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In a considerable fraction of patients with <jats:italic>Wilms tumor 1<\/jats:italic> mutations, the distinctive histology of immune-complex-type glomerulonephritis has been reported. However, the clinical relevance and etiologic mechanisms remain unknown.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Case presentation<\/jats:title>\n<jats:p>A 5-year-old child presented with steroid-resistant nephrotic range proteinuria. Initial renal biopsy revealed predominant diffuse mesangial proliferation with a double-contour and coexisting milder changes of focal segmental glomerulosclerosis. Immunofluorescence and electron microscopy revealed a full-house-pattern deposition of immune complexes in the subendothelial and paramesangial areas. Serial biopsies at 6 and 8\u2009years of age revealed that more remarkable changes of focal segmental glomerulosclerosis had developed on top of the initial proliferative glomerulonephritis. Identification of a de novo <jats:italic>Wilms tumor 1<\/jats:italic> splice donor-site mutation in intron 9 (NM_024426.6:c.1447\u2009+\u20094C\u2009&gt;\u2009T) and 46,XY-gonadal dysgenesis led to the diagnosis of Frasier syndrome.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Conclusions<\/jats:title>\n<jats:p>Our findings, together with those of others, point to the importance of heterogeneity in clinicopathological phenotypes caused by <jats:italic>Wilms tumor 1<\/jats:italic> mutations and suggest that immune-complex-mediated membranoproliferative glomerulopathy should be considered as a histological variant.<\/jats:p>\n<\/jats:sec>","DOI":"10.1186\/s12882-020-02007-0","type":"journal-article","created":{"date-parts":[[2020,8,24]],"date-time":"2020-08-24T14:03:06Z","timestamp":1598277786000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Immune-complex glomerulonephritis with a membranoproliferative pattern in Frasier syndrome: a case report and review of the literature"],"prefix":"10.1186","volume":"21","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2164-0555","authenticated-orcid":false,"given":"Daisuke","family":"Matsuoka","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shunsuke","family":"Noda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Motoko","family":"Kamiya","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshihiko","family":"Hidaka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hisashi","family":"Shimojo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasushi","family":"Yamada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tsutomu","family":"Miyamoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kandai","family":"Nozu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazumoto","family":"Iijima","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9563-5356","authenticated-orcid":false,"given":"Hiroyasu","family":"Tsukaguchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,8,24]]},"reference":[{"key":"2007_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 MC. 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