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Primary membranous nephropathy in adolescence: A prospective study
Vinod Kumar1, Ashwani Kumar Varma1, Ritambhra Nada2
1Department of Nephrology, Histopathology and Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Aim:
Primary membranous nephropathy (PMN) accounts for only 1-2% of nephrotic syndrome in children. Antibodies to m-type phospholipase A2 receptor (aPLA2 R) is seen in 70% of adult PMN cases. The present study was undertaken to study m-type phospholipase A2 receptor (PLA2 R) status and clinical behavior in adolescent PMN cases.
Methods:
The present prospective observational study included adolescent (10-19 years) onset biopsy proved PMN. Patients were followed on a monthly basis with urine protein, serum albumin and creatinine. Serum aPLA2 R was done at baseline and at 6 and 12 months of starting treatment. Patients were treated as per unit's protocol.
Results:
During the study period a total of 18 patients were enrolled. The mean age of the cases was 16.27 ± 2.39 (11-19) years. Seventeen (94.44%) patients presented with nephrotic syndrome. The mean proteinuria and serum albumin was 4.52 ± 1.93 (2.43-9.20) g/day and 2.1 ± 0.6 (1.1-3.4) g/dL respectively. PMN was PLA2 R related in 83%. aPLA2R and enhanced staining for PLA2 R in glomeruli was seen in 14 (77.78%) and 13 (72.22%), respectively. Clinical remission at the end of 6 and 12 months of therapy was seen in 11 (61.11%) and 9 (50%) subjects respectively. There was a significant association of aPLA2 R to clinical remission/ resistance.
Conclusion:
Primary membranous nephropathy in adolescent population is aPLA2 R related in over three-quarters of the cases and the response to therapy is seen in only half of them. aPLA2 R monitoring is clinically relevant and should be incorporated in the management of adolescent onset PMN.
Insights
Primary membranous nephropathy (PMN) in adolescents is often linked to antibodies against the phospholipase A2 receptor (aPLA2R). While many cases show aPLA2R involvement, only about half achieve clinical remission, highlighting the need for targeted monitoring.
Area of Science:
- Nephrology
- Immunology
- Pediatric Nephrology
Background:
- Primary membranous nephropathy (PMN) is rare in children, accounting for 1-2% of nephrotic syndrome cases.
- Antibodies to the phospholipase A2 receptor (aPLA2R) are implicated in a majority of adult PMN cases.
- The role of aPLA2R in adolescent PMN requires further investigation.
Purpose of the Study:
- To investigate the prevalence of aPLA2R in adolescent-onset PMN.
- To analyze the clinical presentation and behavior of PMN in adolescents.
- To assess the correlation between aPLA2R status and treatment response in this age group.
Main Methods:
- Prospective observational study of 18 adolescent patients (10-19 years) with biopsy-proven PMN.
- Monthly monitoring of proteinuria, serum albumin, and creatinine.
- Serum aPLA2R testing at baseline, 6, and 12 months; glomerular aPLA2R staining performed.
Main Results:
- PMN was aPLA2R-related in 83% of adolescent cases.
- aPLA2R antibodies and enhanced glomerular staining were present in 77.78% and 72.22% of patients, respectively.
- Clinical remission was achieved in 61.11% at 6 months and 50% at 12 months, with a significant association between aPLA2R and remission/resistance.
Conclusions:
- Over three-quarters of adolescent PMN cases are associated with aPLA2R.
- Therapeutic response in adolescent PMN is observed in approximately half of the patients.
- aPLA2R monitoring is clinically significant and recommended for managing adolescent-onset PMN.
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