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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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Detalles Bibliográficos
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
Descripción
Sumario: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.