Denys–Drash syndrome

Also known as: DDS

Early-onset nephropathy, Wilms tumor and 46,XY disorder of sex development.

Medical specialty: Endocrinology
Subspecialty: Endocrinology / Pediatric endocrinology
Sex & age distribution: Male: affected (46,XY); Female: affected; Children: yes, infancy
Gene(s) involved: WT1

Etiology & mechanism

Dominant negative WT1 variants.

Gene(s) involved (1)

WT1

Symptoms & signs (6)

ambiguous genitaliadiffuse mesangial sclerosisgonadal dysgenesisnephrotic syndromerenal failurewilms tumor

SyndromeScope — educational reference. Content is model-generated and not clinically verified; always confirm against OMIM, Orphanet and GeneReviews before clinical use.