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Regression of membranoproliferative glomerulonephritis type II (dense deposit disease): observations in six children
1Division of Pediatric Nephrology, Children's Hospital Medical Center, Cincinnati, OH 45229.
Insights
Alternate-day prednisone therapy significantly reduced mesangial proliferation and improved kidney function in children with membranoproliferative glomerulonephritis Type II (MPGN II-dense deposit disease). Dense deposits were ultrastructurally lost in some patients.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Glomerular Diseases
Background:
- Membranoproliferative glomerulonephritis Type II (MPGN II), also known as dense deposit disease, is a rare kidney disorder.
- MPGN II is characterized by abnormal deposits within the glomerular basement membrane.
- Long-term treatment outcomes for MPGN II in children are not well-established.
Purpose of the Study:
- To evaluate the long-term effects of alternate-day prednisone on renal histology in pediatric MPGN II patients.
- To assess changes in mesangial proliferation and capillary lumen patency over time.
- To investigate ultrastructural changes in glomerular deposits during treatment.
Main Methods:
- Retrospective analysis of serial renal biopsies from six children with MPGN II.
- Patients received continuous alternate-day prednisone therapy for an average of 14 years.
- Histopathological and ultrastructural examination of kidney biopsies.
Main Results:
- All patients showed reduced mesangial proliferation and improved capillary lumen patency.
- Ultrastructural analysis revealed a shift of deposits from the lamina densa to the lamina rara interna in four patients.
- Complete loss of dense deposits was observed in the capillary walls of two patients.
Conclusions:
- Alternate-day prednisone therapy can lead to significant histological improvements in pediatric MPGN II.
- The observed ultrastructural changes suggest a potential mechanism for deposit clearance.
- Long-term prednisone treatment may be a viable therapeutic option for MPGN II in children.
Abstract:
Six children with membranoproliferative glomerulonephritis Type II (MPGN II-dense deposit disease), who were followed an average of 14 years (3.5 to 22 years) and treated continuously with an alternate-day prednisone regimen, have been studied by successive renal biopsies over intervals of 2 to 14 years of disease. All have shown significant reduction in mesangial proliferation and improvement of capillary lumen patency. A change in position of the deposits, from the lamina densa to the lamina rara interna, was demonstrated ultrastructurally in four of the patients, accompanied by reduction in prevalence of deposit in three. This loss of dense deposits, characterized as a process of "dropping off," was complete in the free capillary walls of two of the patients.