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Kartagener syndrome complicated by immunoglobulin A nephropathy

We herein report the case of a 36-year-old woman with Kartagener syndrome (KS), which is an autosomal recessive disorder defined by a triad of bronchiectasis, sinusitis, and situs inversus, with complications of asymptomatic microhematuria and proteinuria. She was finally diagnosed with biopsy-prove...

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Autores principales: Oka, Kentaro, Sugase, Taro, Akimoto, Tetsu, Murakami, Takuya, Nagayama, Izumi, Kaneko, Miwa, Asakura, Maki, Ohara, Ken, Saito, Osamu, Nagata, Daisuke
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Dove Medical Press 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6292226/
https://www.ncbi.nlm.nih.gov/pubmed/30573994
http://dx.doi.org/10.2147/IMCRJ.S185887
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author Oka, Kentaro
Sugase, Taro
Akimoto, Tetsu
Murakami, Takuya
Nagayama, Izumi
Kaneko, Miwa
Asakura, Maki
Ohara, Ken
Saito, Osamu
Nagata, Daisuke
author_facet Oka, Kentaro
Sugase, Taro
Akimoto, Tetsu
Murakami, Takuya
Nagayama, Izumi
Kaneko, Miwa
Asakura, Maki
Ohara, Ken
Saito, Osamu
Nagata, Daisuke
author_sort Oka, Kentaro
collection PubMed
description We herein report the case of a 36-year-old woman with Kartagener syndrome (KS), which is an autosomal recessive disorder defined by a triad of bronchiectasis, sinusitis, and situs inversus, with complications of asymptomatic microhematuria and proteinuria. She was finally diagnosed with biopsy-proven immunoglobulin (Ig) A nephropathy. KS constitutes a subgroup of primary ciliary dyskinesia (PCD) characterized by structural and/or functional ciliary abnormalities resulting in sinopulmonary involvement with varying severity. Our case does not allow us to corroborate the clinical impact of KS and/or PCD as a pathogenic basis for the IgA nephropathy, and each disease might develop independently. However, systematic studies on this topic are quite lacking, so we strongly recommend the accumulation of more cases similar to our own, which would allow us to clarify the nature of this disease state more precisely.
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spelling pubmed-62922262018-12-20 Kartagener syndrome complicated by immunoglobulin A nephropathy Oka, Kentaro Sugase, Taro Akimoto, Tetsu Murakami, Takuya Nagayama, Izumi Kaneko, Miwa Asakura, Maki Ohara, Ken Saito, Osamu Nagata, Daisuke Int Med Case Rep J Case Report We herein report the case of a 36-year-old woman with Kartagener syndrome (KS), which is an autosomal recessive disorder defined by a triad of bronchiectasis, sinusitis, and situs inversus, with complications of asymptomatic microhematuria and proteinuria. She was finally diagnosed with biopsy-proven immunoglobulin (Ig) A nephropathy. KS constitutes a subgroup of primary ciliary dyskinesia (PCD) characterized by structural and/or functional ciliary abnormalities resulting in sinopulmonary involvement with varying severity. Our case does not allow us to corroborate the clinical impact of KS and/or PCD as a pathogenic basis for the IgA nephropathy, and each disease might develop independently. However, systematic studies on this topic are quite lacking, so we strongly recommend the accumulation of more cases similar to our own, which would allow us to clarify the nature of this disease state more precisely. Dove Medical Press 2018-12-10 /pmc/articles/PMC6292226/ /pubmed/30573994 http://dx.doi.org/10.2147/IMCRJ.S185887 Text en © 2018 Oka et al. This work is published and licensed by Dove Medical Press Limited The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed.
spellingShingle Case Report
Oka, Kentaro
Sugase, Taro
Akimoto, Tetsu
Murakami, Takuya
Nagayama, Izumi
Kaneko, Miwa
Asakura, Maki
Ohara, Ken
Saito, Osamu
Nagata, Daisuke
Kartagener syndrome complicated by immunoglobulin A nephropathy
title Kartagener syndrome complicated by immunoglobulin A nephropathy
title_full Kartagener syndrome complicated by immunoglobulin A nephropathy
title_fullStr Kartagener syndrome complicated by immunoglobulin A nephropathy
title_full_unstemmed Kartagener syndrome complicated by immunoglobulin A nephropathy
title_short Kartagener syndrome complicated by immunoglobulin A nephropathy
title_sort kartagener syndrome complicated by immunoglobulin a nephropathy
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6292226/
https://www.ncbi.nlm.nih.gov/pubmed/30573994
http://dx.doi.org/10.2147/IMCRJ.S185887
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