MCID: ADL082
MIFTS: 26

Adult Pulmonary Langerhans Cell Histiocytosis malady

Respiratory, Blood, Cancer categories

Summaries for Adult Pulmonary Langerhans Cell Histiocytosis

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33MalaCards
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MalaCards: Adult Pulmonary Langerhans Cell Histiocytosis, also known as pulmonary histiocytosis x, is related to pulmonary function and bronchogenic carcinoma, 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 SIDS Susceptibility Pathways and Development PDGF signaling via STATs and NF-kB. The compounds cdp571 and interferon gamma have been mentioned in the context of this disorder. Affiliated tissues include lung.

Aliases & Classifications for Adult Pulmonary Langerhans Cell Histiocytosis

Sources:
49Orphanet, 61UMLS, 26ICD10 via Orphanet
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer
Anatomical: Respiratory, Blood


Characteristics (Orphanet epidemiological data):

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


Aliases & Descriptions:

adult pulmonary langerhans cell histiocytosis 49 61
pulmonary histiocytosis x 49 61


External Ids:

ICD10 via Orphanet26 C96.5

Related Diseases for Adult Pulmonary Langerhans Cell Histiocytosis

Sources:
17GeneCards, 18GeneDecks
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Diseases related to Adult Pulmonary Langerhans Cell Histiocytosis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show all 44)
idRelated DiseaseScoreTop Affiliating Genes
1pulmonary function10.4
2bronchogenic carcinoma10.1
3blau syndrome10.0TNF
4aids dementia complex10.0TNF
5toxic shock syndrome10.0TNF
6chorioamnionitis10.0TNF
7arthropathy10.0TNF
8familial mediterranean fever10.0TNF
9respiratory failure10.0TNF
10interstitial lung disease10.0TNF
11psoriatic arthritis10.0TNF
12periodontal disease10.0TNF
13ankylosing spondylitis10.0TNF
14paracoccidioidomycosis10.0IL10
15common variable immune deficiency10.0IL10
16vasculitis10.0TNF
17schistosomiasis10.0IL10
18tetanus10.0IL10
19myasthenia gravis10.0IL10
20lupus nephritis10.0IL10
21atopy10.0IL10
22leishmaniasis10.0IL10
23acute pancreatitis10.0IL10
24allergic rhinitis10.0IL10
25sarcoidosis10.0TNF
26alcoholic hepatitis10.0IL10, TNF
27acute graft versus host disease10.0TNF, IL10
28cerebral malaria10.0TNF, IL10
29periodontitis10.0IL10, TNF
30graft versus host disease10.0TNF, IL10
31septic shock10.0IL10, TNF
32hepatitis c10.0TNF, IL10
33congenital heart defect10.0TNF, IL10
34atopic dermatitis10.0IL10, TNF
35hypersensitivity reaction type ii disease10.0IL10, TNF
36cork-handlers' disease10.0TNF, IL10
37skin disease10.0TNF, IL10
38connective tissue disease10.0IL10, TNF
39uveitis10.0IL10, TNF
40ulcerative colitis10.0IL10, TNF
41sepsis10.0TNF, IL10
42inflammatory bowel disease10.0TNF, IL10
43encephalomyelitis10.0TNF, IL10
44systemic lupus erythematosus10.0IL10, TNF

Graphical network of the top 20 diseases related to Adult Pulmonary Langerhans Cell Histiocytosis:



Diseases related to adult pulmonary langerhans cell histiocytosis

Clinical Features for Adult Pulmonary Langerhans Cell Histiocytosis

Sources:
49Orphanet
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Symptoms:

49 (show all 6)
  • 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

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

Search CenterWatch for Adult Pulmonary Langerhans Cell Histiocytosis

Drug clinical trials:

Search ClinicalTrials for Adult Pulmonary Langerhans Cell Histiocytosis

Search NIH Clinical Center for Adult Pulmonary Langerhans Cell Histiocytosis

Search CenterWatch for Adult Pulmonary Langerhans Cell Histiocytosis

Genetic Tests for Adult Pulmonary Langerhans Cell Histiocytosis

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

Animal Models for Adult Pulmonary Langerhans Cell Histiocytosis or affiliated genes

Sources:
28inGenious Targeting Laboratory
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Publications for Adult Pulmonary Langerhans Cell Histiocytosis

