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Mechanism of Kemeng Fang's Inhibition of Podocyte Apoptosis in Rats with Membranous Nephropathy through the PI3K/AKT Signaling Pathway
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Mechanism of Kemeng Fang's Inhibition of Podocyte Apoptosis in Rats with Membranous Nephropathy through the PI3K/AKT Signaling Pathway

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Clinical and Histopathologic Characteristics of Pediatric Patients With Primary Membranous Nephropathy

Anne M Kouri1, Tiffany N Caza2, Laurence H Beck3

  • 1Division of Pediatric Nephrology, University of Minnesota Masonic Children's Hospital, Minneapolis, Minnesota, USA.

Kidney International Reports
|November 29, 2023
PubMed
Abstract

Insights

Pediatric primary membranous nephropathy (PMN) often involves novel antigens like PLA2R and EXT1. Children with these antigens show better remission rates, indicating a generally good kidney prognosis.

Area of Science:

  • Nephrology
  • Pediatric Nephrology
  • Immunology

Background:

  • Primary membranous nephropathy (PMN) is rare in children, with limited data on its clinical course.
  • Recent research implicates novel antigens in PMN pathogenesis.
  • Histopathologic features of pediatric PMN are underrepresented in literature.

Purpose of the Study:

  • To analyze the clinical presentation and outcomes of pediatric PMN.
  • To identify novel antigens in pediatric PMN biopsy specimens.
  • To correlate novel antigen presence with clinical outcomes in children.

Main Methods:

  • Retrospective analysis of 21 pediatric PMN cases from three US centers.
  • Identification of novel antigens in patient biopsy specimens.
  • Comparison with a validation cohort of 127 pediatric PMN biopsy specimens.

Main Results:

  • Overall antigen positivity was 62-63% in both cohorts.
  • Phospholipase A2 receptor (PLA2R) and exostosin 1 (EXT1) were the most common antigens.
  • 17 of 21 children achieved complete or partial remission; PLA2R/EXT1 positivity correlated with higher remission rates.

Conclusions:

  • Approximately 60% of pediatric membranous nephropathy cases show novel antigen positivity.
  • The overall clinical prognosis for pediatric PMN is generally favorable.
  • Further research is needed to clarify the clinical implications of individual novel antigens.
Keywords:
exostosin 1 and 2 (EXT1/2)kidney biopsypediatric nephrologyphospholipase A2 receptor (PLA2R)primary membranous nephropathy

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