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Assessment of Kidney Function in Mouse Models of Glomerular Disease
Published on: June 30, 2018
Diagnostic Problems in C3 Glomerulopathy
Leszek Niepolski1, Anna Czekała2, Monika Seget-Dubaniewicz2
1Department of Physiology, Poznan University of Medical Sciences, 60-567 Poznan, Poland.
Insights
Electron microscopy is crucial for diagnosing C3 glomerulopathies (C3GN), a rare kidney disease. This method aids in classifying cases, especially when lesions are subtle or severe, improving diagnostic accuracy for complement-related kidney conditions.
Area of Science:
- Nephrology
- Immunology
- Pathology
Background:
- C3 glomerulopathies (C3GN) are rare kidney diseases characterized by impaired complement regulation and C3 deposition in kidneys.
- Diagnosis involves clinical data, light, fluorescence, and electron microscopy.
- The study analyzed 332 patient biopsy specimens.
Purpose of the Study:
- To evaluate the diagnostic utility of electron microscopy in C3 glomerulopathies.
- To assess the classification of C3GN and dense deposit disease (DDD) cases.
- To determine the necessity of electron microscopy in diagnosing C3 glomerulopathies.
Main Methods:
- Histopathological examination of 332 kidney biopsy specimens.
- Immunofluorescence microscopy to detect C3, C1q, IgA, IgG, and IgM deposits.
- Electron microscopy to examine kidney tissue ultrastructure.
Main Results:
- C3GN (n=111) and DDD (n=17) were identified.
- A significant portion of cases (n=204) were non-classified (NC) due to lesion severity or sclerosis.
- Electron microscopy was performed on all cases.
Conclusions:
- Electron microscopy is essential for diagnosing C3 glomerulopathies, particularly in mild or severe cases.
- It aids in classifying cases where immunofluorescence microscopy findings are equivocal.
- This technique enhances diagnostic accuracy for complement-mediated kidney diseases.
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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