Membranoproliferative Glomerulonephritis (MPGN) in PEDSnet

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dc.contributorNational Institutes of Health
dc.contributor.authorDenburg, Michelle
dc.contributor.otherChildren's Hospital of Philadelphia
dc.date.accessioned2024-07-09T19:50:28Z
dc.date.available2024-07-09T19:50:28Z
dc.descriptionC3 Glomerulopathy (C3G) and Immune Complex-associated Membranoproliferative Glomerulonephritis (IC-MPGN) are diseases with distinct underlying pathophysiologic mechanisms. C3G is widely recognized to be due to defective regulation of the alternative complement pathway, whereas IC-MPGN is associated with classical complement pathway activation by circulating or in situ immune complex formation. Historically, however, these diseases were categorized as various subtypes of MPGN by their histological appearance by electron microscopy, which led to uncertainty as to the precise nature of their disease biology, as well as optimal treatment strategies. The literature describing the clinical characteristics, treatment response, and outcomes of patients with C3G and IC-MPGN are largely based on small retrospective studies, and primarily in adults. Extraction and rigorous phenotyping of C3G and IC-MPGN patients from a large multi-institutional data resource offers the opportunity to more fully characterize the natural history of C3G and IC-MPGN in children, as well as identify predictors of disease response to therapeutic interventions (both complement-targeted and immunosuppressive therapies). #### Hypothesis The purpose of this study is to identify and conduct phenotypic characterization of children with C3G and IC-MPGN in PEDSnet using data science and chart review methods. The long-term goal of this work is to perform a large multi-institutional natural history study, compare clinical outcomes in C3G and IC-MPGN, and identify predictors of disease response or progression. It is hypothesized that among pediatric patients with C3G and IC- will be identifiable subset(s) of patients who have optimal response to immunosuppression regimens and/or complement-targeted therapies. Patient sub-sets will be characterized by and histological, immunological, and clinical characteristics. #### Cohort Description Patients identified by the GLEAN algorithm with a diagnosis of MPGN.
dc.description.abstractStudy to identify and conduct phenotypic characterization of children with C3G and IC-MPGN in PEDSnet using data science and chart review methods. The long-term goal of this work is to perform a large multi-institutional natural history study, compare clinical outcomes in C3G and IC-MPGN, and identify predictors of disease response or progression.
dc.identifier.urihttps://pedsnet.org/metadata/handle/20.500.14642/717
dc.publisherPEDSnet
dc.relationCyclophosphamide
dc.relationRituximab
dc.relationAzathioprine
dc.relationAbatacept
dc.relationMycophenolate
dc.relationMembranoproliferative GN and C3 Glomerulopathy
dc.relationSystemic Steroids
dc.relationTacrolimus and Cyclosporine
dc.relationGlomerular Disease, SNOMED-Only Codes
dc.relation.isreferencedbyDenburg MR, Razzaghi H, Bailey C, Soranno DE, et al. December 2019. "Using Electronic Health Record Data to Rapidly Identify Children with Glomerular Disease for Clinical Research." *JASN* 30(12):p 2427-2435. DOI: [10.1681/ASN.2019040365](doi.urg/10.1681/ASN.2019040365)
dc.rightsa CC-BY 4.0 Attribution license.
dc.rights.urihttps://creativecommons.org/licenses/by-sa/4.0/
dc.subjectStudy::Funded Study::PEDSnet Study
dc.subjectStudy::Funded Study::GLEAN Study
dc.subjectStudy::Funded Study::P50
dc.subject.meshGlomerulonephritis, Membranoproliferative
dc.subject.meshGlomerulonephritis
dc.subject.meshNephritis
dc.subject.meshImmune System Diseases
dc.titleMembranoproliferative Glomerulonephritis (MPGN) in PEDSnet
dspace.entity.typeStudy
local.admin.notehttps://chop365.sharepoint.com/:f:/r/teams/RSCH-ACRC/Shared%20Documents/PEDSnet/PEDSnet%20Studies/Active%20Studies/Denburg_Furth%20PEDSnet%20Projects/Furth_P50%20Original%20Award/MPGN?csf=1&web=1&e=1Ls9jl
local.subject.flatPEDSnet Data Source
local.subject.flatNIH P50
local.subject.flatFederally Funded Research
local.subject.flatRetrospective Study
local.subject.flatCohort Study
project.endDate2021-08
project.startDate2019-06
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Glomerular Learning Network (GLEAN)