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Article

Bilateral Glomerulocystic Kidney Disease With Extensive Embryonal Hyperplasia in a Setting of HNF1B Mutation.

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society - 1 Jan 2026

Havens Jacob A, Hill Benjamin C, Feig Daniel I, Treece Amy L

Abstract excerpt

Glomerulocystic renal disease has numerous etiologies, including HNF1B mutations. In addition to cysts, morphologic renal findings in a setting of HNF1B 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...

Read the complete abstract on PubMed

Topics

Humans
Hepatocyte Nuclear Factor 1-beta
Hyperplasia
Kidney
Male
Female

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Germ-Line Mutation

Kidney Diseases, Cystic

Mutation

Source

Identifiers

PMID: 41731920
DOI: 10.1177/10935266261426188

Genes

HNF1B

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 24 Oct 2026.

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