K.
Vijayalakshmi, Payala
author_facet Sharma, Sonica
Durgaprasad, Bhamidipaty K.
Vijayalakshmi, Payala
author_sort Sharma, Sonica
collection PubMed
description Kartagener's syndrome (KS), characterized by a triad of bronchiectasis, chronic sinusitis, and situs inversus, is a subset of an autosomal recessive hereditary disorder of primary ciliary dyskinesia. We report the case of a 35-year-old male who presented with a history of intermittent episodes of productive cough, breathlessness, and cold since childhood. High resolution computed tomography of chest revealed bronchiectatic changes, dextrocardia, and right-sided aortic arch. Computed tomography (CT) scan of the abdomen revealed situs inversus. CT of the paranasal sinuses revealed combined aplasia of bilateral frontal and sphenoid sinus with sinusitis. Based on these findings, a diagnosis of KS was made. There was no complaint of infertility, which usually accompanies KS, even though an analysis of his seminal fluid revealed reduced count and reduced motility of sperms. The uniqueness of our case is that our patient was a male aged 35 years; besides, the third decade is an unusual age for presentation of combined aplasia/agenesis of bilateral frontal and sphenoid sinuses with hypoplasia of maxillary and ethmoid sinuses. Even though these findings have been reported in children and young adults, there are very few case reports of such a presentation in adults in literature.
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spelling pubmed-82131052021-06-29 A case of Kartagener's syndrome with combined aplasia of frontal and sphenoid sinuses and hypoplasia of maxillary and ethmoid sinuses Sharma, Sonica Durgaprasad, Bhamidipaty K. Vijayalakshmi, Payala J Family Community Med Case Report Kartagener's syndrome (KS), characterized by a triad of bronchiectasis, chronic sinusitis, and situs inversus, is a subset of an autosomal recessive hereditary disorder of primary ciliary dyskinesia. We report the case of a 35-year-old male who presented with a history of intermittent episodes of productive cough, breathlessness, and cold since childhood. High resolution computed tomography of chest revealed bronchiectatic changes, dextrocardia, and right-sided aortic arch. Computed tomography (CT) scan of the abdomen revealed situs inversus. CT of the paranasal sinuses revealed combined aplasia of bilateral frontal and sphenoid sinus with sinusitis. Based on these findings, a diagnosis of KS was made. There was no complaint of infertility, which usually accompanies KS, even though an analysis of his seminal fluid revealed reduced count and reduced motility of sperms. The uniqueness of our case is that our patient was a male aged 35 years; besides, the third decade is an unusual age for presentation of combined aplasia/agenesis of bilateral frontal and sphenoid sinuses with hypoplasia of maxillary and ethmoid sinuses. Even though these findings have been reported in children and young adults, there are very few case reports of such a presentation in adults in literature. Wolters Kluwer - Medknow 2021 2021-05-08 /pmc/articles/PMC8213105/ /pubmed/34194278 http://dx.doi.org/10.4103/jfcm.JFCM_304_20 Text en Copyright: © 2021 Journal of Family and Community Medicine https://creativecommons.org/licenses/by-nc-sa/4.0/This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Case Report
Sharma, Sonica
Durgaprasad, Bhamidipaty K.
Vijayalakshmi, Payala
A case of Kartagener's syndrome with combined aplasia of frontal and sphenoid sinuses and hypoplasia of maxillary and ethmoid sinuses
title A case of Kartagener's syndrome with combined aplasia of frontal and sphenoid sinuses and hypoplasia of maxillary and ethmoid sinuses
title_full A case of Kartagener's syndrome with combined aplasia of frontal and sphenoid sinuses and hypoplasia of maxillary and ethmoid sinuses
title_fullStr A case of Kartagener's syndrome with combined aplasia of frontal and sphenoid sinuses and hypoplasia of maxillary and ethmoid sinuses
title_full_unstemmed A case of Kartagener's syndrome with combined aplasia of frontal and sphenoid sinuses and hypoplasia of maxillary and ethmoid sinuses
title_short A case of Kartagener's syndrome with combined aplasia of frontal and sphenoid sinuses and hypoplasia of maxillary and ethmoid sinuses
title_sort case of kartagener's syndrome with combined aplasia of frontal and sphenoid sinuses and hypoplasia of maxillary and ethmoid sinuses
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8213105/
https://www.ncbi.nlm.nih.gov/pubmed/34194278
http://dx.doi.org/10.4103/jfcm.JFCM_304_20
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