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Published on: June 18, 2020
Membranoproliferative glomerulonephritis in Russian population
V A Dobronravov1, A V Smirnov1
1Research Institute of Nephrology, I.P. Pavlov First Saint Petersburg State Medical University of the Ministry of Health of the Russian Federation, Saint Petersburg, Russia.
Aim:
Analysis of etiology, clinical and morphological manifestations, approaches to therapy and prognosis of membranoproliferative glomerulonephritis (MPGN).
Materials And Methods:
Cases of MPGN were retrospectively identified in the period 2000-2017 with subsequent analysis of etiology, clinical data and morphology (including deposits of immunoglobulins (Ig) and C3 complement fractions). The achievement of complete and partial remissions (PR, CR), overall survival, progression (by composite endpoint: decrease in the estimated GFR (eGFR) ≥50% from the baseline or eGFR <15 ml/min/1.73 m2 or the onset of dialysis).
Results:
214 cases of MPGN entered the study with the average age of 44±16 years. Most patients had nephrotic syndrome and significant hematuria. In 58.4% of cases, eGFR was <60 mL/min/1.73 m2, and every fifth patient had CKD 4 or 5 stages. The prevalence of MPGN among all biopsy-confirmed glomerulopathies was 9.3%. Idiopathic MPGN (iMPGN) was detected in 30.4% of cases, secondary MSGN (sMPGN) - in 69.6% (autoimmune diseases - 34.1%, infectious diseases - 16.4%, monoclonal gammopathies - 9.3%, complement-mediated damage - 9.8%). Ig+C3+MPGN was mainly associated with autoimmune diseases and infections; C3-glomerulopathy or thrombotic microangiopathy were most often causes of Ig-C3+MPGN; Ig-C3-/Ig+C3-MPGN had heterogeneous etiology. The median follow-up period was 28 [7; 37] months. The 10-year total cumulative patient and renal survival rates were 71 and 50%, respectively (without differences between sMPGN and iMPGN). The frequency of the PR/CR was 50% (iMPGN - 46.2%, sMPGN - 51.3%) depending on the etiology of the MPGN (p=0.049). The cumulative 10-year progression-free renal survival was nearly 100% in cases with PR/CR and 0% in non-responders.
Conclusion:
MPGN is a severe variant of glomerular damage with a heterogeneous etiological structure and an unfavorable prognosis. Targeted clinical and morphological diagnostics of MPGN allows to identify the cause of the disease in most cases. This approach is reliable for the adequate treatment choice and improvement of outcomes in MPGN.
Insights
Membranoproliferative glomerulonephritis (MPGN) is a serious kidney disease with diverse causes and poor outcomes. Identifying the specific etiology through diagnostics is crucial for effective treatment and improved patient prognosis.
Area of Science:
- Nephrology
- Pathology
- Immunology
Background:
- Membranoproliferative glomerulonephritis (MPGN) represents a significant cause of kidney disease, characterized by complex immunological and morphological features.
- Understanding the diverse etiologies and clinical presentations of MPGN is essential for effective patient management.
Purpose of the Study:
- To analyze the etiology, clinical and morphological manifestations, therapeutic approaches, and prognosis of MPGN.
- To investigate the relationship between MPGN subtypes, etiology, and patient outcomes.
Main Methods:
- Retrospective analysis of 214 MPGN cases diagnosed between 2000 and 2017.
- Evaluation of clinical data, kidney biopsy morphology, immunoglobulin (Ig) and C3 complement deposits.
- Assessment of complete remission (CR), partial remission (PR), overall survival, and renal progression defined by estimated GFR (eGFR) decline or dialysis initiation.
Main Results:
- MPGN accounted for 9.3% of biopsy-confirmed glomerulopathies, with a mean patient age of 44 years.
- Secondary MPGN (sMPGN) was more prevalent (69.6%) than idiopathic MPGN (iMPGN) (30.4%), with autoimmune diseases, infections, and monoclonal gammopathies as common causes.
- 10-year renal survival was 50%, with 50% of patients achieving CR/PR. Achieving CR/PR correlated with significantly better long-term renal survival.
Conclusions:
- MPGN is a severe kidney disease with a heterogeneous etiology and generally unfavorable prognosis.
- Accurate etiological diagnosis through integrated clinical and morphological assessment is key to selecting appropriate treatments.
- Targeted diagnostics and treatment strategies can improve outcomes for patients with MPGN.
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