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Diagnostic Problems in C3 Glomerulopathy

Background: C3 glomerulopathies (C3GN) are a group of rare kidney diseases associated with impaired complement regulation. The effects of this disease include the accumulation of complement C3 in the kidneys. Based on the clinical data, as well as light, fluorescence, and electron microscopy results...

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Autores principales: Niepolski, Leszek, Czekała, Anna, Seget-Dubaniewicz, Monika, Frydrychowicz, Magdalena, Talarska-Markiewicz, Patrycja, Kowalska, Angelika, Szmelter, Jagoda, Salwa-Żurawska, Wiesława, Sirek, Tomasz, Sobański, Dawid, Grabarek, Beniamin Oskar, Żurawski, Jakub
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10135645/
https://www.ncbi.nlm.nih.gov/pubmed/37189718
http://dx.doi.org/10.3390/biomedicines11041101
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author Niepolski, Leszek
Czekała, Anna
Seget-Dubaniewicz, Monika
Frydrychowicz, Magdalena
Talarska-Markiewicz, Patrycja
Kowalska, Angelika
Szmelter, Jagoda
Salwa-Żurawska, Wiesława
Sirek, Tomasz
Sobański, Dawid
Grabarek, Beniamin Oskar
Żurawski, Jakub
author_facet Niepolski, Leszek
Czekała, Anna
Seget-Dubaniewicz, Monika
Frydrychowicz, Magdalena
Talarska-Markiewicz, Patrycja
Kowalska, Angelika
Szmelter, Jagoda
Salwa-Żurawska, Wiesława
Sirek, Tomasz
Sobański, Dawid
Grabarek, Beniamin Oskar
Żurawski, Jakub
author_sort Niepolski, Leszek
collection PubMed
description Background: C3 glomerulopathies (C3GN) are a group of rare kidney diseases associated with impaired complement regulation. The effects of this disease include the accumulation of complement C3 in the kidneys. Based on the clinical data, as well as light, fluorescence, and electron microscopy results, the diagnoses were verified. The study group consisted of biopsy specimens, which were obtained from 332 patients who were diagnosed with C3 glomerulopathy. In all cases, histopathological examinations were performed; deposits of complement C3 and C1q components, as well as the immunoglobulins IgA, IgG, and IgM, were identified using immunofluorescence. Furthermore, electron microscopy was also performed. Results: The histopathological examination results presented cases of C3GN (n = 111) and dense deposit disease (DDD; n = 17). The non-classified (NC) group was the most numerous (n = 204). The lack of classification was due to the poor severity of the lesions, even on the electron microscopic examination or in the presence of intense sclerotic lesions. Conclusions: In cases of suspected C3 glomerulopathies, we believe an electron microscopy examination is necessary. This examination is beneficial in mild-to-extremely-severe cases of this glomerulopathy, where the lesions are barely discernible when using immunofluorescence microscopy.
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spelling pubmed-101356452023-04-28 Diagnostic Problems in C3 Glomerulopathy Niepolski, Leszek Czekała, Anna Seget-Dubaniewicz, Monika Frydrychowicz, Magdalena Talarska-Markiewicz, Patrycja Kowalska, Angelika Szmelter, Jagoda Salwa-Żurawska, Wiesława Sirek, Tomasz Sobański, Dawid Grabarek, Beniamin Oskar Żurawski, Jakub Biomedicines Article Background: C3 glomerulopathies (C3GN) are a group of rare kidney diseases associated with impaired complement regulation. The effects of this disease include the accumulation of complement C3 in the kidneys. Based on the clinical data, as well as light, fluorescence, and electron microscopy results, the diagnoses were verified. The study group consisted of biopsy specimens, which were obtained from 332 patients who were diagnosed with C3 glomerulopathy. In all cases, histopathological examinations were performed; deposits of complement C3 and C1q components, as well as the immunoglobulins IgA, IgG, and IgM, were identified using immunofluorescence. Furthermore, electron microscopy was also performed. Results: The histopathological examination results presented cases of C3GN (n = 111) and dense deposit disease (DDD; n = 17). The non-classified (NC) group was the most numerous (n = 204). The lack of classification was due to the poor severity of the lesions, even on the electron microscopic examination or in the presence of intense sclerotic lesions. Conclusions: In cases of suspected C3 glomerulopathies, we believe an electron microscopy examination is necessary. This examination is beneficial in mild-to-extremely-severe cases of this glomerulopathy, where the lesions are barely discernible when using immunofluorescence microscopy. MDPI 2023-04-05 /pmc/articles/PMC10135645/ /pubmed/37189718 http://dx.doi.org/10.3390/biomedicines11041101 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Niepolski, Leszek
Czekała, Anna
Seget-Dubaniewicz, Monika
Frydrychowicz, Magdalena
Talarska-Markiewicz, Patrycja
Kowalska, Angelika
Szmelter, Jagoda
Salwa-Żurawska, Wiesława
Sirek, Tomasz
Sobański, Dawid
Grabarek, Beniamin Oskar
Żurawski, Jakub
Diagnostic Problems in C3 Glomerulopathy
title Diagnostic Problems in C3 Glomerulopathy
title_full Diagnostic Problems in C3 Glomerulopathy
title_fullStr Diagnostic Problems in C3 Glomerulopathy
title_full_unstemmed Diagnostic Problems in C3 Glomerulopathy
title_short Diagnostic Problems in C3 Glomerulopathy
title_sort diagnostic problems in c3 glomerulopathy
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10135645/
https://www.ncbi.nlm.nih.gov/pubmed/37189718
http://dx.doi.org/10.3390/biomedicines11041101
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