MCID: ADL082
MIFTS: 36

Adult Pulmonary Langerhans Cell Histiocytosis malady

Respiratory diseases, Bone diseases, Blood diseases, Cancer diseases, Rare diseases categories

Summaries for Adult Pulmonary Langerhans Cell Histiocytosis

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34MalaCards
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MalaCards: Adult Pulmonary Langerhans Cell Histiocytosis, also known as pulmonary histiocytosis x, is related to langerhans-cell histiocytosis and histiocytosis, and has symptoms including respiratory distress/dyspnea/respiratory failure/lung volume reduction, thoracic/chest pain and cough. An important gene associated with Adult Pulmonary Langerhans Cell Histiocytosis is IL10 (interleukin 10), and among its related pathways are Allograft rejection and B Cell Receptor Signaling Pathway. The compounds cdp571 and interferon gamma have been mentioned in the context of this disorder. Affiliated tissues include lung and bone.

Aliases & Classifications for Adult Pulmonary Langerhans Cell Histiocytosis

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50Orphanet, 63UMLS, 27ICD10 via Orphanet, 60SNOMED-CT via Orphanet
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Classifications:



Characteristics (Orphanet epidemiological data):

50
adult pulmonary langerhans cell histiocytosis:
Inheritance: Sporadic; Age of onset: Adulthood


Aliases & Descriptions:

adult pulmonary langerhans cell histiocytosis 50 63
pulmonary histiocytosis x 50 63


External Ids:

ICD10 via Orphanet27 C96.5
SNOMED-CT via Orphanet60 417152008

Related Diseases for Adult Pulmonary Langerhans Cell Histiocytosis

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18GeneCards, 19GeneDecks
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Graphical network of the top 20 diseases related to Adult Pulmonary Langerhans Cell Histiocytosis:



Diseases related to adult pulmonary langerhans cell histiocytosis

Symptoms for Adult Pulmonary Langerhans Cell Histiocytosis

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50Orphanet
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Symptoms:

50
  • respiratory distress/dyspnea/respiratory failure/lung volume reduction
  • thoracic/chest pain
  • cough
  • interstitial/restrictive pneumopathy/restrictive respiratory syndrome
  • respiratory rhythm disorder
  • tapered fingers

Drugs & Therapeutics for Adult Pulmonary Langerhans Cell Histiocytosis

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6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
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Approved drugs:

Search CenterWatch for Adult Pulmonary Langerhans Cell Histiocytosis

Drug clinical trials:

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Search CenterWatch for Adult Pulmonary Langerhans Cell Histiocytosis

Genetic Tests for Adult Pulmonary Langerhans Cell Histiocytosis

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Anatomical Context for Adult Pulmonary Langerhans Cell Histiocytosis

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34MalaCards
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MalaCards organs/tissues related to Adult Pulmonary Langerhans Cell Histiocytosis:

34
Lung, Bone

Animal Models for Adult Pulmonary Langerhans Cell Histiocytosis or affiliated genes

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Publications for Adult Pulmonary Langerhans Cell Histiocytosis

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53PubMed
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Articles related to Adult Pulmonary Langerhans Cell Histiocytosis:

idTitleAuthorsYear
1
A prospective clinical multicentre study on adult pulmonary Langerhans' cell histiocytosis. (23461076)
2012
2
Effect of pregnancy on lung function in adult pulmonary Langerhans cell histiocytosis. (16492595)
2006
3
Evidence that Langerhans cells in adult pulmonary Langerhans cell histiocytosis are mature dendritic cells: importance of the cytokine microenvironment. (10477625)
1999
4
Relapsing nodular lesions in the course of adult pulmonary Langerhans cell histiocytosis. (9620939)
1998

Variations for Adult Pulmonary Langerhans Cell Histiocytosis

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Expression for genes affiliated with Adult Pulmonary Langerhans Cell Histiocytosis

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Adult Pulmonary Langerhans Cell Histiocytosis

Search GEO for disease gene expression data for Adult Pulmonary Langerhans Cell Histiocytosis.

Pathways for genes affiliated with Adult Pulmonary Langerhans Cell Histiocytosis

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51PathCards, 31KEGG, 39NCBI BioSystems Database, 54QIAGEN, 56Reactome, 61Thomson Reuters, 55R&D Systems
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Pathways related to Adult Pulmonary Langerhans Cell Histiocytosis according to GeneCards/GeneDecks:

(show all 20)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.1IL10, TNF
2
Show member pathways
BCR signaling pathway39
TCR Signaling Pathway39
9.1TNF, IL10
39.1IL10, TNF
4
Show member pathways
9.1IL10, TNF
5
Show member pathways
Toll-like receptor signaling pathway39
Regulation of toll-like receptor signaling pathway39
9.1TNF, IL10
6
Show member pathways
9.1IL10, TNF
7
Show member pathways
9.1IL10, TNF
8
Show member pathways
9.1IL10, TNF
9
Show member pathways
9.1IL10, TNF
109.1TNF, IL10
11
Show member pathways
IL23-mediated signaling events39
Immune response IL 10 signaling pathway61
Angiopoietin receptor Tie2-mediated signaling39
Development PDGF signaling via STATs and NF kB61
Development Angiopoietin Tie2 signaling61
9.1IL10, TNF
129.1IL10, TNF
139.1TNF, IL10
14
Show member pathways
9.1IL10, TNF
159.1IL10, TNF
169.1TNF, IL10
179.1TNF, IL10
189.1IL10, TNF
19
Show member pathways
IL27-mediated signaling events39
9.1IL10, TNF
209.1IL10, TNF

Compounds for genes affiliated with Adult Pulmonary Langerhans Cell Histiocytosis

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46Novoseek, 12DrugBank, 52PharmGKB, 62Tocris Bioscience, 30IUPHAR, 3BitterDB, 25HMDB
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Compounds related to Adult Pulmonary Langerhans Cell Histiocytosis according to GeneCards/GeneDecks:

(show top 50)    (show all 56)
idCompoundScoreTop Affiliating Genes
1cdp571469.6IL10, TNF
2interferon gamma469.6IL10, TNF
3gabexate mesilate469.6TNF, IL10
4blys469.6TNF, IL10
5toxin-1469.6IL10, TNF
6ulinastatin469.6IL10, TNF
7mip 1alpha469.6TNF, IL10
8natalizumab46 1210.6IL10, TNF
95 aminosalicylic acid469.6TNF, IL10
10il-12469.6TNF, IL10
11adalimumab46 52 1211.6IL10, TNF
12indoleamine469.6IL10, TNF
13imiquimod46 62 30 1212.6TNF, IL10
14cyclosporine52 1210.5IL10, TNF
15polymyxin b469.5IL10, TNF
16groalpha469.5TNF, IL10
17okt3469.5TNF, IL10
18gm-csf46 3010.5IL10, TNF
19lipoteichoic acid469.5TNF, IL10
208-isoprostane469.5IL10, TNF
212,5-oligoadenylate469.5TNF, IL10
22polyinosinic-polycytidylic acid469.5TNF, IL10
23rolipram46 62 3011.5IL10, TNF
24etanercept46 52 1211.5IL10, TNF
25il 10469.5TNF, IL10
26mycophenolate mofetil46 52 1211.5TNF, IL10
27infliximab46 52 1211.5IL10, TNF
28tarc469.5IL10, TNF
29peptidoglycan46 3010.5TNF, IL10
30ivig469.5IL10, TNF
31ccl346 3010.5TNF, IL10
32pentoxifylline46 1210.5TNF, IL10
33neopterin469.5IL10, TNF
3415-deoxy-delta-12,14-prostaglandin j2469.4IL10, TNF
35rituximab46 52 1211.4TNF, IL10
36thalidomide46 52 62 1212.4IL10, TNF
37chloroquine46 3 52 30 1213.4IL10, TNF
38atorvastatin46 52 30 25 1213.4TNF, IL10
39prednisolone46 30 1211.4TNF, IL10
40hydrocortisone46 3 62 1212.4IL10, TNF
41tgf beta1469.4IL10, TNF
42simvastatin46 52 62 30 25 1214.3TNF, IL10
43endotoxin469.3IL10, TNF
44ionomycin469.3IL10, TNF
45prostacyclin469.2TNF, IL10
46tacrolimus46 52 1211.2IL10, TNF
47calcitriol46 62 25 1212.1IL10, TNF
48sb 20358046 6210.1IL10, TNF
49methotrexate52 46 1211.0TNF, IL10
50rantes468.8IL10, TNF

GO Terms for genes affiliated with Adult Pulmonary Langerhans Cell Histiocytosis

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17Gene Ontology
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Biological processes related to Adult Pulmonary Langerhans Cell Histiocytosis according to GeneCards/GeneDecks:

(show all 11)
idNameGO IDScoreTop Affiliating Genes
1receptor biosynthetic processGO:0328009.4IL10, TNF
2negative regulation of cytokine secretion involved in immune responseGO:0027409.4IL10, TNF
3negative regulation of growth of symbiont in hostGO:0441309.3TNF, IL10
4negative regulation of interleukin-6 productionGO:0327159.3IL10, TNF
5positive regulation of cytokine secretionGO:0507159.3IL10, TNF
6response to activityGO:0148239.2TNF, IL10
7response to glucocorticoidGO:0513849.2IL10, TNF
8positive regulation of sequence-specific DNA binding transcription factor activityGO:0510919.1IL10, TNF
9positive regulation of transcription, DNA-templatedGO:0458939.1IL10, TNF
10response to drugGO:0424939.0TNF, IL10
11inflammatory responseGO:0069548.8IL10, TNF

Molecular functions related to Adult Pulmonary Langerhans Cell Histiocytosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cytokine activityGO:0051259.1IL10, TNF

Products for genes affiliated with Adult Pulmonary Langerhans Cell Histiocytosis

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Sources for Adult Pulmonary Langerhans Cell Histiocytosis

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet