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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...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Dove Medical Press
2018
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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. |
format | Online Article Text |
id | pubmed-6292226 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Dove Medical Press |
record_format | MEDLINE/PubMed |
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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