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The significance of pure diffuse mesangial proliferation in idiopathic nephrotic syndrome
Abstract:
The prognostic significance of the finding of diffuse mesangial proliferation (DMP) in patients presenting with idiopathic nephrotic syndrome (INS) has not been well established. The clinical course, therapeutic response and final outcome of 38 patients in whom renal biopsy showed DMP are reported. They have been subdivided into 2 groups according to the absence (18 patients: group I) or presence (20 patients: group II) of superimposed lesions of focal and segmental sclerosis and/or hyalinosis (FSS/H). Clinical presentation was similar in both groups although patients in group I were less severely affected. Non of the patients of group II responded to corticosteroids, whereas in group I 2/16 responded and 2 infants remitted without treatment. At the latest assessment, 5/18 patients in group I and 10/20 in group II had progressed to terminal renal failure or had impaired renal function. Five patients in group I and 3 in group II were in clinical remission. Eight of 11 repeat biopsies performed in patients of group I showed the development of FSS/H. Thus patients with DMP seem prone to develop lesions of FSS/H. Their course if often worse than that of minimal change with FSS/H since 7 of the 10 patients who developed renal failure did so within 3 years of onset. The finding of DMP in a patient with idiopathic nephrotic syndrome is usually but not invariably an ominous feature.
Insights
Diffuse mesangial proliferation (DMP) in idiopathic nephrotic syndrome (INS) often indicates a poor prognosis, with many patients progressing to renal failure. Some patients with DMP may develop focal and segmental sclerosis and/or hyalinosis (FSS/H).
Area of Science:
- Nephrology
- Pathology
Background:
- The prognostic significance of diffuse mesangial proliferation (DMP) in idiopathic nephrotic syndrome (INS) remains unclear.
- Understanding DMP's impact is crucial for managing INS patients.
Purpose of the Study:
- To evaluate the clinical course, treatment response, and outcomes of patients with DMP and INS.
- To investigate the development of focal and segmental sclerosis and/or hyalinosis (FSS/H) in DMP patients.
Main Methods:
- Retrospective analysis of 38 INS patients with DMP on renal biopsy.
- Patients were categorized into two groups: DMP alone (Group I) and DMP with superimposed FSS/H (Group II).
- Clinical presentation, response to corticosteroids, and renal function at follow-up were assessed.
Main Results:
- Group II patients showed no response to corticosteroids, unlike some in Group I.
- A significant proportion of patients in both groups progressed to renal failure or impaired renal function.
- Eight of eleven repeat biopsies in Group I showed the development of FSS/H, suggesting a predisposition.
Conclusions:
- DMP in INS is often an ominous sign, frequently associated with a worse prognosis.
- Patients with DMP appear prone to developing FSS/H lesions.
- Early development of renal failure is noted in a subset of DMP patients.