Sources:
51PubMed
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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
Pulmonary Langerhans cell histiocytosis: a comparative study of computed tomography in children and adults. (21042223)
2012
3
Pulmonary Langerhans cell histiocytosis in adults: high-resolution CT-pathology comparisons and evolutional changes at CT. (21311888)
2011
4
Pulmonary histiocytic sarcoma mimicking pulmonary Langerhans cell histiocytosis in a young adult presenting with spontaneous pneumothorax: a potential diagnostic pitfall. (19562369)
2009
5
Effect of pregnancy on lung function in adult pulmonary Langerhans cell histiocytosis. (16492595)
2006
6
Evidence that Langerhans cells in adult pulmonary Langerhans cell histiocytosis are mature dendritic cells: importance of the cytokine microenvironment. (10477625)
1999
7
Relapsing nodular lesions in the course of adult pulmonary Langerhans cell histiocytosis. (9620939)
1998

Genetic Variations for Adult Pulmonary Langerhans Cell Histiocytosis

Expression for genes affiliated with Adult Pulmonary Langerhans Cell Histiocytosis

Sources:
1BioGPS, 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

Sources:
38NCBI BioSystems Database, 12EMD Millipore, 30KEGG, 52QIAGEN, 53R&D Systems
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Compounds for genes affiliated with Adult Pulmonary Langerhans Cell Histiocytosis

Sources:
45Novoseek, 11DrugBank, 50PharmGKB, 29IUPHAR, 60Tocris Bioscience, 2BitterDB, 24HMDB
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Compounds related to Adult Pulmonary Langerhans Cell Histiocytosis according to GeneCards/GeneDecks:

(show all 41)
idCompoundScoreTop Affiliating Genes
1cdp571459.6IL10, TNF
2interferon gamma459.6IL10, TNF
3blys459.6TNF, IL10
4toxin-1459.6IL10, TNF
5mip 1alpha459.5IL10, TNF
6natalizumab45 1110.5TNF, IL10
7adalimumab45 50 1111.5IL10, TNF
8il-12459.5TNF, IL10
95 aminosalicylic acid459.5TNF, IL10
10indoleamine459.5IL10, TNF
11imiquimod45 29 60 1112.5TNF, IL10
12polymyxin b459.5IL10, TNF
13groalpha459.5TNF, IL10
14okt3459.5TNF, IL10
15gm-csf45 2910.5IL10, TNF
16lipoteichoic acid459.5TNF, IL10
178-isoprostane459.5IL10, TNF
18polyinosinic-polycytidylic acid459.5TNF, IL10
192,5-oligoadenylate459.5TNF, IL10
20etanercept45 50 1111.5IL10, TNF
21il 10459.5TNF, IL10
22infliximab45 50 1111.5IL10, TNF
23tarc459.5IL10, TNF
24peptidoglycan45 2910.5TNF, IL10
25ivig459.4IL10, TNF
26ccl345 2910.4TNF, IL10
27neopterin459.4IL10, TNF
28rituximab45 50 1111.4IL10, TNF
2915-deoxy-delta-12,14-prostaglandin j2459.4TNF, IL10
30thalidomide45 50 60 1112.4IL10, TNF
31chloroquine45 2 50 29 1113.4TNF, IL10
32atorvastatin45 50 29 11 2413.4IL10, TNF
33tgf beta1459.3IL10, TNF
34simvastatin45 50 60 29 11 2414.3TNF, IL10
35ionomycin459.3IL10, TNF
36prostacyclin459.2TNF, IL10
37tacrolimus45 50 1111.2IL10, TNF
38calcitriol45 60 11 2412.1IL10, TNF
39sb 20358045 6010.1IL10, TNF
40methotrexate45 50 1111.0TNF, IL10
41rantes458.8IL10, TNF

GO Terms for genes affiliated with Adult Pulmonary Langerhans Cell Histiocytosis

Sources:
16Gene Ontology
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Biological processes related to Adult Pulmonary Langerhans Cell Histiocytosis according to GeneCards/GeneDecks:

(show all 10)
idNameGO IDScoreTop Affiliating Genes
1receptor biosynthetic processGO:0328009.4IL10, TNF
2negative regulation of cytokine secretion involved in immune responseGO:0027409.3IL10, TNF
3negative regulation of growth of symbiont in hostGO:0441309.3TNF, IL10
4negative regulation of interleukin-6 productionGO:0327159.3IL10, TNF
5response to activityGO:0148239.2IL10, TNF
6positive regulation of cytokine secretionGO:0507159.2TNF, IL10
7response to glucocorticoid stimulusGO:0513849.1IL10, TNF
8inflammatory responseGO:0069549.1IL10, TNF
9positive regulation of sequence-specific DNA binding transcription factor activityGO:0510919.0IL10, TNF
10response to drugGO:0424938.8IL10, 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

3CDC
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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet