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[Minimal change disease and focal segmental glomerulosclerosis]
J Müller-Deile1, H Schenk2, M Schiffer3,4
1Medizinische Klinik IV, Friedrich-Alexander-Universität Erlangen-Nürnberg, Ulmenweg 18, 91054, Erlangen, Deutschland.
Abstract:
Minimal change disease (MCD) or minimal change glomerulonephritis and focal segmental glomerulosclerosis (FSGS) are the two major causes of nephrotic syndrome in children and young adults. Both disease entities resemble each other and can sometimes only be discriminated on the basis of their clinical courses. MCD and FSGS display two classical examples that share a common pathophysiology in which the glomerular podocyte and the cytoskeleton of its foot processes play important roles. Therefore, the term "podocytopathy" was introduced for both diseases. In this article, we compare their differences and similarities, and summarized new data on pathophysiology and treatment. In adults, only a renal biopsy including electron microscopy allows for the discrimination of MCD and FSGS and other differential diagnoses. The identification of a primary or secondary form of the disease is based on the clinical course. Data from studies on the treatment are sparse; hence, treatment is still based on high-dose steroids followed by additional immunosuppressive agents. In secondary forms, treatment of the underlying disease is elementary.
Insights
Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are leading causes of nephrotic syndrome. Both podocytopathies share pathophysiology, but diagnosis and treatment differ, especially in adults where biopsy is key.
Area of Science:
- Nephrology
- Pathology
Background:
- Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are primary causes of nephrotic syndrome in pediatric and young adult populations.
- These conditions share a common pathophysiology involving glomerular podocytes and their cytoskeletal structure, leading to the classification as
- podocytopathies
Purpose of the Study:
- To compare the similarities and differences between MCD and FSGS.
- To summarize recent findings on the pathophysiology and treatment of these glomerular diseases.
Main Methods:
- Review of clinical courses for disease discrimination.
- Electron microscopy of renal biopsies in adults for definitive diagnosis.
- Analysis of current treatment strategies and sparse clinical data.
Main Results:
- In adults, distinguishing MCD from FSGS and other diagnoses necessitates renal biopsy with electron microscopy.
- Clinical course is crucial for identifying primary versus secondary disease forms.
- Treatment remains largely empirical, relying on high-dose steroids and immunosuppressants, with a focus on addressing underlying conditions in secondary cases.
Conclusions:
- MCD and FSGS represent distinct podocytopathies with overlapping yet differentiating clinical and pathological features.
- Accurate diagnosis, particularly in adults, relies on advanced techniques like electron microscopy.
- Further research is needed to optimize treatment protocols for these challenging nephrotic syndromes.
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