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Article

Frasier syndrome: early gonadoblastoma and cyclosporine responsiveness.

Pediatric nephrology (Berlin, Germany) - 1 Oct 2010

Sinha Aditi, Sharma Sonika, Gulati Ashima, Sharma Alok, Agarwala Sandeep, Hari Pankaj, Bagga Arvind

Abstract excerpt

Frasier syndrome is characterized by progressive glomerulopathy that is unresponsive to corticosteroids, male pseudohermaphroditism, and an increased risk of genitourinary tumors. Of 21 girls with steroid-resistant nephrotic syndrome secondary to focal segmental glomerulosclerosis (FSGS) who were screened for mutations in the WT1 gene, two showed Frasier syndrome. Both patients had donor splice-site mutations in...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

Identifiers

PMID: 20419325
DOI: 10.1007/s00467-010-1518-x

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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