{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,11]],"date-time":"2026-09-11T00:06:23Z","timestamp":1789085183663,"version":"build-2803163510"},"reference-count":27,"publisher":"SAGE Publications","issue":"5","license":[{"start":{"date-parts":[[2026,2,23]],"date-time":"2026-02-23T00:00:00Z","timestamp":1771804800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Pediatr Dev Pathol"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:p>\n                    Glomerulocystic renal disease has numerous etiologies, including\n                    <jats:italic toggle=\"yes\">HNF1B<\/jats:italic>\n                    mutations. In addition to cysts, morphologic renal findings in a setting of\n                    <jats:italic toggle=\"yes\">HNF1B<\/jats:italic>\n                    mutations include cystic renal dysplasia, solitary functioning kidney, horseshoe kidney, and oligomeganephronia. Embryonal hyperplasia resembling nephrogenic rests has been reported in rare cases of glomerulocystic disease, but none have been genetically characterized. We report a case of bilateral glomerulocystic kidney disease (GCKD) showing extensive embryonal hyperplasia in a setting of germline\n                    <jats:italic toggle=\"yes\">HNF1B<\/jats:italic>\n                    mutation with progressive renal failure. Explant showed numerous epithelial proliferations throughout the intervening stroma. GCKD may be seen in a setting of\n                    <jats:italic toggle=\"yes\">HNF1B<\/jats:italic>\n                    mutation; however, the additional finding of extensive embryonal hyperplasia in a case with a known mutation has never been reported. Reports of similar embryonal hyperplasia, associated with either cystic kidney disease or other disease processes, appear to represent a heterogeneous population of presentations and etiologies, though there is sufficient evidence to suggest that this is a finding, that is, recurrently associated with cystic kidney diseases. The underlying pathobiology of embryonal hyperplasia and the neoplastic potential in this setting is unknown. We report this case to highlight a novel combination of morphologic and genetic findings in GCKD and to raise awareness of this rare finding.\n                  <\/jats:p>","DOI":"10.1177\/10935266261426188","type":"journal-article","created":{"date-parts":[[2026,2,24]],"date-time":"2026-02-24T06:10:22Z","timestamp":1771913422000},"page":"547-552","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Bilateral Glomerulocystic Kidney Disease With Extensive Embryonal Hyperplasia in a Setting of\n                    <i>HNF1B<\/i>\n                    Mutation"],"prefix":"10.1177","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-0214-088X","authenticated-orcid":false,"given":"Jacob A.","family":"Havens","sequence":"first","affiliation":[{"name":"Department of Pathology, The University of Alabama at Birmingham, AL, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin C.","family":"Hill","sequence":"additional","affiliation":[{"name":"Department of Pathology, Children\u2019s of Alabama, Birmingham, AL, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel I.","family":"Feig","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Pediatrics, The University of Alabama at Birmingham, AL, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy L.","family":"Treece","sequence":"additional","affiliation":[{"name":"Department of Pathology, Children\u2019s of Alabama, Birmingham, AL, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2026,2,23]]},"reference":[{"issue":"4","key":"e_1_3_2_2_2","first-page":"186","article-title":"Glomerulocystic kidney. Report of a case","volume":"100","author":"Taxy JB","year":"1976","unstructured":"Taxy JB, Filmer RB. Glomerulocystic kidney. Report of a case. Arch Pathol Lab Med. 1976;100(4):186-188.","journal-title":"Arch Pathol Lab Med"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00857576"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.5858\/134.4.583"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-012-2221-x"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-015-3142-2"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1038\/nrneph.2014.232"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1523-1755.2000.057003898.x"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1523-1755.2002.00272.x"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/8.11.2001"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.2215\/CJN.06810909"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-018-4188-8"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1001\/archpedi.1941.02000070125011"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.1097\/01.pap.0000201831.77472.d3"},{"key":"e_1_3_2_15_2","unstructured":"Nephropathology. (2021 June). Case 184 Diagnosis: Glomerulocystic Kidney Disease and Diffuse Nephroblasto-matosis. https:\/\/www.kidneypathology.com\/English_version\/Case_184.html"},{"key":"e_1_3_2_16_2","doi-asserted-by":"publisher","DOI":"10.1097\/01.asn.0000023431.16173.55"},{"key":"e_1_3_2_17_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01600289"},{"key":"e_1_3_2_18_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-002-1019-7"},{"issue":"1","key":"e_1_3_2_19_2","first-page":"71","article-title":"Studies on \u201cend-stage\u201d kidneys. II. Embryonal hyperplasia of Bowman\u2019s capsular epithelium","volume":"91","author":"Hughson MD","year":"1978","unstructured":"Hughson MD, McManus JF, Hennigar GR. Studies on \u201cend-stage\u201d kidneys. II. Embryonal hyperplasia of Bowman\u2019s capsular epithelium. Am J Pathol. 1978;91(1):71-84.","journal-title":"Am J Pathol"},{"key":"e_1_3_2_20_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-024-06293-w"},{"key":"e_1_3_2_21_2","doi-asserted-by":"publisher","DOI":"10.1007\/s100240010093"},{"key":"e_1_3_2_22_2","doi-asserted-by":"publisher","DOI":"10.4103\/jcrt.jcrt_275_22"},{"key":"e_1_3_2_23_2","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2006030277"},{"key":"e_1_3_2_24_2","doi-asserted-by":"publisher","DOI":"10.1093\/ndt\/gfm603"},{"key":"e_1_3_2_25_2","doi-asserted-by":"publisher","DOI":"10.1086\/316945"},{"key":"e_1_3_2_26_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-140-7-200404060-00009"},{"key":"e_1_3_2_27_2","doi-asserted-by":"publisher","DOI":"10.1177\/10935266241242173"},{"key":"e_1_3_2_28_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-009-1416-2"}],"container-title":["Pediatric and Developmental Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/10935266261426188","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/10935266261426188","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/10935266261426188","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T23:16:23Z","timestamp":1789082183000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/10935266261426188"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2,23]]},"references-count":27,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2026,9]]}},"alternative-id":["10.1177\/10935266261426188"],"URL":"https:\/\/doi.org\/10.1177\/10935266261426188","relation":{},"ISSN":["1093-5266","1615-5742"],"issn-type":[{"value":"1093-5266","type":"print"},{"value":"1615-5742","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2,23]]}}}