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Frasier syndrome

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Frasier syndrome

For the anatomical abnormality observed in 1965, see Fraser syndrome.
Frasier syndrome
Classification and external resources
OMIM DiseasesDB MeSH D052159

Frasier syndrome is a urogenital anomaly associated with the WT1 gene.[1]

It was first characterized in 1964.[2]


Frasier syndrome presents at birth with male pseudohermaphroditism (the external genitalia have a female appearance despite an XY genotype), streak gonads and progressive glomerulonephropathy (focal segmental glomerulosclerosis). Patients are also at increased risk of genito-urinary tumors (usually gonadoblastoma).

The glomerulonephropathy presents later than in Denys-Drash syndrome, and the tumour risk phenotype is different - whilst DDS is associated with Wilms' tumour, Frasier syndrome is associated with gonadoblastoma. Differentiating between the two syndromes can be challenging.[3]

See also


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