DiseaseID 26484

Perlman综合征

Perlman Syndrome

NCI2016_02D:A rare, autosomal recessive syndrome characterized by the presence of polyhydramnios, neonatal macrosomia, craniofacial abnormalities, nephroblastomatosis, and predisposition to Wilms tumor. The prognosis is

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Disease: 1Symptom: 4Target: 5Links: 9
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Record Fields

Scalar fields from the final disease record.

Disease Id
26484
Core Entity Id
119052
Source Entity Count
1
Preferred Name
Perlman Syndrome
Name Cn
Perlman综合征
Name Pinyin
Perlman Zong He Zheng
Name En
Perlman Syndrome
Name Latin
Bilingual Status
complete
Disease Type
Umls Disease Type
Disgenet Type
Mesh Class
Do Class
Hpo Class
Mesh Class Name
Hpo Class Name
Do Class Name
Disease Definition
NCI2016_02D:A rare, autosomal recessive syndrome characterized by the presence of polyhydramnios, neonatal macrosomia, craniofacial abnormalities, nephroblastomatosis, and predisposition to Wilms tumor. The prognosis is poor.|JABL99:A syndrome of renal dysplasia with Wilms tumor, fetal gigantism, fetal ascites, macrosomia, hyperplasia of the endocrine pancreas, typical facial appearance, and mental retardation in those who survive past early infancy. The phenotype varies and some clinical features overlap those of the Beckwith-Wiedemann syndrome
Version
v1,v2
Suppressed
No

Names

Preferred names, aliases, and source labels retained in the final schema.

Name
Perlman Syndrome
Role
preferred
Source
SymMap_v2
Preferred
Yes

Cross References

Trusted external identifiers retained for this final record.

Umls
C0796113
Sym Map
SMDE03704

Attributes

Merged source attributes and domain-specific metadata.

Version
v1,v2
Suppress
0
Disease Definition
NCI2016_02D:A rare, autosomal recessive syndrome characterized by the presence of polyhydramnios, neonatal macrosomia, craniofacial abnormalities, nephroblastomatosis, and predisposition to Wilms tumor. The prognosis is poor.|JABL99:A syndrome of renal dysplasia with Wilms tumor, fetal gigantism, fetal ascites, macrosomia, hyperplasia of the endocrine pancreas, typical facial appearance, and mental retardation in those who survive past early infancy. The phenotype varies and some clinical features overlap those of the Beckwith-Wiedemann syndrome