MCID: ADL082
MIFTS: 33

Adult Pulmonary Langerhans Cell Histiocytosis malady

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

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 histiocytosis and langerhans-cell 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 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 bone and lung.

Aliases & Classifications for Adult Pulmonary Langerhans Cell Histiocytosis

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48Orphanet, 60UMLS, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet
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Classifications:

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


Characteristics (Orphanet epidemiological data):

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


Aliases & Descriptions:

adult pulmonary langerhans cell histiocytosis 48 60
pulmonary histiocytosis x 48 60


External Ids:

ICD10 via Orphanet26 C96.5
SNOMED-CT via Orphanet57 417152008

Related Diseases for Adult Pulmonary Langerhans Cell Histiocytosis

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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 45)
idRelated DiseaseScoreTop Affiliating Genes
1histiocytosis10.6
2langerhans-cell histiocytosis10.6
3bronchogenic carcinoma10.1
4blau syndrome10.0TNF
5aids dementia complex10.0TNF
6toxic shock syndrome10.0TNF
7chorioamnionitis10.0TNF
8arthropathy10.0TNF
9familial mediterranean fever10.0TNF
10respiratory failure10.0TNF
11interstitial lung disease10.0TNF
12psoriatic arthritis10.0TNF
13periodontal disease10.0TNF
14ankylosing spondylitis10.0TNF
15paracoccidioidomycosis10.0IL10
16common variable immune deficiency10.0IL10
17vasculitis10.0TNF
18schistosomiasis10.0IL10
19tetanus10.0IL10
20myasthenia gravis10.0IL10
21lupus nephritis10.0IL10
22atopy10.0IL10
23leishmaniasis10.0IL10
24acute pancreatitis10.0IL10
25allergic rhinitis10.0IL10
26sarcoidosis10.0TNF
27alcoholic hepatitis10.0IL10, TNF
28acute graft versus host disease10.0TNF, IL10
29cerebral malaria10.0IL10, TNF
30periodontitis10.0IL10, TNF
31graft versus host disease10.0IL10, TNF
32septic shock10.0IL10, TNF
33hepatitis c10.0IL10, TNF
34congenital heart defect10.0IL10, TNF
35atopic dermatitis10.0TNF, IL10
36hypersensitivity reaction type ii disease10.0IL10, TNF
37cork-handlers' disease10.0TNF, IL10
38skin disease10.0IL10, TNF
39connective tissue disease10.0TNF, IL10
40uveitis10.0IL10, TNF
41ulcerative colitis10.0IL10, TNF
42sepsis10.0IL10, TNF
43inflammatory bowel disease10.0TNF, IL10
44encephalomyelitis10.0IL10, TNF
45systemic 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

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48Orphanet
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Symptoms:

48
  • 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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5CenterWatch, 41NIH 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

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Anatomical Context for Adult Pulmonary Langerhans Cell Histiocytosis

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32MalaCards
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MalaCards organs/tissues related to Adult Pulmonary Langerhans Cell Histiocytosis:

32
Bone, Lung

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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Genetic 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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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

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Sources:
37NCBI BioSystems Database, 12EMD Millipore, 29KEGG, 51QIAGEN, 52R&D Systems
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Compounds for genes affiliated with Adult Pulmonary Langerhans Cell Histiocytosis

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44Novoseek, 11DrugBank, 49PharmGKB, 28IUPHAR, 59Tocris 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
1cdp571449.6IL10, TNF
2interferon gamma449.6IL10, TNF
3blys449.6TNF, IL10
4toxin-1449.6TNF, IL10
5mip 1alpha449.5TNF, IL10
6natalizumab44 1110.5IL10, TNF
7adalimumab44 49 1111.5IL10, TNF
8il-12449.5TNF, IL10
95 aminosalicylic acid449.5IL10, TNF
10indoleamine449.5IL10, TNF
11imiquimod44 28 59 1112.5TNF, IL10
12polymyxin b449.5IL10, TNF
13groalpha449.5IL10, TNF
14okt3449.5TNF, IL10
15gm-csf44 2810.5IL10, TNF
16lipoteichoic acid449.5IL10, TNF
178-isoprostane449.5IL10, TNF
182,5-oligoadenylate449.5TNF, IL10
19polyinosinic-polycytidylic acid449.5TNF, IL10
20etanercept44 49 1111.5IL10, TNF
21il 10449.5TNF, IL10
22infliximab44 49 1111.5TNF, IL10
23tarc449.5IL10, TNF
24peptidoglycan44 2810.5IL10, TNF
25ivig449.4TNF, IL10
26ccl344 2810.4IL10, TNF
27neopterin449.4TNF, IL10
28rituximab44 49 1111.4IL10, TNF
2915-deoxy-delta-12,14-prostaglandin j2449.4TNF, IL10
30thalidomide44 49 59 1112.4IL10, TNF
31chloroquine44 2 49 28 1113.4IL10, TNF
32atorvastatin44 49 28 11 2413.4TNF, IL10
33tgf beta1449.3IL10, TNF
34simvastatin44 49 59 28 11 2414.3IL10, TNF
35ionomycin449.3IL10, TNF
36prostacyclin449.2TNF, IL10
37tacrolimus44 49 1111.2IL10, TNF
38calcitriol44 59 11 2412.1TNF, IL10
39sb 20358044 5910.1TNF, IL10
40methotrexate44 49 1111.0TNF, IL10
41rantes448.8TNF, IL10

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 10)
idNameGO IDScoreTop Affiliating Genes
1receptor biosynthetic processGO:0328009.4TNF, IL10
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:0327159.3TNF, IL10
5response to activityGO:0148239.2TNF, IL10
6positive regulation of cytokine secretionGO:0507159.2TNF, IL10
7response to glucocorticoid stimulusGO:0513849.1IL10, TNF
8inflammatory responseGO:0069549.1TNF, IL10
9positive regulation of sequence-specific DNA binding transcription factor activityGO:0510919.0IL10, TNF
10response to drugGO:0424938.8TNF, IL10

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
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet