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Membranoproliferative glomerulonephritis is not commonly associated with disorders of sex development but has been recently identified as a <jats:italic>WT1<\/jats:italic>-associated nephropathy, but usually in cases of exonic mutations in either isolated Wilms tumor or Denys-Drash syndrome.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>The clinical and genetic data from 3 individuals are reported.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>This report describes the kidney manifestations in 3 individuals from 2 unrelated families with Frasier syndrome intronic <jats:italic>WT1<\/jats:italic> mutations, noting that 2 of the 3 individuals have histologically confirmed membranoproliferative glomerulonephritis.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>These case reports support expansion of the clinical spectrum of the kidney phenotypes associated with Frasier syndrome providing evidence of an association between <jats:italic>WT1<\/jats:italic> mutation and an immune complex-related membranoproliferative glomerulonephritis.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Graphical abstract<\/jats:title>\n                <jats:p>A higher resolution version of the Graphical abstract is available as Supplementary information<\/jats:p>\n                \n              <\/jats:sec>","DOI":"10.1007\/s00467-022-05421-8","type":"journal-article","created":{"date-parts":[[2022,2,24]],"date-time":"2022-02-24T19:02:42Z","timestamp":1645729362000},"page":"2369-2374","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["WT1 complete gonadal dysgenesis with membranoproliferative glomerulonephritis: case series and literature review"],"prefix":"10.1007","volume":"37","author":[{"given":"Erin","family":"Anderson","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Melanie","family":"Aldridge","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ross","family":"Turner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"James","family":"Harraway","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sam","family":"McManus","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"Stewart","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter","family":"Borzi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter","family":"Trnka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John","family":"Burke","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6303-6471","authenticated-orcid":false,"given":"David","family":"Coman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2022,2,24]]},"reference":[{"key":"5421_CR1","doi-asserted-by":"publisher","first-page":"1653","DOI":"10.1007\/s00467-006-0208-1","volume":"21","author":"P Niaudet","year":"2006","unstructured":"Niaudet P, Gubler M-C (2006) WT1 and glomerular diseases. 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