DiseaseID 11928
德尼-德拉什综合征
disease
NCI2016_NICHD_1602D:A condition comprising congenital nephrotic syndrome, developmental sex disorder and Wilms tumor; associated with mutation(s) in the WT1 gene.|NCI2016_NCI-GLOSS_1602D:A rare disorder that causes kidne
Relationship Network
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Disease: 1Formula: 19Herb: 12Symptom: 1Target: 17Links: 56
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Record Fields
Scalar fields from the final disease record.
- Disease Id
- 11928
- Core Entity Id
- 69378
- Source Entity Count
- 1
- Preferred Name
- Denys-Drash Syndrome
- Name Cn
- 德尼-德拉什综合征
- Name Pinyin
- De Ni - De La Shen Zong He Zheng
- Name En
- Denys-Drash Syndrome
- Name Latin
- Bilingual Status
- complete
- Disease Type
- disease
- Umls Disease Type
- Disease or Syndrome
- Disgenet Type
- disease
- Mesh Class
- Female Urogenital Diseases and Pregnancy Complications; Neoplasms; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Male Urogenital Diseases; Endocrine System Diseases
- Do Class
- genetic disease
- Hpo Class
- Mesh Class Name
- Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Neoplasms; Female Urogenital Diseases and Pregnancy Complications; Male Urogenital Diseases; Endocrine System Diseases
- Hpo Class Name
- Do Class Name
- genetic disease
- Disease Definition
- NCI2016_NICHD_1602D:A condition comprising congenital nephrotic syndrome, developmental sex disorder and Wilms tumor; associated with mutation(s) in the WT1 gene.|NCI2016_NCI-GLOSS_1602D: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 cancer.|NCI2016_02D:A rare congenital syndrome caused by mutations in the WT1 gene. It is characterized by the presence of congenital nephropathy (diffuse mesangial sclerosis), Wilms tumor, and intersex disorders.|MSH2017_2016_08_12:A disorder of sex development characterized by UROGENITAL ABNORMALITIES; GONADAL DYSGENESIS; and WILMS TUMOR. It is caused by a mutation in the Wilms tumor suppressor gene (GENES, WILMS TUMOR) on chromosome 11.
- Version
- v1,v2
- Suppressed
- No
Names
Preferred names, aliases, and source labels retained in the final schema.
Name
Denys-Drash Syndrome
Role
preferred
Name
DDS
Role
alias
Name
DRASH SYNDROME
Role
alias
Name
NEPHROPATHY, WILMS TUMOR, AND GENITAL ANOMALIES
Role
alias
Name
WILMS TUMOR AND PSEUDO- OR TRUE HERMAPHRODITISM
Role
alias
Cross References
Trusted external identifiers retained for this final record.
Herb
HBDIS012571
Me Sh
D030321
Omim
194080
Umls
C0950121
Sym Map
SMDE02517
Do Class
DOID:630
Dis Ge Net
C0950121
Umls Sty
T047
Me Sh Class
C04C12C13C16C19
Etcm Disease
Denys-Drash Syndrome
Tcmbank Disease
11890
Itcmdb Generated
ITX-DISEASE-5B1F2E9A5CE7
Attributes
Merged source attributes and domain-specific metadata.
Version
v1,v2
Suppress
0
Page Title
Disease Denys-Drash Syndrome Details page
Do Class Name
genetic disease
Disease Type
disease
Do Disease Class
genetic disease
Umls Disease Type
Disease or Syndrome
Basic Information
Disease Name
Denys-Drash Syndrome
Global Category
Cancer diseases;Fetal diseases;Genetic diseases;Rare diseases
Anatomical Category
Endocrine diseases;Nephrological diseases;Reproductive diseases
Disease Definition
NCI2016_NICHD_1602D:A condition comprising congenital nephrotic syndrome, developmental sex disorder and Wilms tumor; associated with mutation(s) in the WT1 gene.|NCI2016_NCI-GLOSS_1602D: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 cancer.|NCI2016_02D:A rare congenital syndrome caused by mutations in the WT1 gene. It is characterized by the presence of congenital nephropathy (diffuse mesangial sclerosis), Wilms tumor, and intersex disorders.|MSH2017_2016_08_12:A disorder of sex development characterized by UROGENITAL ABNORMALITIES; GONADAL DYSGENESIS; and WILMS TUMOR. It is caused by a mutation in the Wilms tumor suppressor gene (GENES, WILMS TUMOR) on chromosome 11.
Me Sh Disease Class
Female Urogenital Diseases and Pregnancy Complications; Neoplasms; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Male Urogenital Diseases; Endocrine System Diseases
Dis Ge Net Disease Type
disease
Disease Class Name Me Sh
Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Neoplasms; Female Urogenital Diseases and Pregnancy Complications; Male Urogenital Diseases; Endocrine System Diseases
Umls Semantic Type Name
Disease or Syndrome