MCID: ADL082
MIFTS: 20

Adult Pulmonary Langerhans Cell Histiocytosis

Categories: Respiratory diseases, Bone diseases, Blood diseases, Rare diseases

Aliases & Classifications for Adult Pulmonary Langerhans Cell Histiocytosis

MalaCards integrated aliases for Adult Pulmonary Langerhans Cell Histiocytosis:

Name: Adult Pulmonary Langerhans Cell Histiocytosis 59 73
Pulmonary Histiocytosis X 59 73

Characteristics:

Orphanet epidemiological data:

59
adult pulmonary langerhans cell histiocytosis
Inheritance: Not applicable; Age of onset: Adult;

Classifications:



External Ids:

Orphanet 59 ORPHA99874
UMLS via Orphanet 74 C1455705
ICD10 via Orphanet 34 C96.5

Summaries for Adult Pulmonary Langerhans Cell Histiocytosis

MalaCards based summary : Adult Pulmonary Langerhans Cell Histiocytosis, also known as pulmonary histiocytosis x, is related to langerhans cell histiocytosis and histiocytosis. Affiliated tissues include lung and bone, and related phenotypes are dyspnea and chest pain

Related Diseases for Adult Pulmonary Langerhans Cell Histiocytosis

Diseases related to Adult Pulmonary Langerhans Cell Histiocytosis via text searches within MalaCards or GeneCards Suite gene sharing:

# Related Disease Score Top Affiliating Genes
1 langerhans cell histiocytosis 10.4
2 histiocytosis 10.3
3 pulmonary hypertension 9.9

Symptoms & Phenotypes for Adult Pulmonary Langerhans Cell Histiocytosis

Human phenotypes related to Adult Pulmonary Langerhans Cell Histiocytosis:

59 32
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 dyspnea 59 32 hallmark (90%) Very frequent (99-80%) HP:0002094
2 chest pain 59 32 occasional (7.5%) Occasional (29-5%) HP:0100749
3 cough 59 32 hallmark (90%) Very frequent (99-80%) HP:0012735
4 tapered finger 59 32 occasional (7.5%) Occasional (29-5%) HP:0001182
5 abnormal pattern of respiration 59 32 hallmark (90%) Very frequent (99-80%) HP:0002793

Drugs & Therapeutics for Adult Pulmonary Langerhans Cell Histiocytosis

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Prospective Evaluation of Adult Pulmonary Langerhans Cell Histiocytosis Completed NCT01225601
2 Evaluation of Outcome of Adult Pulmonary Langerhans Cell Histiocytosis Based on Lung HRCT and Lung Function Completed NCT01651507

Search NIH Clinical Center for Adult Pulmonary Langerhans Cell Histiocytosis

Genetic Tests for Adult Pulmonary Langerhans Cell Histiocytosis

Anatomical Context for Adult Pulmonary Langerhans Cell Histiocytosis

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

41
Lung, Bone

Publications for Adult Pulmonary Langerhans Cell Histiocytosis

Articles related to Adult Pulmonary Langerhans Cell Histiocytosis:

# Title Authors Year
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
[Adult pulmonary Langerhans cell histiocytosis. Report of one case]. ( 21773662 )
2011
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

Variations for Adult Pulmonary Langerhans Cell Histiocytosis

Expression for Adult Pulmonary Langerhans Cell Histiocytosis

Search GEO for disease gene expression data for Adult Pulmonary Langerhans Cell Histiocytosis.

Pathways for Adult Pulmonary Langerhans Cell Histiocytosis

GO Terms for Adult Pulmonary Langerhans Cell Histiocytosis

Sources for Adult Pulmonary Langerhans Cell Histiocytosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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