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 based summary: 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, respiratory rhythm disorder 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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Sources:
48Orphanet, 62UMLS, 26ICD10 via Orphanet
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Adult Pulmonary Langerhans Cell Histiocytosis, Aliases & Descriptions:

Name: Adult Pulmonary Langerhans Cell Histiocytosis 48 62
 
Pulmonary Histiocytosis X 48 62


Classifications:



Characteristics (Orphanet epidemiological data):

48
adult pulmonary langerhans cell histiocytosis:
Inheritance: Sporadic; Age of onset: Adulthood


External Ids:

ICD10 via Orphanet26 C96.5

Related Diseases for Adult Pulmonary Langerhans Cell Histiocytosis

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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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Symptoms:

48
  • respiratory distress/dyspnea/respiratory failure/lung volume reduction
  • respiratory rhythm disorder
  • cough
  • interstitial/restrictive pneumopathy/restrictive respiratory syndrome
  • tapered fingers
  • thoracic/chest pain

HPO human phenotypes related to Adult Pulmonary Langerhans Cell Histiocytosis:

id Description Frequency HPO Source Accession
1 restrictive lung disease hallmark (90%) HP:0002091
2 respiratory insufficiency hallmark (90%) HP:0002093
3 abnormal pattern of respiration hallmark (90%) HP:0002793
4 tapered finger occasional (7.5%) HP:0001182
5 chest pain occasional (7.5%) HP:0100749

Drugs & Therapeutics for Adult Pulmonary Langerhans Cell Histiocytosis

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Drug clinical trials:

Search ClinicalTrials for Adult Pulmonary Langerhans Cell Histiocytosis

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

32
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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Articles related to Adult Pulmonary Langerhans Cell Histiocytosis:

idTitleAuthorsYear
1
Severe pulmonary hypertension in adult pulmonary Langerhans cell histiocytosis: the effect of sildenafil as a bridge to lung transplantation. (25175135)
2014
2
A prospective clinical multicentre study on adult pulmonary Langerhans' cell histiocytosis. (23461076)
2012
3
Effect of pregnancy on lung function in adult pulmonary Langerhans cell histiocytosis. (16492595)
2006
4
Evidence that Langerhans cells in adult pulmonary Langerhans cell histiocytosis are mature dendritic cells: importance of the cytokine microenvironment. (10477625)
1999
5
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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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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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 pathway37
TCR Signaling Pathway37
9.1TNF, IL10
39.1IL10, TNF
4
Show member pathways
9.1IL10, TNF
5
Show member pathways
Toll-like receptor signaling pathway37
Regulation of toll-like receptor signaling pathway37
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 events37
Immune response IL 10 signaling pathway60
Angiopoietin receptor Tie2-mediated signaling37
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 events37
9.1IL10, TNF
209.1IL10, TNF

Compounds for genes affiliated with Adult Pulmonary Langerhans Cell Histiocytosis

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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
1cdp571449.6IL10, TNF
2interferon gamma449.6IL10, TNF
3gabexate mesilate449.6TNF, IL10
4blys449.6TNF, IL10
5toxin-1449.6IL10, TNF
6ulinastatin449.6IL10, TNF
7mip 1alpha449.6TNF, IL10
8natalizumab44 1110.6IL10, TNF
95 aminosalicylic acid449.6TNF, IL10
10il-12449.6TNF, IL10
11adalimumab44 50 1111.6IL10, TNF
12indoleamine449.6IL10, TNF
13imiquimod44 61 28 1112.6TNF, IL10
14cyclosporine50 1110.5IL10, TNF
15polymyxin b449.5IL10, TNF
16groalpha449.5TNF, IL10
17okt3449.5TNF, IL10
18gm-csf44 2810.5IL10, TNF
19lipoteichoic acid449.5TNF, IL10
208-isoprostane449.5IL10, TNF
212,5-oligoadenylate449.5TNF, IL10
22polyinosinic-polycytidylic acid449.5TNF, IL10
23rolipram44 61 2811.5IL10, TNF
24etanercept44 50 1111.5IL10, TNF
25il 10449.5TNF, IL10
26mycophenolate mofetil44 50 1111.5TNF, IL10
27infliximab44 50 1111.5IL10, TNF
28tarc449.5IL10, TNF
29peptidoglycan44 2810.5TNF, IL10
30ivig449.5IL10, TNF
31ccl344 2810.5TNF, IL10
32pentoxifylline44 1110.5TNF, IL10
33neopterin449.5IL10, TNF
3415-deoxy-delta-12,14-prostaglandin j2449.4IL10, TNF
35rituximab44 50 1111.4TNF, IL10
36thalidomide44 50 61 1112.4IL10, TNF
37chloroquine44 2 50 28 1113.4IL10, TNF
38atorvastatin44 50 28 24 1113.4TNF, IL10
39prednisolone44 28 1111.4TNF, IL10
40hydrocortisone44 2 61 1112.4IL10, TNF
41tgf beta1449.4IL10, TNF
42simvastatin44 50 61 28 24 1114.3TNF, IL10
43endotoxin449.3IL10, TNF
44ionomycin449.3IL10, TNF
45prostacyclin449.2TNF, IL10
46tacrolimus44 50 1111.2IL10, TNF
47calcitriol44 61 24 1112.1IL10, TNF
48sb 20358044 6110.1IL10, TNF
49methotrexate50 44 1111.0TNF, IL10
50rantes448.8IL10, TNF

GO Terms for genes affiliated with Adult Pulmonary Langerhans Cell Histiocytosis

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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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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet