MCID: ADL082
MIFTS: 36

Adult Pulmonary Langerhans Cell Histiocytosis malady

Respiratory diseases, Bone diseases, Blood diseases, Cancer diseases, Rare diseases categories
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Summaries for Adult Pulmonary Langerhans Cell Histiocytosis

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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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49Orphanet, 62UMLS, 26ICD10 via Orphanet, 59SNOMED-CT via Orphanet
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Classifications:



Characteristics (Orphanet epidemiological data):

49
adult pulmonary langerhans cell histiocytosis:
Inheritance: Sporadic; Age of onset: Adulthood


Aliases & Descriptions:

adult pulmonary langerhans cell histiocytosis 49 62
pulmonary histiocytosis x 49 62


External Ids:

ICD10 via Orphanet26 C96.5
SNOMED-CT via Orphanet59 417152008

Related Diseases for Adult Pulmonary Langerhans Cell Histiocytosis

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17GeneCards, 18GeneDecks
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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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49Orphanet
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Symptoms:

49
  • 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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42NIH Clinical Center, 6ClinicalTrials
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Drug clinical trials:

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Search NIH Clinical Center 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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33MalaCards
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MalaCards organs/tissues related to Adult Pulmonary Langerhans Cell Histiocytosis:

33
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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52PubMed
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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, 15Gene 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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50PathCards, 30KEGG, 38NCBI BioSystems Database, 53QIAGEN, 55Reactome, 60Thomson Reuters, 54R&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 pathway38
TCR Signaling Pathway38
9.1TNF, IL10
39.1IL10, TNF
4
Show member pathways
9.1IL10, TNF
5
Show member pathways
Toll-like receptor signaling pathway38
Regulation of toll-like receptor signaling pathway38
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 events38
Immune response IL 10 signaling pathway60
Angiopoietin receptor Tie2-mediated signaling38
Development PDGF signaling via STATs and NF kB60
Development Angiopoietin Tie2 signaling60
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 events38
9.1IL10, TNF
209.1IL10, TNF

Compounds for genes affiliated with Adult Pulmonary Langerhans Cell Histiocytosis

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45Novoseek, 11DrugBank, 51PharmGKB, 61Tocris Bioscience, 29IUPHAR, 3BitterDB, 24HMDB
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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
1cdp571459.6IL10, TNF
2interferon gamma459.6IL10, TNF
3gabexate mesilate459.6TNF, IL10
4blys459.6TNF, IL10
5toxin-1459.6IL10, TNF
6ulinastatin459.6IL10, TNF
7mip 1alpha459.6TNF, IL10
8natalizumab45 1110.6IL10, TNF
95 aminosalicylic acid459.6TNF, IL10
10il-12459.6TNF, IL10
11adalimumab45 51 1111.6IL10, TNF
12indoleamine459.6IL10, TNF
13imiquimod45 61 29 1112.6TNF, IL10
14cyclosporine51 1110.5IL10, TNF
15polymyxin b459.5IL10, TNF
16groalpha459.5TNF, IL10
17okt3459.5TNF, IL10
18gm-csf45 2910.5IL10, TNF
19lipoteichoic acid459.5TNF, IL10
208-isoprostane459.5IL10, TNF
212,5-oligoadenylate459.5TNF, IL10
22polyinosinic-polycytidylic acid459.5TNF, IL10
23rolipram45 61 2911.5IL10, TNF
24etanercept45 51 1111.5IL10, TNF
25il 10459.5TNF, IL10
26mycophenolate mofetil45 51 1111.5TNF, IL10
27infliximab45 51 1111.5IL10, TNF
28tarc459.5IL10, TNF
29peptidoglycan45 2910.5TNF, IL10
30ivig459.5IL10, TNF
31ccl345 2910.5TNF, IL10
32pentoxifylline45 1110.5TNF, IL10
33neopterin459.5IL10, TNF
3415-deoxy-delta-12,14-prostaglandin j2459.4IL10, TNF
35rituximab45 51 1111.4TNF, IL10
36thalidomide45 51 61 1112.4IL10, TNF
37chloroquine45 3 51 29 1113.4IL10, TNF
38atorvastatin45 51 29 24 1113.4TNF, IL10
39prednisolone45 29 1111.4TNF, IL10
40hydrocortisone45 3 61 1112.4IL10, TNF
41tgf beta1459.4IL10, TNF
42simvastatin45 51 61 29 24 1114.3TNF, IL10
43endotoxin459.3IL10, TNF
44ionomycin459.3IL10, TNF
45prostacyclin459.2TNF, IL10
46tacrolimus45 51 1111.2IL10, TNF
47calcitriol45 61 24 1112.1IL10, TNF
48sb 20358045 6110.1IL10, TNF
49methotrexate51 45 1111.0TNF, IL10
50rantes458.8IL10, TNF

GO Terms for genes affiliated with Adult Pulmonary Langerhans Cell Histiocytosis

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16Gene 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
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