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M-type phospholipase A2 receptor (PLA2R) glomerular staining in pediatric idiopathic membranous nephropathy
Shoichiro Kanda1, Shigeru Horita2, Takeshi Yanagihara3
1Department of Pediatric Nephrology, School of Medicine, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan.
Background:
Identifying M-type phospholipase A2 receptor (PLA2R) is a landmark breakthrough for understanding adult idiopathic membranous nephropathy (iMN). However, potential roles for PLA2R in pediatric iMN have not been well investigated.
Methods:
A total of 34 pediatric iMN patients who underwent kidney biopsy between 1972 and 2015 were enrolled in this study. The study cohort consisted of 15 children aged from 3 to 9 years and 19 aged from 10 to 15 years. In all cases, secondary causes of MN, including infections, autoimmune diseases, and others, were ruled out. We examined PLA2R glomerular staining in stored, formalin-fixed, paraffin-embedded kidney biopsy samples. Kidney biopsy specimens obtained from an adult patient with iMN and an adult patient with lupus-associated MN were also examined to assess our PLA2R staining procedure.
Results:
Granular staining of PLA2R along glomerular capillary loops was present in two patients: an 11-year-old girl and 12-year-old boy identified during a school urine screening test and who presented with mild proteinuria at the time of biopsy. Interestingly, the intensity of PLA2R glomerular staining in these patients was weaker than that of a PLA2R-positive adult iMN patient. There were no PLA2R-positive patients among our cohort of children younger than 10 years.
Conclusions:
This preliminary study suggests PLA2R may play a role in some adolescent and preteen iMN patients but may be less frequently associated with iMN during childhood.
Insights
Phospholipase A2 receptor (PLA2R) may be involved in adolescent idiopathic membranous nephropathy (iMN). However, PLA2R is less frequently associated with iMN in younger children, indicating a potential age-related role.
Area of Science:
- Nephrology
- Immunology
- Pediatrics
Background:
- Idiopathic membranous nephropathy (iMN) in adults is strongly linked to M-type phospholipase A2 receptor (PLA2R).
- The role of PLA2R in pediatric iMN remains largely uninvestigated.
- Understanding PLA2R's involvement in childhood iMN is crucial for diagnosis and treatment.
Purpose of the Study:
- To investigate the presence and significance of PLA2R in pediatric idiopathic membranous nephropathy.
- To determine if PLA2R is associated with iMN in children across different age groups.
Main Methods:
- Analyzed kidney biopsy samples from 34 pediatric iMN patients (ages 3-15).
- Excluded secondary causes of membranous nephropathy.
- Performed glomerular PLA2R staining on formalin-fixed, paraffin-embedded samples.
- Used adult iMN and lupus nephritis samples as controls.
Main Results:
- PLA2R glomerular staining was detected in two adolescent patients (11 and 12 years old) with mild proteinuria.
- Staining intensity in these pediatric cases was weaker compared to adult iMN.
- No PLA2R positivity was observed in children younger than 10 years.
Conclusions:
- PLA2R may play a role in adolescent and preteen idiopathic membranous nephropathy.
- PLA2R-associated iMN appears less common in younger children.
- Further research is needed to elucidate the age-dependent role of PLA2R in iMN.
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