MCID: PLM006
MIFTS: 52

Pulmonary Alveolar Proteinosis malady

Respiratory diseases, Rare diseases categories
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Summaries for Pulmonary Alveolar Proteinosis

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Sources:
8Disease Ontology, 65Wikipedia, 33MalaCards
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Disease Ontology:8 A lung disease that is characterized by abnormal accumulation of surfactant occurs within the alveoli, interfering with gas exchange.

MalaCards: Pulmonary Alveolar Proteinosis is related to pneumonia and myelodysplastic syndromes. An important gene associated with Pulmonary Alveolar Proteinosis is SFTPB (surfactant protein B), and among its related pathways are Regulation of retinoblastoma protein and Signaling by Interleukins. The compounds infasurf and 1,2-dipalmitoyl-sn-glycero-3-phosphocholine have been mentioned in the context of this disorder. Affiliated tissues include lung, myeloid and bone, and related mouse phenotypes are homeostasis/metabolism and immune system.

Wikipedia:65 Pulmonary alveolar proteinosis (abbreviated PAP), is a rare lung disease in which abnormal accumulation... more...

Aliases & Classifications for Pulmonary Alveolar Proteinosis

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8Disease Ontology, 22GTR, 10DISEASES, 45Novoseek, 62UMLS, 35MeSH, 27ICD9CM, 58SNOMED-CT, 40NCIt
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Respiratory diseases


Aliases & Descriptions:

pulmonary alveolar proteinosis 8 22 10 45 62


External Ids:

Disease Ontology8 DOID:12120
MeSH35 D011649
SNOMED-CT58 10501004
ICD9CM27 516.0
NCIt40 C85037

Related Diseases for Pulmonary Alveolar Proteinosis

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17GeneCards, 18GeneDecks
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Diseases in the Pulmonary Alveolar Proteinosis family:

Congenital Pulmonary Alveolar Proteinosis Pulmonary Alveolar Proteinosis Acquired

Diseases related to Pulmonary Alveolar Proteinosis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 109)
idRelated DiseaseScoreTop Affiliating Genes
1pneumonia31.1SFTPB, SFTPD, CSF2, SFTPC
2myelodysplastic syndromes31.0IL3, CSF2, CSF1
3pulmonary fibrosis30.8SFTPC, SFTPD
4respiratory failure30.8SFTPD, SFTPB
5lung cancer30.7SFTPB, CSF2, SFTPD
6idiopathic pulmonary fibrosis30.6SFTPB, SFTPD, SFTPC
7myeloid leukemia30.5CSF1, CSF2, IL3
8aspergillosis30.4SFTPD, CSF1
9eosinophilia30.3CSF2, CSF1, IL3
10myeloma30.3IL3, CSF1, CSF2
11idiopathic pulmonary alveolar proteinosis10.9
12leukemia10.8
13congenital pulmonary alveolar proteinosis10.8
14tuberculosis10.6
15lysinuric protein intolerance10.5
16pulmonary alveolar proteinosis acquired10.5
17desquamative interstitial pneumonia10.4
18fanconi's anemia10.4
19cryptococcosis10.4
20amyloidosis10.4
21arthritis10.4
22lung squamous cell carcinoma10.4
23mediastinitis10.4
24pneumothorax10.4
25psoriasis10.4
26surfactant metabolism dysfunction, pulmonary, 110.4
27surfactant metabolism dysfunction, pulmonary, 510.4
28lymphoid leukemia10.2
29congenital dyserythropoietic anemia10.2
30pulmonary tuberculosis10.2
31bronchiectasis10.2
32nocardiosis10.2
33acquired immunodeficiency syndrome10.2
34silicosis10.2
35chronic myeloid leukemia10.2
36septic arthritis10.2
37lipid pneumonia10.2
38lymphangioleiomyomatosis10.2
39cerebritis10.2
40acute myeloid leukemia10.2
41adenosine deaminase deficiency10.2
42systemic lupus erythematosus10.2
43common variable immunodeficiency10.2
44pulmonary alveolar microlithiasis10.2
45wegener's granulomatosis10.2
46hermansky-pudlak syndrome10.2
47behcet's disease10.2
48agammaglobulinemia10.2
49glomerulonephritis10.2
50histoplasmosis10.2

Graphical network of the top 20 diseases related to Pulmonary Alveolar Proteinosis:



