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Article

Frasier syndrome, a potential cause of end-stage renal failure in childhood.

Pediatric nephrology (Berlin, Germany) - 1 Mar 2010

Bache Manon, Dheu Céline, Doray Bérénice, Fothergill Hélène, Soskin Sylvie, Paris Françoise, Sultan Charles, Fischbach Michel

Abstract excerpt

The diagnosis of Frasier syndrome is based on the association of male pseudohermaphroditism (as a result of gonadal dysgenesis), with steroid-resistant nephrotic syndrome due to focal and segmental glomerular sclerosis (FSGS), which progresses to end-stage renal failure (ESRF) during adolescence or adulthood. Frasier syndrome results from mutations in the Wilms' tumour suppressor gene WT1, which is responsible...

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PMID: 19921279
DOI: 10.1007/s00467-009-1343-2

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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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