DiseaseID 13913

常染色体隐性遗传包涵体肌病

disease

Intellectual disability-developmental delay-contractures syndrome, formerly known as Wieacker-Wolff syndrome, is a severe X-linked recessive neurodevelopmental disorder characterized by severe contrac

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

Scalar fields from the final disease record.

Disease Id
13913
Core Entity Id
71623
Source Entity Count
1
Preferred Name
Inclusion Body Myopathy, Autosomal Recessive
Name Cn
常染色体隐性遗传包涵体肌病
Name Pinyin
Chang Ran Se Ti Yin Xing Yi Chuan Bao Han Ti Ji Bing
Name En
Inclusion Body Myopathy, Autosomal Recessive
Name Latin
Bilingual Status
complete
Disease Type
disease
Umls Disease Type
Disease or Syndrome
Disgenet Type
disease
Mesh Class
Nervous System Diseases; Musculoskeletal Diseases
Do Class
Hpo Class
Mesh Class Name
Musculoskeletal Diseases; Nervous System Diseases
Hpo Class Name
Do Class Name
Disease Definition
Intellectual disability-developmental delay-contractures syndrome, formerly known as Wieacker-Wolff syndrome, is a severe X-linked recessive neurodevelopmental disorder characterized by severe contrac
Version
v1
Suppressed
No

Names

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

Name
Inclusion Body Myopathy, Autosomal Recessive
Role
preferred
Name
Gne Myopathy
Role
preferred
Name
Intellectual Disability-Developmental Delay-Contractures Syndrome
Role
preferred
Name
Myopathy Due To Calsequestrin And Serca1 Protein Overload
Role
preferred

Cross References

Trusted external identifiers retained for this final record.

Herb
HBDIS015173
Umls
C0796200C1833373C1853926
Icd10
G71.8
Sym Map
SMDE00563SMDE02730SMDE04454SMDE09812
Dis Ge Net
C1833373
Orphanet
345460288635
Umls Sty
T047
Me Sh Class
C05C10
Tcmbank Disease
4024

Attributes

Merged source attributes and domain-specific metadata.

Version
v1v1,v2v2
Suppress
0
Disease Type
disease
Umls Disease Type
Disease or Syndrome
Disease Definition
Intellectual disability-developmental delay-contractures syndrome, formerly known as Wieacker-Wolff syndrome, is a severe X-linked recessive neurodevelopmental disorder characterized by severe contracMyopathy due to calsequestrin and SERCA1 protein overload is characterised by mild myopathy or elevated levels of creatine kinase in the blood without associated symptoms.Nonaka distal myopathy (described in Japan) and the quadriceps-sparing autosomal recessive inclusion body myopathy type 2 (IBM2; independently described in Iranian Jews and later in other Jewish and n
Me Sh Disease Class
Nervous System Diseases; Musculoskeletal Diseases
Dis Ge Net Disease Type
disease
Disease Class Name Me Sh
Musculoskeletal Diseases; Nervous System Diseases
Umls Semantic Type Name
Disease or Syndrome