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Article

Clinical spectrum of Denys-Drash and Frasier syndrome.

Pediatric nephrology (Berlin, Germany) - 1 Apr 2001

McTaggart S J, Algar E, Chow C W, Powell H R, Jones C L

Abstract excerpt

Denys-Drash syndrome (DDS) and Frasier syndrome (FS) are two related conditions caused by mutations of the Wilms tumor gene, WT1. Both syndromes are characterized by male pseudohermaphroditism, a progressive glomerulopathy, and the development of genitourinary tumors. DDS and FS have previously been distinguished by differences in nephropathy, with DDS patients demonstrating diffuse mesangial sclerosis (DMS) in...

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PMID: 11354777
DOI: 10.1007/s004670000541

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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WT1 gene mutations in three girls with nephrotic syndrome.Semantic proximity Read ↗

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