Disease #01611 (DDS (Denys-Drash syndrome (DDS)), OMIM:194080)

Official abbreviation DDS
Name Denys-Drash syndrome (DDS)
OMIM ID 194080
Human Phenotype Ontology Project (HPO) HPO
Inheritance Autosomal dominant, Somatic mutation
Individuals reported having this disease 16
Phenotype entries for this disease 16
Associated with 1 gene WT1
Associated tissues -
Disease features -
Remarks -
Date created 2014-09-25 23:29:40 +02:00 (CEST)
Date last edited 2021-12-10 21:51:32 +01:00 (CET)


Individuals

16 entries on 1 page. Showing entries 1 - 16.
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00204202 - Journal: Regev 2008 - M no Israel Indian - - - - DDS Son, 46, XY, ambiguous genitalia and ventricular septal defect. Laparoscopy performed at 1 year for undescended testes - bilateral fallopian tubes and right fimbriae, no uterus, left gonad looked like a testicle, right one like an ova-testis. No Wilms tumor at 6 years of age. WT1 WT1 1 1 Olga Beltcheva
00204211 - PubMed: Chernin 2010 - - - United States - - - - - DDS Renal biopsy WT1 WT1 1 1 Olga Beltcheva
00204212 - - - M - - - - - - - DDS Male (XY) with female genitalia with clitoromegaly, labial fusion. Gonadal hystology demonstrates the presence of testes. WT1 WT1 1 1 Olga Beltcheva
00204213 - PubMed: Clarkson;PubMed: Little - - - - - - - - - DDS Male - 46XY, cryptorchidism, perineal hypospadias, Wilms tumour and diffuse mesangial sclerosis WT1 WT1 1 1 Olga Beltcheva
00204214 - PubMed: Pelletier Right gonad-dysgenic testis; Left gonad-streak; No MQIlerian structures; No Wolffian structures - - - - - - - - DDS 46XY patient with nephropathy and gonadoblastoma WT1 WT1 1 1 Olga Beltcheva
00204215 - PubMed: Chernin 2010 - - - United States - - - - - DDS DMS WT1 WT1 1 1 LOVD
00204216 - PubMed: Pelletier No Mulerian structures; Left Wolffian structures - - - - - - - - DDS 46 XY patient woth ambiguous genitalia, Wilms tumor and gondaoblastoma WT1 WT1 1 1 Olga Beltcheva
00204217 - PubMed: Clarkson,PubMed: Baird Histological examination of renal biopsy showed diffuse mesangial sclerosis; laparotomy demostrated internal female genitalia M - - - - - - - DDS Male patient (46,XY) born with normal external female genitalia, presented with nephroticsyndrome. WT1 WT1 1 1 Olga Beltcheva
00204218 - PubMed: Pelletier - - - - - - - - - DDS Ambiguous genitalia, nephropathy WT1 WT1 1 1 Olga Beltcheva
00204219 - PubMed: Pelletier Right gonad-dysgenic testis; Left gonad-dysgenic testis; Bilateral Mu11erian, No Wolffian structures - - - - - - - - DDS 46 XY, Ambiguou genitalia, Wilms tumor, gonadoblastoma WT1 WT1 1 1 Olga Beltcheva
00204220 - PubMed: Pelletier - F - - - - - - - DDS Nephropathy, bilateral Wilms tumor WT1 WT1 1 1 Olga Beltcheva
00204221 - PubMed: Pelletier Right gonad-dysgenic testis; Left gonad-normal testis; Bicornuated uterus - - - - - - - - DDS 46XY patient with ambiguous, nepropathy, bilateral Wilms tumor WT1 WT1 1 1 Olga Beltcheva
00204222 - PubMed: Pelletier Normaol vary attached to anterior pole of kidney; normal uterus - - - - - - - - DDS 46XX patient with nepropathy and Wilms tumor WT1 WT1 1 1 Olga Beltcheva
00204223 - PubMed: Pelletier Right gonad-dysgenic; Left gonad-streak; Normal fallopian tubes, uterus and vagina - - - - - - - - DDS 46XX patient with nepropathy and Wilms tumor WT1 WT1 1 1 Olga Beltcheva
00204224 - PubMed: Pelletier Right gonads-dysgenic testis; Left gonad-dysgenic testis - - - - - - - - DDS 46XY patient with ambiguous genitalia, nepropathy and Wilms tumor WT1 WT1 1 1 Olga Beltcheva
00204226 - PubMed: Pelletier - - - - - - - - - DDS 46XX patient with nephropathy WT1 WT1 1 1 Olga Beltcheva
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