Denys-Drash syndrome

Denys-Drash syndrome


Caption =
DiseasesDB = 31499
ICD10 =
ICD9 =
OMIM = 194080
MedlinePlus =
eMedicineSubj = ped
eMedicineTopic = 564
MeshName = Denys-Drash+Syndrome
MeshNumber = C04.557.435.595.220

Denys-Drash syndrome is a syndrome characterized by the following conditions:

* gonadal dysgenesis
* nephropathy
* Wilms tumor

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 chromosome 11 (11p13).

ee also

* WAGR syndrome
* Wilms' tumor

External links




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Look at other dictionaries:

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  • Syndrome de denys-drash — Autre nom {{{Autre nom}}} Référence MIM …   Wikipédia en Français

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