MCID: ADL082
MIFTS: 32

Adult Pulmonary Langerhans Cell Histiocytosis malady

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

Aliases & Classifications for Adult Pulmonary Langerhans Cell Histiocytosis

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Sources:
47Orphanet, 60UMLS, 26ICD10 via Orphanet
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Adult Pulmonary Langerhans Cell Histiocytosis, Aliases & Descriptions:

Name: Adult Pulmonary Langerhans Cell Histiocytosis 47 60
 
Pulmonary Histiocytosis X 47 60


Classifications:



Characteristics (Orphanet epidemiological data):

47
adult pulmonary langerhans cell histiocytosis:
Inheritance: Not applicable; Age of onset: Adult


External Ids:

Orphanet47 99874
ICD10 via Orphanet26 C96.5

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 restrictive lung disease, respiratory insufficiency and abnormal pattern of respiration. 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.

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:

 47
  • 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:

31
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
The natural history of adult pulmonary Langerhans cell histiocytosis: a prospective multicentre study. (25887097)
2015
2
Severe pulmonary hypertension in adult pulmonary Langerhans cell histiocytosis: the effect of sildenafil as a bridge to lung transplantation. (25175135)
2014
3
A prospective clinical multicentre study on adult pulmonary Langerhans' cell histiocytosis. (23461076)
2012
4
Effect of pregnancy on lung function in adult pulmonary Langerhans cell histiocytosis. (16492595)
2006
5
Evidence that Langerhans cells in adult pulmonary Langerhans cell histiocytosis are mature dendritic cells: importance of the cytokine microenvironment. (10477625)
1999
6
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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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 Suite gene sharing:

(show all 20)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.1IL10, TNF
2
Show member pathways
BCR signaling pathway36
TCR Signaling Pathway36
9.1TNF, IL10
39.1IL10, TNF
4
Show member pathways
9.1IL10, TNF
5
Show member pathways
Toll-like receptor signaling pathway36
Regulation of toll-like receptor signaling pathway36
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 events36
Immune response IL 10 signaling pathway58
Angiopoietin receptor Tie2-mediated signaling36
Development PDGF signaling via STATs and NF kB58
Development Angiopoietin Tie2 signaling58
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 events36
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 Suite gene sharing:

(show top 50)    (show all 56)
idCompoundScoreTop Affiliating Genes
1cdp571439.6IL10, TNF
2interferon gamma439.6IL10, TNF
3gabexate mesilate439.6TNF, IL10
4blys439.6TNF, IL10
5toxin-1439.6IL10, TNF
6ulinastatin439.6IL10, TNF
7mip 1alpha439.6TNF, IL10
8natalizumab43 1210.6IL10, TNF
95 aminosalicylic acid439.6TNF, IL10
10il-12439.6TNF, IL10
11adalimumab43 49 1211.6IL10, TNF
12indoleamine439.6IL10, TNF
13imiquimod43 59 28 1212.6TNF, IL10
14cyclosporine49 1210.5IL10, TNF
15polymyxin b439.5IL10, TNF
16groalpha439.5TNF, IL10
17okt3439.5TNF, IL10
18gm-csf43 2810.5IL10, TNF
19lipoteichoic acid439.5TNF, IL10
208-isoprostane439.5IL10, TNF
212,5-oligoadenylate439.5TNF, IL10
22polyinosinic-polycytidylic acid439.5TNF, IL10
23rolipram43 59 2811.5IL10, TNF
24etanercept43 49 1211.5IL10, TNF
25il 10439.5TNF, IL10
26mycophenolate mofetil43 49 1211.5TNF, IL10
27infliximab43 49 1211.5IL10, TNF
28tarc439.5IL10, TNF
29peptidoglycan43 2810.5TNF, IL10
30ivig439.5IL10, TNF
31ccl343 2810.5TNF, IL10
32pentoxifylline43 1210.5TNF, IL10
33neopterin439.5IL10, TNF
3415-deoxy-delta-12,14-prostaglandin j2439.4IL10, TNF
35rituximab43 49 1211.4TNF, IL10
36thalidomide43 49 59 1212.4IL10, TNF
37chloroquine43 2 49 28 1213.4IL10, TNF
38atorvastatin43 49 28 24 1213.4TNF, IL10
39prednisolone43 28 1211.4TNF, IL10
40hydrocortisone43 2 59 1212.4IL10, TNF
41tgf beta1439.4IL10, TNF
42simvastatin43 49 59 28 24 1214.3TNF, IL10
43endotoxin439.3IL10, TNF
44ionomycin439.3IL10, TNF
45prostacyclin439.2TNF, IL10
46tacrolimus43 49 1211.2IL10, TNF
47calcitriol43 59 24 1212.1IL10, TNF
48sb 20358043 5910.1IL10, TNF
49methotrexate49 43 1211.0TNF, IL10
50rantes438.8IL10, 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 Suite gene sharing:

(show all 11)
idNameGO IDScoreTop Affiliating Genes
1receptor biosynthetic processGO:00328009.4IL10, TNF
2negative regulation of cytokine secretion involved in immune responseGO:00027409.4IL10, TNF
3negative regulation of growth of symbiont in hostGO:00441309.3TNF, IL10
4negative regulation of interleukin-6 productionGO:00327159.3IL10, TNF
5positive regulation of cytokine secretionGO:00507159.3IL10, TNF
6response to activityGO:00148239.2TNF, IL10
7response to glucocorticoidGO:00513849.2IL10, TNF
8positive regulation of sequence-specific DNA binding transcription factor activityGO:00510919.1IL10, TNF
9positive regulation of transcription, DNA-templatedGO:00458939.1IL10, TNF
10response to drugGO:00424939.0TNF, IL10
11inflammatory responseGO:00069548.8IL10, TNF

Molecular functions related to Adult Pulmonary Langerhans Cell Histiocytosis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1cytokine activityGO:00051259.1IL10, 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
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet