Denys-Drash syndrome

Orpha code: 220OMIM code: 194080

Definition

A rare genetic, syndromic glomerular disorder characterized by the association of nephropathy presenting as persistent proteinuria or overt nephrotic syndrome, Wilms tumor and genitourinary structural defects. In addition, disorders of testicular development are common in subjects with 46,XY karyotype.

Disease data
Classification

Disease

Synonyms
Drash syndrome
Guz Wilmsa i pseudohermfrodytyzm
Zespół Drasha
Wilms tumor-DSD syndrome
Wilms tumor-disorder of sex development syndrome
ORPHA code
220
OMIM code
194080
ICD10 code
N04.1
ICD11 code
LD2A.Y

No additional description.

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