DiseaseID 5447

青少年型脊髓性肌萎缩症

disease

NCI2016_02D:A group of congenital neurodegenerative disorders affecting motor neurons, resulting in muscle weakness and atrophy in childhood. Signs and symptoms include hypotonia, difficulty breathing, poor feeding, and

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Disease: 1Experiment: 1Symptom: 3Target: 12Links: 16
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Record Fields

Scalar fields from the final disease record.

Disease Id
5447
Core Entity Id
61947
Source Entity Count
1
Preferred Name
Juvenile Spinal Muscular Atrophy
Name Cn
青少年型脊髓性肌萎缩症
Name Pinyin
Qing Shao Nian Xing Ji Sui Xing Ji Wei Suo Zheng
Name En
Juvenile Spinal Muscular Atrophy
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; Nervous System Diseases
Do Class
disease of anatomical entitydisease of anatomical entity; genetic disease
Hpo Class
Mesh Class Name
Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nervous System Diseases
Hpo Class Name
Do Class Name
disease of anatomical entitygenetic disease; disease of anatomical entity
Disease Definition
NCI2016_02D:A group of congenital neurodegenerative disorders affecting motor neurons, resulting in muscle weakness and atrophy in childhood. Signs and symptoms include hypotonia, difficulty breathing, poor feeding, and flaccid quadriplegia.|MSH2017_2016_08_12:A group of recessive inherited diseases that feature progressive muscular atrophy and hypotonia. They are classified as type I (Werdnig-Hoffman disease), type II (intermediate form), and type III (Kugelberg-Welander disease). Type I is fatal in infancy, type II has a late infantile onset and is associated with survival into the second or third decade. Type III has its onset in childhood, and is slowly progressive. (J Med Genet 1996 Apr:33(4):281-3)
Version
v1
Suppressed
No

Names

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

Name
Juvenile Spinal Muscular Atrophy
Role
preferred
Name
Muscular Atrophy, Spinal, Infantile Chronic Form
Role
preferred
Name
Muscular Atrophy, Spinal, Type Ii
Role
preferred
Name
Spinal Muscular Atrophies Of Childhood
Role
preferred
Name
Spinal Muscular Atrophy, Type Ii
Role
preferred
Name
HMN (Hereditary Motor Neuropathy) Proximal Type I
Role
preferred
Name
Infantile Spinal Muscular Atrophy, Type I [Werdnig-Hoffman]
Role
alias
Name
Intermediate Spinal Muscular Atrophy
Role
alias
Name
MUSCULAR ATROPHY, SPINAL, INTERMEDIATE TYPE
Role
alias
Name
SMA II
Role
alias
Name
SMA2
Role
alias
Name
Spinal Muscular Atrophy, Type I
Role
alias
Name
Spinal Muscular Atrophy, Type Iii
Role
alias
Name
Survival Motor Neuron Spinal Muscular Atrophy
Role
alias
Name
Werdnig-Hoffmann Disease
Role
alias

Cross References

Trusted external identifiers retained for this final record.

Herb
HBDIS003144HBDIS003674HBDIS008658HBDIS010695HBDIS020284
Me Sh
D014897
Omim
253300253400253550
Umls
C0393538C0700595C2931358
Icd10
G12.0
Sym Map
SMDE00766SMDE11168SMDE11169SMDE13345
Do Class
DOID:630DOID:7
Dis Ge Net
C0043116C0152109C0393538C0700595C2931358
Umls Sty
T047
Me Sh Class
C10C16
Tcmbank Disease
141242366224948250967355
Itcmdb Generated
ITX-DISEASE-D96BA3563878ITX-DISEASE-FC9DEF93FA8E

Attributes

Merged source attributes and domain-specific metadata.

Version
v1v2
Suppress
0
Do Class Name
disease of anatomical entitygenetic disease; disease of anatomical entity
Disease Type
disease
Do Disease Class
disease of anatomical entitydisease of anatomical entity; genetic disease
Umls Disease Type
Disease or Syndrome
Disease Definition
NCI2016_02D:A group of congenital neurodegenerative disorders affecting motor neurons, resulting in muscle weakness and atrophy in childhood. Signs and symptoms include hypotonia, difficulty breathing, poor feeding, and flaccid quadriplegia.|MSH2017_2016_08_12:A group of recessive inherited diseases that feature progressive muscular atrophy and hypotonia. They are classified as type I (Werdnig-Hoffman disease), type II (intermediate form), and type III (Kugelberg-Welander disease). Type I is fatal in infancy, type II has a late infantile onset and is associated with survival into the second or third decade. Type III has its onset in childhood, and is slowly progressive. (J Med Genet 1996 Apr:33(4):281-3)
Me Sh Disease Class
Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nervous System Diseases
Dis Ge Net Disease Type
disease
Disease Class Name Me Sh
Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nervous System Diseases
Umls Semantic Type Name
Disease or Syndrome