Denys-Drash syndrome

GPTKB entity

Statements (25)
Predicate Object
gptkbp:instanceOf gptkb:disease
genetic disorder
gptkbp:affectsOrgan gptkb:kidney
gonads
gptkbp:complication renal failure
hypertension
gptkbp:firstDescribed 1967
https://www.w3.org/2000/01/rdf-schema#label Denys-Drash syndrome
gptkbp:ICD-10_code Q96.8
gptkbp:inheritance sporadic
gptkbp:mutationAssociatedWith gptkb:WT1_gene
gptkbp:namedAfter Albert Drash
Frederick Denys
gptkbp:OMIM 194080
gptkbp:onset childhood
gptkbp:prevalence rare
gptkbp:prognosis poor without treatment
gptkbp:symptom gptkb:Wilms_tumor
nephrotic syndrome
gonadal dysgenesis
gptkbp:treatment chemotherapy
kidney transplantation
nephrectomy
gptkbp:bfsParent gptkb:rhabdomyosarcoma
gptkbp:bfsLayer 5