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A 1994 review of 150 cases reported in the literature found that 38% had died with a mean age of death of 2 years. 32% were still alive at the time of the report with a mean age of 4.65. No data were available for the remainder. The author described living with DDS as "walking a multidimensional
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da Silva TE, Nishi MY, Costa EM, et al. (August 2011). "A novel WT1 heterozygous nonsense mutation (p.K248X) causing a mild and slightly progressive nephropathy in a 46,XY patient with Denys–Drash syndrome".
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The condition can be inherited in an autosomal dominant pattern, but most cases occur due to new genetic mutations in people with no family history of the disorder.
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282:(9 and 8) and mutations in some alleles of the WT1 gene. Genetically, the syndrome is due to mutations in the Wilms tumor suppressor gene, WT1, which is on
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551:(1970). "A syndrome of pseudohermaphroditism, Wilms' tumor, hypertension, and degenerative renal disease".
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286:(11p13). These mutations are usually found in exons 8 or 9, but at least one has been reported in exon 4.
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The presenting characteristics of DDS include loss of playfulness, decreased appetite, weight loss,
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and undescended testes. Females with Denys-Drash syndrome typically have normal genitalia.
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gene (Wilms tumor suppressor gene). These abnormalities include changes in certain
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The cause of DDS is most commonly (96% of patients) an abnormality in the
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Denys P, Malvaux P, Van Den Berghe H, Tanghe W, Proesmans W (1967). "".
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and progresses to mesangial renal sclerosis, and ultimately
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Clinically, Denys–Drash is characterized by the triad of
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57:. Unsourced material may be challenged and removed.
1629:Ectrodactyly–ectodermal dysplasia–cleft syndrome 3
240:—usually within the first three years of life.
1521:Yemenite deaf-blind hypopigmentation syndrome
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396:Genetic and Rare Diseases Information Center
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232:. The condition first manifests as early
117:Learn how and when to remove this message
1125:Posterior polymorphous corneal dystrophy
955:Autoimmune polyendocrine syndrome type 1
243:Males with Denys-Drash syndrome exhibit
1316:Anterior segment mesenchymal dysgenesis
380:
386:
384:
848:X-linked adrenal hypoplasia congenita
7:
900:Greig cephalopolysyndactyly syndrome
55:adding citations to reliable sources
1274:Iridogoniodysgenesis, dominant type
25:
1721:Transcription factor deficiencies
254:, abnormal skeletal development,
1288:Lymphedema–distichiasis syndrome
884:Tricho–rhino–phalangeal syndrome
858:Familial partial lipodystrophy 3
300:
31:
1731:Syndromes affecting the kidneys
1552:(0) Other transcription factors
843:Estrogen insensitivity syndrome
811:Androgen insensitivity syndrome
42:needs additional citations for
1433:Hyperimmunoglobulin E syndrome
838:PHA1AD pseudohypoaldosteronism
349:first described the syndrome.
1:
1695:Atrichia with papular lesions
566:10.1016/S0022-3476(70)80409-7
1402:Popliteal pterygium syndrome
1344:Enlarged vestibular aqueduct
1183:Waardenburg syndrome 1&3
968:(3) Helix-turn-helix domains
727:relating to deficiencies of
1502:Premature ovarian failure 7
1358:Premature ovarian failure 3
1230:Congenital hypothyroidism 2
466:Journal of Medical Genetics
460:Mueller, R F (1994-06-01).
359:Beckwith–Wiedemann syndrome
1757:
1649:Transcription coregulators
1419:with minor groove contacts
806:Thyroid hormone resistance
547:, Sherman F, Hartmann WH,
462:"The Denys-Drash syndrome"
166:obstetrics and gynaecology
1671:Rubinstein–Taybi syndrome
1497:SRY XY gonadal dysgenesis
1302:Bamforth–Lazarus syndrome
914:Duane-radial ray syndrome
863:SF1 XY gonadal dysgenesis
431:10.1007/s00467-011-1847-4
1539:Cleidocranial dysostosis
756:Saethre–Chotzen syndrome
1516:Waardenburg syndrome 4c
1216:Coloboma of optic nerve
1064:Tooth and nail syndrome
904:Pallister–Hall syndrome
1459:Ulnar–mammary syndrome
1417:(4) β-Scaffold factors
1397:Van der Woude syndrome
929:Townes–Brocks syndrome
802:Intracellular receptor
392:"Denys-Drash syndrome"
309:This section is empty.
196:is a rare disorder or
66:"Denys–Drash syndrome"
1736:Syndromes with tumors
1633:Limb–mammary syndrome
1621:Rapp–Hodgkin syndrome
1593:Pitt–Hopkins syndrome
1169:Papillorenal syndrome
1144:Mowat–Wilson syndrome
1050:Nail–patella syndrome
934:Acrocallosal syndrome
228:renal sclerosis, and
222:pseudohermaphroditism
1475:Campomelic dysplasia
1454:Li–Fraumeni syndrome
939:Myotonic dystrophy 2
909:Denys–Drash syndrome
729:transcription factor
478:10.1136/jmg.31.6.471
186:Denys–Drash syndrome
135:Denys–Drash syndrome
51:improve this article
18:Denys-drash syndrome
1741:Intersex variations
1269:Axenfeld syndrome 3
1083:Axenfeld syndrome 1
1022:SPD1 synpolydactyly
787:DNA-binding domains
1625:Hay–Wells syndrome
1449:Holt–Oram syndrome
1211:Gillespie syndrome
1130:Fuchs' dystrophy 3
1008:Currarino syndrome
657:External resources
519:Arch. Fr. Pediatr.
258:, abdominal pain,
245:gonadal dysgenesis
234:nephrotic syndrome
216:Signs and symptoms
202:gonadal dysgenesis
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989:Ohtahara syndrome
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833:Kennedy's disease
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751:Feingold syndrome
737:(1) Basic domains
725:Genetic disorders
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200:characterized by
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129:Medical condition
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16:(Redirected from
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994:Lissencephaly X2
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879:Barakat syndrome
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419:Pediatr. Nephrol
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1566:Kabuki syndrome
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785:(2) Zinc finger
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772:Tietz syndrome
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606:Classification
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585:External links
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560:(4): 585–593.
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347:Allan L. Drash
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320:September 2021
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238:kidney failure
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364:WAGR syndrome
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345:P. Denys and
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337:tight rope".
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158:endocrinology
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68: –
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62:Find sources:
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40:This article
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1682:Corepressor:
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1657:Coactivator:
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399:. Retrieved
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369:Wilms' tumor
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313:adding to it
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260:constipation
252:growth delay
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230:Wilms' tumor
219:
210:Wilms' tumor
193:
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49:Please help
44:verification
41:
554:J. Pediatr.
549:Blizzard RM
401:22 February
206:nephropathy
140:Other names
107:August 2020
1715:Categories
642:DiseasesDB
375:References
77:newspapers
1579:Ungrouped
666:eMedicine
486:0022-2593
332:Prognosis
293:Mechanism
226:mesangial
149:Specialty
677:Orphanet
447:42090144
439:21559934
353:See also
256:insomnia
198:syndrome
154:Oncology
1330:ACD/MPV
671:ped/564
636:D030321
574:4316066
545:Drash A
531:4292870
504:8071974
495:1049926
341:History
162:urology
91:scholar
1665:CREBBP
1485:MODY 5
1480:MODY 3
1244:STHAG3
1197:MODY 9
1105:POU3F4
1097:DFNA15
1091:POU4F3
1036:MODY 4
1016:HOXD13
924:MRX 89
919:MODY 7
853:MODY 1
625:194080
597:CHORUS
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264:anuria
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592:00527
443:S2CID
280:exons
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1587:TCF4
1532:4.11
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1224:PAX8
1205:PAX6
1191:PAX4
1177:PAX3
1163:PAX2
1138:ZEB2
1119:ZEB1
1069:OFC5
1058:MSX1
1030:PDX1
1002:MNX1
826:CAIS
821:MAIS
816:PAIS
631:MeSH
620:OMIM
570:PMID
527:PMID
500:PMID
482:ISSN
435:PMID
403:2023
70:news
1559:0.6
1491:SF1
1468:4.7
1442:4.3
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