Journal article
Clinical and Histopathologic Characteristics of Pediatric Patients with Primary Membranous Nephropathy
Kidney international reports, Vol.8(11), pp.2368-2375
11/2023
DOI: 10.1016/j.ekir.2023.08.018
PMCID: PMC10658230
PMID: 38025223
Abstract
Introduction
Primary membranous nephropathy (PMN) is uncommon in children. As such, data on the clinical course of affected children are scarce. In recent years, several novel antigens have been implicated in the pathogenesis of PMN. However, the histopathologic characteristics of pediatric patients with PMN remain poorly represented in the literature.
Methods
We have retrospectively analyzed the clinical presentation and outcomes data of 21 children with PMN from 3 centers in the United States. In addition, we have identified novel antigens in biopsy specimens from these patients and correlated their presence or absence to clinical outcomes. Finally, we compared the results of the novel antigen staining from our clinical cohort to a validation cohort of 127 biopsy specimens from children with PMN at Arkana Laboratories.
Results
The data from the two cohorts demonstrated similar overall antigen positivity rates of 62— 63%, with PLA2R and EXT1 being the most commonly found antigens. Results from the clinical cohort showed that overall, the kidney prognosis for children with PMN was good, with 17/21 patients entering a complete or partial remission. Children who were positive for PLA2R or EXT1 were significantly more likely to enter remission than those in the antigen negative group.
Conclusion
Approximately 60% of pediatric membranous cases are positive for a novel antigen on kidney biopsy and the clinical prognosis is generally favorable. More studies are needed to understand the clinical implications of each specific novel antigen.
Details
- Title: Subtitle
- Clinical and Histopathologic Characteristics of Pediatric Patients with Primary Membranous Nephropathy
- Creators
- Anne M. KouriTiffany N. CazaLaurence H. BeckJason M. MisuracMichael D. EvansCarrie L. PhillipsMichael T. EadonChristopher P. LarsenSharon P. AndreoliLihong BuMichelle N. RheaultMyda Khalid
- Resource Type
- Journal article
- Publication Details
- Kidney international reports, Vol.8(11), pp.2368-2375
- DOI
- 10.1016/j.ekir.2023.08.018
- PMID
- 38025223
- PMCID
- PMC10658230
- NLM abbreviation
- Kidney Int Rep
- ISSN
- 2468-0249
- eISSN
- 2468-0249
- Grant note
- DOI: 10.13039/100006108, name: National Center for Advancing Translational Sciences, award: UL1TR002494; DOI: 10.13039/100000002, name: National Institutes of Health
- Language
- English
- Electronic publication date
- 08/2023
- Date published
- 11/2023
- Academic Unit
- Nephrology, Dialysis and Transplantation; Stead Family Department of Pediatrics
- Record Identifier
- 9984458106102771
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