DiseaseID 26485

补体成分C1r/C1s缺陷

Complement Component C1R/C1S Deficiency

NCI2016_NICHD_1602D:Lack of production of either functional C1r or C1s protein, due to a genetic defect. Approximately 60% of patients with a C1r/C1s deficiency will develop a severe systemic lupus erythematosus at an ea

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Disease: 1Symptom: 1Target: 1Links: 2
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Record Fields

Scalar fields from the final disease record.

Disease Id
26485
Core Entity Id
119053
Source Entity Count
1
Preferred Name
Complement Component C1R/C1S Deficiency
Name Cn
补体成分C1r/C1s缺陷
Name Pinyin
Bu Ti Cheng Fen C1r/c1s Que Xian
Name En
Complement Component C1R/C1S Deficiency
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_NICHD_1602D:Lack of production of either functional C1r or C1s protein, due to a genetic defect. Approximately 60% of patients with a C1r/C1s deficiency will develop a severe systemic lupus erythematosus at an early age. Patients also present with frequent sinopulmonary infections often with Streptococcus pneumoniae.|NCI2016_02D:Lack of production of either functional C1r or C1s protein, due to a genetic defect. Approximately 60% of patients with a C1r/C1s deficiency will develop a severe systemic lupus erythematosus at an early age. Patients also present with frequent sinopulmonary infections often with Streptococcus pneumoniae.
Version
v1,v2
Suppressed
No

Names

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

Name
Complement Component C1R/C1S Deficiency
Role
preferred
Name
C1r/C1s DEFICIENCY
Role
alias

Cross References

Trusted external identifiers retained for this final record.

Omim
216950
Umls
C3150274
Sym Map
SMDE03707
Itcmdb Generated
ITX-DISEASE-C483A907990E

Attributes

Merged source attributes and domain-specific metadata.

Version
v1,v2
Suppress
0
Disease Definition
NCI2016_NICHD_1602D:Lack of production of either functional C1r or C1s protein, due to a genetic defect. Approximately 60% of patients with a C1r/C1s deficiency will develop a severe systemic lupus erythematosus at an early age. Patients also present with frequent sinopulmonary infections often with Streptococcus pneumoniae.|NCI2016_02D:Lack of production of either functional C1r or C1s protein, due to a genetic defect. Approximately 60% of patients with a C1r/C1s deficiency will develop a severe systemic lupus erythematosus at an early age. Patients also present with frequent sinopulmonary infections often with Streptococcus pneumoniae.