DiseaseID 5015
肺泡蛋白沉积症
disease
NCI2016_02D:A rare lung disorder characterized by the filling of the pulmonary alveoli with proteinaceous material which stains positive with periodic acid-Schiff stain. It may be idiopathic or secondary due to hematolog
Relationship Network
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Disease: 1Symptom: 3Target: 12Links: 15
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Record Fields
Scalar fields from the final disease record.
- Disease Id
- 5015
- Core Entity Id
- 61452
- Source Entity Count
- 1
- Preferred Name
- Pulmonary Alveolar Proteinosis
- Name Cn
- 肺泡蛋白沉积症
- Name Pinyin
- Fei Pao Dan Bai Chen Ji Zheng
- Name En
- Pulmonary Alveolar Proteinosis
- 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; Respiratory Tract DiseasesRespiratory Tract Diseases
- Do Class
- disease of anatomical entity
- Hpo Class
- Abnormality of the respiratory system
- Mesh Class Name
- Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Respiratory Tract DiseasesRespiratory Tract Diseases
- Hpo Class Name
- Abnormality of the respiratory system
- Do Class Name
- disease of anatomical entity
- Disease Definition
- NCI2016_02D:A rare lung disorder characterized by the filling of the pulmonary alveoli with proteinaceous material which stains positive with periodic acid-Schiff stain. It may be idiopathic or secondary due to hematologic malignancies or the inhalation of mineral dusts. Signs and symptoms include dyspnea, cough and low grade fever.|MSH2017_2016_08_12:A PULMONARY ALVEOLI-filling disease, characterized by dense phospholipoproteinaceous deposits in the alveoli, cough, and DYSPNEA. This disease is often related to, congenital or acquired, impaired processing of PULMONARY SURFACTANTS by alveolar macrophages, a process dependent on GRANULOCYTE-MACROPHAGE COLONY-STIMULATING FACTOR.|HPO2016_07_04:Abnormal accumulation of surfactant-like, periodic acid-schiff-positive lipoproteinaceous material in macrophages within the alveolar spaces and distal bronchioles. This results in gas exchange impairment leading to dyspnea and alveolar infiltrates. [HPO:probinson, pmid:22891182]|CSP2006:unknown etiology marked by chronic filling of alveoli with proteinaceous, lipid-rich, granular material consisting of surfactant and debris of necrotic cells.
- Version
- v1,v2
- Suppressed
- No
Names
Preferred names, aliases, and source labels retained in the final schema.
Name
Pulmonary Alveolar Proteinosis
Role
preferred
Name
Surfactant Metabolism Dysfunction, Pulmonary, 1
Role
preferred
Name
Surfactant Metabolism Dysfunction, Pulmonary, 3
Role
preferred
Name
Surfactant Metabolism Dysfunction, Pulmonary, 4
Role
preferred
Name
Surfactant Metabolism Dysfunction, Pulmonary, 5
Role
preferred
Name
Pulmonary Alveolar Proteinosis, Congenital
Role
preferred
Name
SURFACTANT METABOLISM DYSFUNCTION, PULMONARY, 2 (disorder)
Role
preferred
Name
Alveolar Proteinosis
Role
alias
Name
CSF2RB DEFICIENCY
Role
alias
Name
INTERSTITIAL LUNG DISEASE DUE TO ABCA3 DEFICIENCY
Role
alias
Name
INTERSTITIAL LUNG DISEASE DUE TO SURFACTANT PROTEIN B DEFICIENCY
Role
alias
Name
INTERSTITIAL LUNG DISEASE, NONSPECIFIC, DUE TO SURFACTANT PROTEIN B DEFICIENCY
Role
alias
Name
PAP DUE TO CSF2RB DEFICIENCY
Role
alias
Name
PULMONARY ALVEOLAR PROTEINOSIS 5
Role
alias
Name
PULMONARY ALVEOLAR PROTEINOSIS, CONGENITAL, 1
Role
alias
Name
PULMONARY ALVEOLAR PROTEINOSIS, CONGENITAL, 3
Role
alias
Name
SMDP1
Role
alias
Name
SMDP3
Role
alias
Name
SMDP5
Role
alias
Name
Smdp2
Role
alias
Name
Surfactant Metabolism Dysfunction, Pulmonary, 2
Role
alias
Cross References
Trusted external identifiers retained for this final record.
Hpo
HP:0006517
Herb
HBDIS002534HBDIS018677HBDIS018842HBDIS018845HBDIS019527HBDIS020203HBDIS021318
Me Sh
D011649
Omim
265120300770610913610921614370
Umls
C0034050C1968602C1970456C2677877C3280574
Icd10
J84.01
Sym Map
SMDE00093SMDE01015SMDE04882SMDE12580SMDE13747
Do Class
DOID:7
Dis Ge Net
C0034050C1968602C1970456C1970470C2677877C2931035C3280574
Umls Sty
T047
Hpo Class
HP:0002086
Me Sh Class
C08C16
Tcmbank Disease
14213144851482716147239702444931411
Attributes
Merged source attributes and domain-specific metadata.
Version
v1,v2v2
Suppress
0
Do Class Name
disease of anatomical entity
Disease Type
disease
Hpo Class Name
Abnormality of the respiratory system
Do Disease Class
disease of anatomical entity
Hpo Disease Class
Abnormality of the respiratory system
Umls Disease Type
Disease or Syndrome
Disease Definition
NCI2016_02D:A rare lung disorder characterized by the filling of the pulmonary alveoli with proteinaceous material which stains positive with periodic acid-Schiff stain. It may be idiopathic or secondary due to hematologic malignancies or the inhalation of mineral dusts. Signs and symptoms include dyspnea, cough and low grade fever.|MSH2017_2016_08_12:A PULMONARY ALVEOLI-filling disease, characterized by dense phospholipoproteinaceous deposits in the alveoli, cough, and DYSPNEA. This disease is often related to, congenital or acquired, impaired processing of PULMONARY SURFACTANTS by alveolar macrophages, a process dependent on GRANULOCYTE-MACROPHAGE COLONY-STIMULATING FACTOR.|HPO2016_07_04:Abnormal accumulation of surfactant-like, periodic acid-schiff-positive lipoproteinaceous material in macrophages within the alveolar spaces and distal bronchioles. This results in gas exchange impairment leading to dyspnea and alveolar infiltrates. [HPO:probinson, pmid:22891182]|CSP2006:unknown etiology marked by chronic filling of alveoli with proteinaceous, lipid-rich, granular material consisting of surfactant and debris of necrotic cells.
Me Sh Disease Class
Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Respiratory Tract DiseasesRespiratory Tract Diseases
Dis Ge Net Disease Type
disease
Disease Class Name Me Sh
Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Respiratory Tract DiseasesRespiratory Tract Diseases
Umls Semantic Type Name
Disease or Syndrome