DiseaseID 19157

Loeys-Dietz综合征2型

disease

NCI2016_02D:A rare autosomal dominant inherited disorder of connective tissue caused by mutations in either the TGFBR1 or TGFBR2 gene. Like Loeys-Dietz syndrome type I the disease is characterized by enlargement of the a

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Disease: 1Symptom: 1Target: 3Links: 4
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Record Fields

Scalar fields from the final disease record.

Disease Id
19157
Core Entity Id
77648
Source Entity Count
1
Preferred Name
Loeys-Dietz Syndrome Type 2
Name Cn
Loeys-Dietz综合征2型
Name Pinyin
Loeys-dietz Zong He Zheng 2 Xing
Name En
Loeys-Dietz Syndrome Type 2
Name Latin
Bilingual Status
complete
Disease Type
disease
Umls Disease Type
Disease or Syndrome
Disgenet Type
disease
Mesh Class
Cardiovascular Diseases; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Musculoskeletal Diseases
Do Class
Hpo Class
Mesh Class Name
Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Musculoskeletal Diseases; Cardiovascular Diseases
Hpo Class Name
Do Class Name
Disease Definition
NCI2016_02D:A rare autosomal dominant inherited disorder of connective tissue caused by mutations in either the TGFBR1 or TGFBR2 gene. Like Loeys-Dietz syndrome type I the disease is characterized by enlargement of the aorta and other arteries, and arterial tortuosity, but skeletal signs are typically less severe or absent in type 2. Skin abnormalities, such as velvety skin are often present in type 2.
Version
v2
Suppressed
No

Names

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

Name
Loeys-Dietz Syndrome Type 2
Role
preferred

Cross References

Trusted external identifiers retained for this final record.

Herb
HBDIS022928
Umls
C3898580
Sym Map
SMDE10510
Dis Ge Net
C3898580
Umls Sty
T047
Me Sh Class
C05C14C16
Tcmbank Disease
19055
Itcmdb Generated
ITX-DISEASE-6AA7F8048529

Attributes

Merged source attributes and domain-specific metadata.

Version
v2
Suppress
0
Disease Type
disease
Umls Disease Type
Disease or Syndrome
Disease Definition
NCI2016_02D:A rare autosomal dominant inherited disorder of connective tissue caused by mutations in either the TGFBR1 or TGFBR2 gene. Like Loeys-Dietz syndrome type I the disease is characterized by enlargement of the aorta and other arteries, and arterial tortuosity, but skeletal signs are typically less severe or absent in type 2. Skin abnormalities, such as velvety skin are often present in type 2.
Me Sh Disease Class
Cardiovascular Diseases; 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; Cardiovascular Diseases
Umls Semantic Type Name
Disease or Syndrome