Diseases related to pulmonary alveolar proteinosis

Symptoms for Pulmonary Alveolar Proteinosis

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Drugs & Therapeutics for Pulmonary Alveolar Proteinosis

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Sources:
42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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Drug clinical trials:

Search ClinicalTrials for Pulmonary Alveolar Proteinosis

Search NIH Clinical Center for Pulmonary Alveolar Proteinosis

Genetic Tests for Pulmonary Alveolar Proteinosis

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22GTR
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Genetic tests related to Pulmonary Alveolar Proteinosis:

id Genetic test Affiliating Genes
1 Pulmonary Alveolar Proteinosis22

Anatomical Context for Pulmonary Alveolar Proteinosis

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33MalaCards
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MalaCards organs/tissues related to Pulmonary Alveolar Proteinosis:

33
Lung, Myeloid, Bone, Bone marrow, Monocytes, Brain, Testes, Heart, T cells

Animal Models for Pulmonary Alveolar Proteinosis or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Pulmonary Alveolar Proteinosis:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053767.6CSF1, CSF2, IL3, SFTPD, SFTPB
2MP:00053877.2SFTPD, SFTPC, IL3, CSF2, CSF1
3MP:00053887.1CSF1, CSF2, IL3, SFTPC, SFTPD, SFTPB
4MP:00107687.0SFTPB, SFTPD, SFTPC, IL3, CSF2, CSF1

Publications for Pulmonary Alveolar Proteinosis

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52PubMed
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Articles related to Pulmonary Alveolar Proteinosis:

(show top 50)    (show all 497)
idTitleAuthorsYear
1
Development of pulmonary alveolar proteinosis following exposure to dust after the Great East Japan Earthquake. (24238228)
2013
2
De novo complex X chromosome rearrangement unmasking maternally inherited CSF2RA deletion in a girl with pulmonary alveolar proteinosis. (23918747)
2013
3
Pulmonary alveolar proteinosis requiring "hybrid" extracorporeal life support, and complicated by acute necrotizing pneumonia. (23523160)
2013
4
Standardized serum GM-CSF autoantibody testing for the routine clinical diagnosis of autoimmune pulmonary alveolar proteinosis. (24275678)
2013
5
Extracorporeal membrane oxygenation to support whole-lung lavage in pulmonary alveolar proteinosis: salvage of the drowned lungs. (23328142)
2013
6
Pulmonary alveolar proteinosis: quantitative CT and pulmonary functional correlations. (21621361)
2012
7
Spontaneous pulmonary alveolar proteinosis in captive "moustached tamarins" (Saguinus mystax). (21734058)
2012
8
Pulmonary alveolar proteinosis in an indium-processing worker. (20529594)
2010
9
Pulmonary alveolar proteinosis in a 67-year-old woman with Wegener's granulomatosis. (20623200)
2010
10
Patient-derived granulocyte/macrophage colony-stimulating factor autoantibodies reproduce pulmonary alveolar proteinosis in nonhuman primates. (20224064)
2010
11
Whole-lung lavage in infants and children with pulmonary alveolar proteinosis. (21199121)
2010
12
F-18 FDG PET/CT in pulmonary alveolar proteinosis. (19352264)
2009
13
Differences in the immunolocalization of surfactant protein (SP)-A, SP-D, and KL-6 in pulmonary alveolar proteinosis. (18251786)
2008
14
Secondary pulmonary alveolar proteinosis in a patient with chronic myeloid leukemia in the accelerated phase. (21318986)
2008
15
Clinical importance of bronchoalveolar lavage fluid and blood cytokines, surfactant protein D, and Kerbs von Lungren 6 antigen in idiopathic pulmonary alveolar proteinosis. (19046553)
2008
16
GM-CSF autoantibodies in pulmonary alveolar proteinosis. (17506160)
2007
17
Pulmonary alveolar proteinosis--is host defense awry? (17287475)
2007
18
Pulmonary alveolar proteinosis. (16916009)
2006
19
Pulmonary alveolar proteinosis with myeloproliferative syndrome with myelodysplasia: bronchoalveolar lavage reduces white blood cell count. (16906593)
2006
20
Serum antibody against granulocyte/macrophage colony-stimulating factor and KL-6 in idiopathic pulmonary alveolar proteinosis. (16565598)
2006
21
Aerosol granulocyte-macrophage colony-stimulating factor for pulmonary alveolar proteinosis. (16507860)
2006
22
Pulmonary alveolar proteinosis. What is the role of GM-CSF in disease pathogenesis and treatment? (15350161)
2004
23
Secondary pulmonary alveolar proteinosis associated with myelodysplastic syndrome. (12636240)
2003
24
Disseminated Mycobacterium kansasii infection with pulmonary alveolar proteinosis in a patient with chronic myelogenous leukemia. (14587059)
2003
25
Analysis of the GM-CSF and GM-CSF/IL-3/IL-5 receptor common beta chain in a patient with pulmonary alveolar proteinosis. (11930665)
2002
26
Detection of granulocyte-macrophage colony-stimulating factor in patients with pulmonary alveolar proteinosis. (10764326)
2000
27
Pulmonary alveolar proteinosis in a child: HRCT findings before and after bronchoalveolar lavage. (9866787)
1998
28
Serum KL-6 level as a monitoring marker in a patient with pulmonary alveolar proteinosis. (10319067)
1998
29
Pulmonary alveolar proteinosis with respiratory failure--therapeutic role of bronchoscopic and whole lung lavage. (11229289)
1998
30
Immunoglobulin G is associated with surfactant protein A aggregate isolated from patients with pulmonary alveolar proteinosis. (9154892)
1997
31
Pulmonary alveolar proteinosis. (9157392)
1997
32
Bronchoalveolar lavage cytology in pulmonary alveolar proteinosis. (8853040)
1996
33
Interstitial lattice shadow and mediastinal lymphadenopathy with an elevation of carcinoembryonic antigen in severe pulmonary alveolar proteinosis. (1611200)
1992
34
Pulmonary alveolar proteinosis (with review of literature). (3060464)
1988
35
Pulmonary alveolar proteinosis complicated by cerebral abscess: report of a case. (3528083)
1986
36
Inorganic particulates associated with pulmonary alveolar proteinosis: SEM and X-ray microanalysis results. (3036185)
1986
37
Pulmonary alveolar proteinosis in the newborn. (3838881)
1985
38
Pulmonary alveolar proteinosis: prospective clinical experience in 23 patients for 15 years. (6492867)
1984
39
Progressive intrapartum respiratory insufficiency due to pulmonary alveolar proteinosis. Amelioration by therapeutic whole-lung bronchopulmonary lavage. (6468015)
1984
40
The immunoinhibitory activities of the lung lavage materials and sera from patients with pulmonary alveolar proteinosis (PAP). (6894313)
1981
41
Particulate concentrations in pulmonary alveolar proteinosis. (318522)
1978
42
Pulmonary Alveolar proteinosis. Lobar localization and surgical excision. (1262145)
1976
43
Pulmonary alveolar proteinosis--physiologic observations during and after pulmonary lavage. (4531921)
1973
44
Pulmonary alveolar proteinosis. Home treatment with intermittent positive pressure breathing. (4573995)
1973
45
Pulmonary alveolar proteinosis. (5058156)
1972
46
Focal pulmonary alveolar proteinosis in pulmonary tuberculosis. (5766260)
1969
47
Pulmonary alveolar proteinosis. (6071228)
1967
48
Pulmonary alveolar proteinosis. Treatment in a case complicated by tuberculosis. (6066626)
1967
49
Pulmonary alveolar proteinosis presenting with pneumothorax. (6073913)
1967
50
Studies of pulmonary alveolar microlithiasis and pulmonary alveolar proteinosis. (13775542)
1960

Variations for Pulmonary Alveolar Proteinosis

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Expression for genes affiliated with Pulmonary Alveolar Proteinosis

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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Pulmonary Alveolar Proteinosis

Search GEO for disease gene expression data for Pulmonary Alveolar Proteinosis.

Pathways for genes affiliated with Pulmonary Alveolar Proteinosis

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Sources:
50PathCards, 38NCBI BioSystems Database, 55Reactome, 30KEGG, 53QIAGEN
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Compounds for genes affiliated with Pulmonary Alveolar Proteinosis

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45Novoseek, 11DrugBank, 51PharmGKB, 29IUPHAR, 61Tocris Bioscience, 24HMDB, 3BitterDB
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Compounds related to Pulmonary Alveolar Proteinosis according to GeneCards/GeneDecks:

(show top 50)    (show all 122)
idCompoundScoreTop Affiliating Genes
1infasurf4510.0SFTPB, SFTPC
21,2-dipalmitoyl-sn-glycero-3-phosphocholine4510.0SFTPC, SFTPB
3dppg4510.0SFTPB, SFTPC
4popg4510.0SFTPC, SFTPB
5curosurf459.9SFTPB, SFTPC
6survanta459.9SFTPC, SFTPB
7dopc459.9SFTPB, SFTPC
8bmmp459.7IL3, CSF2
9dppc459.7SFTPB, SFTPC
10pegfilgrastim45 1110.5CSF2, IL3
11mesna459.4CSF2, IL3
12doxycycline45 1110.4SFTPB, SFTPC, IL3
13a23187459.3CSF2, IL3
14bleomycin45 1110.3SFTPC, CSF2, SFTPB
15thiotepa45 51 1111.0CSF2, IL3
16molgramostim459.0CSF2, CSF1, IL3
17pixy321458.9CSF1, CSF2, IL3
18filgrastim45 119.9CSF2, CSF1, IL3
19bryostatin 1458.9CSF1, CSF2, IL3
20ltc4458.9CSF2, CSF1, IL3
21gm-csf45 299.9CSF1, CSF2, IL3
22budesonide45 51 1110.9CSF1, CSF2, SFTPD
23ifosfamide45 119.9CSF1, IL3, CSF2
24mitoxantrone45 51 1110.9CSF2, CSF1, IL3
25methylcellulose458.9CSF1, CSF2, IL3
26il 10458.9CSF1, CSF2, IL3
27anti-fungal458.9CSF1, CSF2, SFTPD
28n-formylmethionyl-leucylphenylalanine458.9CSF1, IL3, CSF2
29ivig458.9CSF1, CSF2, IL3
30neopterin458.8CSF1, CSF2, IL3
31daunorubicin45 51 1110.8IL3, CSF2, CSF1
32zidovudine45 51 1110.8IL3, CSF1, CSF2
33carboplatin45 51 1110.8CSF2, CSF1, IL3
34hydroxyurea45 51 1110.7CSF2, IL3, CSF1
35gp 130458.7IL3, CSF2, CSF1
36leukotriene b445 61 2410.7IL3, CSF2, CSF1
37chloramphenicol45 3 51 1111.7CSF1, IL3, SFTPC, SFTPB
38inositol458.5CSF1, SFTPD, CSF2
3912-o-tetradecanoylphorbol 13-acetate458.5CSF2, IL3, SFTPB, CSF1
40valine458.5IL3, CSF2, CSF1, SFTPC
41cytarabine45 51 1110.4CSF2, IL3, CSF1
42histamine45 29 2410.4IL3, CSF2, CSF1, SFTPD
43lactate458.3SFTPD, IL3, CSF2, CSF1
44phosphatidylcholine458.2SFTPC, SFTPB, SFTPD, CSF1, IL3
45actinomycin d458.1CSF1, CSF2, IL3, SFTPC, SFTPB
46cycloheximide458.1SFTPC, IL3, CSF2, CSF1, SFTPB
47retinoic acid45 249.0CSF1, CSF2, IL3, SFTPC, SFTPB
48thymidine45 249.0SFTPB, CSF1, CSF2, IL3, SFTPD
49dexamethasone45 51 29 1110.6CSF2, CSF1, SFTPD, SFTPB, SFTPC, IL3
50cysteine457.6SFTPB, SFTPD, SFTPC, IL3, CSF2, CSF1

GO Terms for genes affiliated with Pulmonary Alveolar Proteinosis

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16Gene Ontology
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Cellular components related to Pulmonary Alveolar Proteinosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:0056157.1CSF1, CSF2, IL3, SFTPC, SFTPD, SFTPB

Biological processes related to Pulmonary Alveolar Proteinosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1positive regulation of tyrosine phosphorylation of Stat5 proteinGO:0425239.2CSF2, IL3
2respiratory gaseous exchangeGO:0075859.2SFTPC, SFTPD, SFTPB
3positive regulation of macrophage derived foam cell differentiationGO:0107449.0CSF2, CSF1
4positive regulation of DNA replicationGO:0457409.0CSF2, IL3
5positive regulation of cell proliferationGO:0082848.5IL3, CSF2, CSF1

Molecular functions related to Pulmonary Alveolar Proteinosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cytokine activityGO:0051258.5CSF1, CSF2, IL3
2growth factor activityGO:0080838.2CSF1, CSF2, IL3

Products for genes affiliated with Pulmonary Alveolar Proteinosis

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Sources for Pulmonary Alveolar Proteinosis

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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet