{"@context":{"@vocab":"https://cir.nii.ac.jp/schema/1.0/","rdfs":"http://www.w3.org/2000/01/rdf-schema#","dc":"http://purl.org/dc/elements/1.1/","dcterms":"http://purl.org/dc/terms/","foaf":"http://xmlns.com/foaf/0.1/","prism":"http://prismstandard.org/namespaces/basic/2.0/","cinii":"http://ci.nii.ac.jp/ns/1.0/","datacite":"https://schema.datacite.org/meta/kernel-4/","ndl":"http://ndl.go.jp/dcndl/terms/","jpcoar":"https://github.com/JPCOAR/schema/blob/master/2.0/"},"@id":"https://cir.nii.ac.jp/crid/1363670321180620160.json","@type":"Article","productIdentifier":[{"identifier":{"@type":"DOI","@value":"10.1038/s41572-020-0196-7"}},{"identifier":{"@type":"URI","@value":"https://www.nature.com/articles/s41572-020-0196-7.pdf"}},{"identifier":{"@type":"URI","@value":"https://www.nature.com/articles/s41572-020-0196-7"}},{"identifier":{"@type":"DOI","@value":"10.1038/s41572-020-0208-7"}},{"identifier":{"@type":"PMID","@value":"32792477"}},{"identifier":{"@type":"PMID","@value":"32792490"}},{"identifier":{"@type":"HANDLE","@value":"2434/794566"}},{"identifier":{"@type":"HANDLE","@value":"2158/1222454"}}],"dc:title":[{"@value":"Podocytopathies"}],"description":[{"notation":[{"@value":"Podocytopathies are kidney diseases in which direct or indirect podocyte injury drives proteinuria or nephrotic syndrome. In children and young adults, genetic variants in50 podocyte-expressed genes, syndromal non-podocyte-specific genes and phenocopies with other underlying genetic abnormalities cause podocytopathies associated with steroid-resistant nephrotic syndrome or severe proteinuria. A variety of genetic variants likely contribute to disease development. Among genes with non-Mendelian inheritance, variants in APOL1 have the largest effect size. In addition to genetic variants, environmental triggers such as immune-related, infection-related, toxic and haemodynamic factors and obesity are also important causes of podocyte injury and frequently combine to cause various degrees of proteinuria in children and adults. Typical manifestations on kidney biopsy are minimal change lesions and focal segmental glomerulosclerosis lesions. Standard treatment for primary podocytopathies manifesting with focal segmental glomerulosclerosis lesions includes glucocorticoids and other immunosuppressive drugs; individuals not responding with a resolution of proteinuria have a poor renal prognosis. Renin-angiotensin system antagonists help to control proteinuria and slow the progression of fibrosis. Symptomatic management may include the use of diuretics, statins, infection prophylaxis and anticoagulation. This Primer discusses a shift in paradigm from patient stratification based on kidney biopsy findings towards personalized management based on clinical, morphological and genetic data as well as pathophysiological understanding."}]}],"creator":[{"@id":"https://cir.nii.ac.jp/crid/1383670321180620161","@type":"Researcher","foaf:name":[{"@value":"Jeffrey B. Kopp"}]},{"@id":"https://cir.nii.ac.jp/crid/1383670321180620160","@type":"Researcher","foaf:name":[{"@value":"Hans-Joachim Anders"}]},{"@id":"https://cir.nii.ac.jp/crid/1383670321180620163","@type":"Researcher","foaf:name":[{"@value":"Katalin Susztak"}]},{"@id":"https://cir.nii.ac.jp/crid/1383670321180620162","@type":"Researcher","foaf:name":[{"@value":"Manuel A. Podestà"}]},{"@id":"https://cir.nii.ac.jp/crid/1383670321180620164","@type":"Researcher","foaf:name":[{"@value":"Giuseppe Remuzzi"}]},{"@id":"https://cir.nii.ac.jp/crid/1383670321180620166","@type":"Researcher","foaf:name":[{"@value":"Friedhelm Hildebrandt"}]},{"@id":"https://cir.nii.ac.jp/crid/1383670321180620165","@type":"Researcher","foaf:name":[{"@value":"Paola Romagnani"}]}],"publication":{"publicationIdentifier":[{"@type":"EISSN","@value":"2056676X"}],"prism:publicationName":[{"@value":"Nature Reviews Disease Primers"}],"dc:publisher":[{"@value":"Springer Science and Business Media LLC"}],"prism:publicationDate":"2020-08-13","prism:volume":"6","prism:number":"1","prism:startingPage":"68"},"reviewed":"false","dcterms:accessRights":"http://purl.org/coar/access_right/c_abf2","dc:rights":["https://www.springernature.com/gp/researchers/text-and-data-mining","https://www.springernature.com/gp/researchers/text-and-data-mining"],"url":[{"@id":"https://www.nature.com/articles/s41572-020-0196-7.pdf"},{"@id":"https://www.nature.com/articles/s41572-020-0196-7"}],"createdAt":"2020-08-13","modifiedAt":"2024-12-11","foaf:topic":[{"@id":"https://cir.nii.ac.jp/all?q=Proteinuria","dc:title":"Proteinuria"},{"@id":"https://cir.nii.ac.jp/all?q=chronic%20kidney%20disease,%20podocyte,%20glomerulus,%20glomerulonephritis","dc:title":"chronic kidney disease, podocyte, glomerulus, glomerulonephritis"},{"@id":"https://cir.nii.ac.jp/all?q=Nephrotic%20Syndrome","dc:title":"Nephrotic Syndrome"},{"@id":"https://cir.nii.ac.jp/all?q=Podocytes","dc:title":"Podocytes"},{"@id":"https://cir.nii.ac.jp/all?q=Prevalence","dc:title":"Prevalence"},{"@id":"https://cir.nii.ac.jp/all?q=Humans","dc:title":"Humans"},{"@id":"https://cir.nii.ac.jp/all?q=Kidney","dc:title":"Kidney"}],"relatedProduct":[{"@id":"https://cir.nii.ac.jp/crid/1360013168880234880","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Genetic Deficiency of Adipose Triglyceride Lipase Is Associated With a Novel Type of Podocytopathy"}]},{"@id":"https://cir.nii.ac.jp/crid/1360016861548896640","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Protective effects of tadalafil on damaged podocytes in an adriamycin-induced nephrotic syndrome model"}]},{"@id":"https://cir.nii.ac.jp/crid/1360017282446386432","@type":"Article","resourceType":"学術雑誌論文(journal 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