Denys-Drash syndrome


Denys-Drash syndrome

Infobox_Disease
Name = PAGENAME


Caption =
DiseasesDB = 31499
ICD10 =
ICD9 =
ICDO =
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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References

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Look at other dictionaries:

  • Denys–Drash syndrome — Classification and external resources OMIM 194080 DiseasesDB 31499 eMedicine …   Wikipedia

  • Denys-Drash syndrome — DDS. A rare disorder that causes kidney failure before age 3, abnormal development of the sexual organs, and, in most cases, Wilms’ tumor (a type of kidney cancer). Children with Denys Drash syndrome are also at high risk of some other types of… …   English dictionary of cancer terms

  • Denys-Drash syndrome — Den·ys Drash syndrome (denґis drash) [Joseph Denys, Belgian bacteriologist, 1857–1932; Allan Lee Drash, American pediatrician, born 1931] see under syndrome …   Medical dictionary

  • Denys-Drash syndrome — a rare syndrome that includes male pseudohermaphroditism, nephropathy leading to renal failure, and, in most cases, Wilms tumor. It is caused by a genetic abnormality in the p13 region of chromosome 11 …   Medical dictionary

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

  • Syndrome de Denys-Drash — Référence MIM 194080 Transmission Dominante Chromosome 11p13 Gène WT1 Mutation Ponctuelle …   Wikipédia en Français

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