- Denys-Drash syndrome
Name = PAGENAME
DiseasesDB = 31499
OMIM = 194080
eMedicineSubj = ped
eMedicineTopic = 564
MeshName = Denys-Drash+Syndrome
MeshNumber = C04.557.435.595.220
Denys-Drash syndrome is a
syndromecharacterized by the following conditions:
Denys-Drash Syndrome (DDS) is a very rare disorder. The characteristics of DDS include loss of playfulness, loss of appetite, loss of sleep and the inability to urinate properly.
The causes of DDS is most commonly (96% of patients) an abnormality in the WT1 gene (Wilms' tumor suppressor gene). These abnormalities include changes in certain exons (9 and 8) and mutations in some alleles of the WT1 gene. Symptoms of DDS include decreased activity, low weight, loss of growth, abnormal skeletal development, abdominal pain and severe constipation.
The Wilm's tumor suppressor gene, WT1, is on
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