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

Specialty
Endocrinology
Subspecialty
Endocrinology / Pediatric endocrinology
Sex & age distribution
Male: affected (46,XY); Female: affected; Children: yes, infancy
Gene(s) involved
WT1
Alternative names & eponyms
DDS

Etiology & mechanism

Dominant negative WT1 variants.

Gene(s) involved (1)

WT1

Symptoms & signs (6)

In plain terms, someone with Denys–Drash syndrome may experience the following — click any to explore related syndromes:

Same gene(s)

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Overlapping symptoms

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