MCID: PLM006
MIFTS: 47

Pulmonary Alveolar Proteinosis malady

Rare diseases, Respiratory diseases, Cardiovascular diseases categories

Summaries for Pulmonary Alveolar Proteinosis

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Disease Ontology:9 A lung disease that is characterized by abnormal accumulation of surfactant occurs within the alveoli, interfering with gas exchange.

MalaCards based summary: Pulmonary Alveolar Proteinosis is related to respiratory failure and pulmonary fibrosis. An important gene associated with Pulmonary Alveolar Proteinosis is SFTPB (surfactant protein B), and among its related pathways are Regulation of retinoblastoma protein and Calcineurin-regulated NFAT-dependent transcription in lymphocytes. 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 marrow, and related mouse phenotypes are homeostasis/metabolism and immune system.

Wikipedia:63 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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Sources:
9Disease Ontology, 11DISEASES, 43Novoseek, 22GTR, 60UMLS, 38NCIt, 27ICD9CM, 33MeSH, 55SNOMED-CT
See all sources

Pulmonary Alveolar Proteinosis, Aliases & Descriptions:

Name: Pulmonary Alveolar Proteinosis 9 11 43 22 60


Classifications:

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


External Ids:

Disease Ontology9 DOID:12120
NCIt38 C85037
SNOMED-CT55 10501004
ICD9CM27 516.0
MeSH33 D011649

Related Diseases for Pulmonary Alveolar Proteinosis

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Diseases in the Pulmonary Alveolar Proteinosis family:

Congenital Pulmonary Alveolar Proteinosis Pulmonary Alveolar Proteinosis Acquired
Secondary Pulmonary Alveolar Proteinosis Severe Congenital Pulmonary Alveolar Proteinosis

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

(show top 50)    (show all 104)
idRelated DiseaseScoreTop Affiliating Genes
1respiratory failure31.0SFTPD, SFTPB
2pulmonary fibrosis31.0SFTPC, SFTPD
3pneumonia30.9SFTPB, SFTPD, CSF2, SFTPC
4myelodysplastic syndrome30.8IL3, CSF2, CSF1
5lung cancer30.6SFTPB, CSF2, SFTPD
6aspergillosis30.4SFTPD, CSF1
7myeloid leukemia30.4CSF1, CSF2, IL3
8eosinophilia30.1CSF2, CSF1, IL3
9myeloma30.0IL3, CSF1, CSF2
10congenital pulmonary alveolar proteinosis10.9
11secondary pulmonary alveolar proteinosis10.9
12leukemia10.8
13pulmonary alveolar proteinosis acquired10.8
14tuberculosis10.6
15lysinuric protein intolerance10.6
16severe congenital pulmonary alveolar proteinosis10.5
17meconium aspiration syndrome10.4SFTPB
18arthritis10.4
19desquamative interstitial pneumonia10.4
20cryptococcosis10.4
21mediastinitis10.4
22pneumothorax10.4
23psoriasis10.4
24systemic lupus erythematosus10.3
25surfactant metabolism dysfunction, pulmonary, 110.3
26breast cancer10.3
27immunodeficiency 2110.3
28pulmonary alveolar microlithiasis10.3
29pulmonary function10.3
30surfactant metabolism dysfunction, pulmonary, 510.3
31surfactant metabolism dysfunction, pulmonary, 410.3
32multiple myeloma10.3
33behcet's disease10.3
34common variable immunodeficiency10.3
35glomerulonephritis10.3
36lymphoid leukemia10.3
37silicosis10.3
38congenital dyserythropoietic anemia10.3
39pulmonary tuberculosis10.3
40bronchiectasis10.3
41nocardiosis10.3
42acquired immunodeficiency syndrome10.3
43septic arthritis10.3
44lipid pneumonia10.3
45cerebritis10.3
46adenosine deaminase deficiency10.3
47hermansky-pudlak syndrome10.3
48agammaglobulinemia10.3
49amyloidosis10.3
50histoplasmosis10.3

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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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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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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MalaCards organs/tissues related to Pulmonary Alveolar Proteinosis:

31
Lung, Myeloid, Bone marrow, Bone, Testes, Monocytes, Brain, Heart, Breast, T cells, Neutrophil

Animal Models for Pulmonary Alveolar Proteinosis or affiliated genes

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MGI Mouse Phenotypes related to Pulmonary Alveolar Proteinosis:

35
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053767.6CSF1, SFTPB, SFTPD, IL3, CSF2
2MP:00053877.2SFTPC, IL3, CSF2, CSF1, SFTPD
3MP:00053887.1CSF1, SFTPB, SFTPD, SFTPC, IL3, CSF2
4MP:00107687.0CSF1, SFTPC, SFTPB, SFTPD, CSF2, IL3

Publications for Pulmonary Alveolar Proteinosis

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Articles related to Pulmonary Alveolar Proteinosis:

(show top 50)    (show all 531)
idTitleAuthorsYear
1
Secondary pulmonary alveolar proteinosis in hematologic malignancies. (25300566)
2014
2
Localized pulmonary alveolar proteinosis: two case reports. (25337424)
2014
3
A crazy cause of dyspnoea: pulmonary alveolar proteinosis. (25152273)
2014
4
Development of pulmonary alveolar proteinosis following exposure to dust after the Great East Japan Earthquake. (24238228)
2013
5
De novo complex X chromosome rearrangement unmasking maternally inherited CSF2RA deletion in a girl with pulmonary alveolar proteinosis. (23918747)
2013
6
Pulmonary alveolar proteinosis requiring "hybrid" extracorporeal life support, and complicated by acute necrotizing pneumonia. (23523160)
2013
7
Standardized serum GM-CSF autoantibody testing for the routine clinical diagnosis of autoimmune pulmonary alveolar proteinosis. (24275678)
2013
8
Extracorporeal membrane oxygenation to support whole-lung lavage in pulmonary alveolar proteinosis: salvage of the drowned lungs. (23328142)
2013
9
Pulmonary alveolar proteinosis: quantitative CT and pulmonary functional correlations. (21621361)
2012
10
Spontaneous pulmonary alveolar proteinosis in captive "moustached tamarins" (Saguinus mystax). (21734058)
2012
11
Pulmonary alveolar proteinosis in an indium-processing worker. (20529594)
2010
12
Pulmonary alveolar proteinosis in a 67-year-old woman with Wegener's granulomatosis. (20623200)
2010
13
Patient-derived granulocyte/macrophage colony-stimulating factor autoantibodies reproduce pulmonary alveolar proteinosis in nonhuman primates. (20224064)
2010
14
Whole-lung lavage in infants and children with pulmonary alveolar proteinosis. (21199121)
2010
15
F-18 FDG PET/CT in pulmonary alveolar proteinosis. (19352264)
2009
16
Differences in the immunolocalization of surfactant protein (SP)-A, SP-D, and KL-6 in pulmonary alveolar proteinosis. (18251786)
2008
17
Secondary pulmonary alveolar proteinosis in a patient with chronic myeloid leukemia in the accelerated phase. (21318986)
2008
18
GM-CSF autoantibodies in pulmonary alveolar proteinosis. (17506160)
2007
19
Pulmonary alveolar proteinosis--is host defense awry? (17287475)
2007
20
Pulmonary alveolar proteinosis. (16916009)
2006
21
Pulmonary alveolar proteinosis with myeloproliferative syndrome with myelodysplasia: bronchoalveolar lavage reduces white blood cell count. (16906593)
2006
22
Serum antibody against granulocyte/macrophage colony-stimulating factor and KL-6 in idiopathic pulmonary alveolar proteinosis. (16565598)
2006
23
Pulmonary alveolar proteinosis. What is the role of GM-CSF in disease pathogenesis and treatment? (15350161)
2004
24
Secondary pulmonary alveolar proteinosis associated with myelodysplastic syndrome. (12636240)
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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Search GEO for disease gene expression data for Pulmonary Alveolar Proteinosis.

Pathways for genes affiliated with Pulmonary Alveolar Proteinosis

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Compounds for genes affiliated with Pulmonary Alveolar Proteinosis

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Compounds related to Pulmonary Alveolar Proteinosis according to GeneCards Suite gene sharing:

(show top 50)    (show all 122)
idCompoundScoreTop Affiliating Genes
1infasurf4310.0SFTPB, SFTPC
21,2-dipalmitoyl-sn-glycero-3-phosphocholine4310.0SFTPC, SFTPB
3dppg4310.0SFTPB, SFTPC
4popg4310.0SFTPC, SFTPB
5curosurf439.9SFTPB, SFTPC
6survanta439.9SFTPC, SFTPB
7dopc439.9SFTPB, SFTPC
8bmmp439.7IL3, CSF2
9dppc439.7SFTPB, SFTPC
10pegfilgrastim43 1210.5CSF2, IL3
11mesna439.4CSF2, IL3
12doxycycline43 1210.4SFTPB, SFTPC, IL3
13a23187439.3CSF2, IL3
14bleomycin43 1210.3SFTPC, CSF2, SFTPB
15thiotepa43 49 1211.0CSF2, IL3
16molgramostim439.0CSF2, CSF1, IL3
17pixy321438.9CSF1, CSF2, IL3
18filgrastim43 129.9CSF2, CSF1, IL3
19bryostatin 1438.9CSF1, CSF2, IL3
20ltc4438.9CSF2, CSF1, IL3
21gm-csf43 289.9CSF1, CSF2, IL3
22budesonide43 49 1210.9CSF1, CSF2, SFTPD
23ifosfamide43 129.9CSF1, IL3, CSF2
24mitoxantrone43 49 1210.9CSF2, CSF1, IL3
25methylcellulose438.9CSF1, CSF2, IL3
26il 10438.9CSF1, CSF2, IL3
27anti-fungal438.9CSF1, CSF2, SFTPD
28n-formylmethionyl-leucylphenylalanine438.9CSF1, IL3, CSF2
29ivig438.9CSF1, CSF2, IL3
30neopterin438.8CSF1, CSF2, IL3
31daunorubicin43 49 1210.8IL3, CSF2, CSF1
32zidovudine43 49 1210.8IL3, CSF1, CSF2
33carboplatin43 49 1210.8CSF2, CSF1, IL3
34hydroxyurea43 49 1210.7CSF2, IL3, CSF1
35gp 130438.7IL3, CSF2, CSF1
36leukotriene b443 59 2410.7IL3, CSF2, CSF1
37chloramphenicol43 2 49 1211.7CSF1, IL3, SFTPC, SFTPB
38inositol438.5CSF1, SFTPD, CSF2
3912-o-tetradecanoylphorbol 13-acetate438.5CSF2, IL3, SFTPB, CSF1
40valine438.5IL3, CSF2, CSF1, SFTPC
41cytarabine43 49 1210.4CSF2, IL3, CSF1
42histamine43 28 2410.4IL3, CSF2, CSF1, SFTPD
43lactate438.3SFTPD, IL3, CSF2, CSF1
44phosphatidylcholine438.2SFTPC, SFTPB, SFTPD, CSF1, IL3
45actinomycin d438.1CSF1, CSF2, IL3, SFTPC, SFTPB
46cycloheximide438.1SFTPC, IL3, CSF2, CSF1, SFTPB
47retinoic acid43 249.0CSF1, CSF2, IL3, SFTPC, SFTPB
48thymidine43 249.0SFTPB, CSF1, CSF2, IL3, SFTPD
49dexamethasone43 49 28 1210.6CSF2, CSF1, SFTPD, SFTPB, SFTPC, IL3
50cysteine437.6SFTPB, SFTPD, SFTPC, IL3, CSF2, CSF1

GO Terms for genes affiliated with Pulmonary Alveolar Proteinosis

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Cellular components related to Pulmonary Alveolar Proteinosis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:00056157.1CSF1, CSF2, IL3, SFTPC, SFTPD, SFTPB

Biological processes related to Pulmonary Alveolar Proteinosis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1positive regulation of tyrosine phosphorylation of Stat5 proteinGO:00425239.2IL3, CSF2
2respiratory gaseous exchangeGO:00075859.2SFTPC, SFTPD, SFTPB
3positive regulation of macrophage derived foam cell differentiationGO:00107449.0CSF1, CSF2
4positive regulation of DNA replicationGO:00457409.0CSF2, IL3
5positive regulation of cell proliferationGO:00082848.5CSF1, CSF2, IL3

Molecular functions related to Pulmonary Alveolar Proteinosis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1cytokine activityGO:00051258.5CSF1, CSF2, IL3
2growth factor activityGO:00080838.2CSF1, CSF2, IL3

Products for genes affiliated with Pulmonary Alveolar Proteinosis

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Sources for Pulmonary Alveolar Proteinosis

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet