Preferred Name | Denys-Drash Syndrome | |
Synonyms |
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Definitions |
An autosomal dominant disease that is characterized by the association of diffuse mesangial sclerosis (DMS), male pseudohermaphroditism with a 46,XY karyotype, and nephroblastoma that derives_from an abnormality in the WT1 gene (Wilms' tumor suppressor gene). |
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ID |
http://www.phoc.org.cn/pmo/class/PMO_00038158 |
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Database_Cross_Reference |
RCD:X30I1 MTH:NOCODE OMIM:607102 MDR:10070179 NDFRT:N0000011086 MEDCIN:351160 OMIM:194080 SNOMEDCT_US:236385009 CHV:0000053528 NCI_NICHD:C84668 DO:DOID:3764 NCI_NCI-GLOSS:CDR0000440105 NCI_NCI-GLOSS:CDR0000440104 NCI:C84668 MSH:D030321 RCDAE:X30I1 |
|
Definition |
An autosomal dominant disease that is characterized by the association of diffuse mesangial sclerosis (DMS), male pseudohermaphroditism with a 46,XY karyotype, and nephroblastoma that derives_from an abnormality in the WT1 gene (Wilms' tumor suppressor gene). |
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label |
Denys-Drash Syndrome |
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MCID |
MC00337846 |
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PMOID |
PMO:00038158 |
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prefixIRI |
pmo:PMO_00038158 |
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prefLabel |
Denys-Drash Syndrome |
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Synonym |
DRASH SYNDROME WILMS TUMOR AND PSEUDO- OR TRUE HERMAPHRODITISM Drash syndrome Wilms' tumor + nephrotic syndrome + pseudohermaphroditism Nephropathy, Wilms Tumor, and Genital Anomalies Nephrotic syndrome with pseudohermaphroditism DENYS-DRASH SYNDROME NEPHROPATHY, WILMS TUMOR, AND GENITAL ANOMALIES Denys-Drash Syndrome [Disease/Finding] Wilms' tumor and nephrotic syndrome with pseudohermaphroditism denys-drash syndrome Syndrome, Denys-Drash Nephrotic Syndrome Type 4 nephrotic syndrome congenital type 4 (diagnosis) Wilms' tumour and nephrotic syndrome with pseudohermaphroditism Wilms' tumour + nephrotic syndrome + pseudohermaphroditism Wilms Tumor and Pseudohermaphroditism Pseudohermaphroditism, Nephron Disorder and Wilms' Tumor Wilms tum + NS + pseudohermaph Drash syndrome (disorder) Nephr synd +pseudohermaphrodit DDS Drash Syndrome denys drash syndrome nephrotic syndrome congenital type 4 Denys Drash Syndrome Syndrome, Drash Denys-Drash syndrome |
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Tree Number |
T9.24.4.6.2.4 T9.12.2.10.3.1.1.2 T9.24.1.3.1.1.2 T9.19.2.5.6.2.4 T9.8.4.36.2.2 T9.12.1.12.1.2 T9.24.2.3.12.1.2 T9.8.3.11.6.2.4 T9.19.2.1.3.1.1.2 T9.12.7.2.2 T9.9.9.5.2.4 T9.19.2.2.3.12.1.2 |
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subClassOf |
http://www.phoc.org.cn/pmo/class/PMO_00014295 |