DiseaseID 3808

Camurati-Engelmann综合征

disease

NCI2016_02D:An autosomal dominant skeletal disorder caused by mutations in the TGFB1 gene. It is characterized by thickening of the bones, particularly the long bones of the extremities. It is associated with muscle weak

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Disease: 1Symptom: 12Target: 12Links: 24
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Record Fields

Scalar fields from the final disease record.

Disease Id
3808
Core Entity Id
60059
Source Entity Count
1
Preferred Name
Camurati-Engelmann Syndrome
Name Cn
Camurati-Engelmann综合征
Name Pinyin
Camurati-engelmann Zong He Zheng
Name En
Camurati-Engelmann Syndrome
Name Latin
Bilingual Status
complete
Disease Type
disease
Umls Disease Type
Disease or Syndrome
Disgenet Type
disease
Mesh Class
Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Musculoskeletal Diseases
Do Class
disease of anatomical entity; genetic disease
Hpo Class
Mesh Class Name
Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Musculoskeletal Diseases
Hpo Class Name
Do Class Name
genetic disease; disease of anatomical entity
Disease Definition
NCI2016_02D:An autosomal dominant skeletal disorder caused by mutations in the TGFB1 gene. It is characterized by thickening of the bones, particularly the long bones of the extremities. It is associated with muscle weakness and tiredness.|MSH2017_2016_08_12:An autosomal dominant form of dysplasia that is characterized by progressive thickening of diaphyseal cortex of long bones. Mutations in the gene that encodes TRANSFORMING GROWTH FACTOR BETA1 are one cause of this disorder.|HPO2016_07_04:An elevation in bone density in one or more diaphyses. Sclerosis is normally detected on a radiograph as an area of increased opacity. [HPO:probinson]
Version
v2
Suppressed
No

Names

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

Name
Camurati-Engelmann Syndrome
Role
preferred
Name
Camurati-Engelmann Disease
Role
alias
Name
Progressive Diaphyseal Dysplasia
Role
alias

Cross References

Trusted external identifiers retained for this final record.

Herb
HBDIS000788
Me Sh
D003966
Omim
131300
Icd10
Q78.3
Sym Map
SMDE06708
Do Class
DOID:630DOID:7
Dis Ge Net
C0011989
Umls Sty
T047
Me Sh Class
C05C16
Tcmbank Disease
4138

Attributes

Merged source attributes and domain-specific metadata.

Version
v2
Suppress
0
Do Class Name
genetic disease; disease of anatomical entity
Disease Type
disease
Do Disease Class
disease of anatomical entity; genetic disease
Umls Disease Type
Disease or Syndrome
Disease Definition
NCI2016_02D:An autosomal dominant skeletal disorder caused by mutations in the TGFB1 gene. It is characterized by thickening of the bones, particularly the long bones of the extremities. It is associated with muscle weakness and tiredness.|MSH2017_2016_08_12:An autosomal dominant form of dysplasia that is characterized by progressive thickening of diaphyseal cortex of long bones. Mutations in the gene that encodes TRANSFORMING GROWTH FACTOR BETA1 are one cause of this disorder.|HPO2016_07_04:An elevation in bone density in one or more diaphyses. Sclerosis is normally detected on a radiograph as an area of increased opacity. [HPO:probinson]
Me Sh Disease Class
Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Musculoskeletal Diseases
Dis Ge Net Disease Type
disease
Disease Class Name Me Sh
Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Musculoskeletal Diseases
Umls Semantic Type Name
Disease or Syndrome