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 55 69
Pulmonary Histiocytosis X 55 69

Characteristics:

Orphanet epidemiological data:

55
adult pulmonary langerhans cell histiocytosis
Inheritance: Not applicable; Age of onset: Adult;

Classifications:



External Ids:

Orphanet 55 ORPHA99874
UMLS via Orphanet 70 C1455705
ICD10 via Orphanet 33 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, and has symptoms including tapered finger, dyspnea and abnormal pattern of respiration. Affiliated tissues include lung and bone.

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 10.3
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:

55 31
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 tapered finger 55 31 occasional (7.5%) Occasional (29-5%) HP:0001182
2 dyspnea 55 31 hallmark (90%) Very frequent (99-80%) HP:0002094
3 abnormal pattern of respiration 55 31 hallmark (90%) Very frequent (99-80%) HP:0002793
4 cough 55 31 hallmark (90%) Very frequent (99-80%) HP:0012735
5 chest pain 55 31 occasional (7.5%) Occasional (29-5%) HP:0100749

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:

38
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
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

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
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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