MCID: ADL082
MIFTS: 24

Adult Pulmonary Langerhans Cell Histiocytosis

Categories: Blood diseases, Bone diseases, Rare diseases, Respiratory 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 72
Pulmonary Histiocytosis X 59 72

Characteristics:

Orphanet epidemiological data:

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

Classifications:



External Ids:

ICD10 via Orphanet 34 C96.5
UMLS via Orphanet 73 C1455705
Orphanet 59 ORPHA99874
UMLS 72 C1455705 C3161104

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, bone and lymph node, and related phenotypes are dyspnea and abnormal pattern of respiration

Related Diseases for Adult Pulmonary Langerhans Cell Histiocytosis

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



Diseases related to Adult Pulmonary Langerhans Cell Histiocytosis

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 abnormal pattern of respiration 59 32 hallmark (90%) Very frequent (99-80%) HP:0002793
3 cough 59 32 hallmark (90%) Very frequent (99-80%) HP:0012735
4 chest pain 59 32 occasional (7.5%) Occasional (29-5%) HP:0100749
5 tapered finger 59 32 occasional (7.5%) Occasional (29-5%) HP:0001182

Drugs & Therapeutics for Adult Pulmonary Langerhans Cell Histiocytosis

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Epidemiology of Adult Adult Pulmonary Langerhans Cell Histiocytosis - A Multicenter Cohort Study Completed NCT01225601
2 Evaluation of Outcome of Adult Pulmonary Langerhans Cell Histiocytosis Based on Lung HRCT and Lung Function. A Multicenter Retrospective Study 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, Lymph Node

Publications for Adult Pulmonary Langerhans Cell Histiocytosis

Articles related to Adult Pulmonary Langerhans Cell Histiocytosis:

(show top 50) (show all 158)
# Title Authors PMID Year
1
[Adult pulmonary Langerhans cell histiocytosis]. 38
27816342 2017
2
The natural history of adult pulmonary Langerhans cell histiocytosis: a prospective multicentre study. 38
25887097 2015
3
Rare interstitial lung disease: Pulmonary Langerhans Cell Histiocytosis in a young non smoking Indian female. 38
25857566 2015
4
[Adult pulmonary Langerhans' cell histiocytosis: approach to the reality of the Spanish population]. 38
24726261 2014
5
Respiratory diseases registries in the national registry of rare diseases. 38
24703816 2014
6
[THE SPONTANEOUS PNEUMOTHORAX INTERPRETATION, DIFFICULTIES DURING HISTIOCYTOSIS X]. 38
26492790 2014
7
Severe pulmonary hypertension in adult pulmonary Langerhans cell histiocytosis: the effect of sildenafil as a bridge to lung transplantation. 38
25175135 2014
8
[Computed tomography in the assessment of causes of restrictive lung disorders in patients with pulmonary histiocytosis X]. 38
25702437 2013
9
A prospective clinical multicentre study on adult pulmonary Langerhans' cell histiocytosis. 38
23461076 2012
10
[Adult pulmonary Langerhans cell histiocytosis. Report of one case]. 38
21773662 2011
11
Langerhans cell histiocytosis of the lung with probably skeletal involvement. 38
21512670 2011
12
[Clinical features of adult pulmonary Langerhans' cell histiocytosis]. 38
19035226 2008
13
[Resolution of a bone lesion in a patient with pulmonary histiocytosis X]. 38
18682155 2008
14
Cigarette smoking and diffuse lung disease. 38
18627208 2008
15
[Pulmonary histiocytosis X]. 38
17526202 2007
16
Pulmonary histiocytosis X in a patient with epithelioid mesothelioma. 38
16918985 2006
17
Adult pulmonary Langerhans' cell histiocytosis. 38
16772390 2006
18
Effect of pregnancy on lung function in adult pulmonary Langerhans cell histiocytosis. 38
16492595 2006
19
[Adult pulmonary Langerhans cell histiocytosis]. 38
15710263 2005
20
[Pulmonary histiocytosis X]. 38
14567097 2003
21
[Adult pulmonary Langerhans' cell histiocytosis]. 38
12706779 2003
22
[Pulmonary histiocytosis X--current presentation, diagnosis, and treatment tactics]. 38
12718227 2003
23
[Histiocytosis X and bronchopulmonary adenocarcinoma: a rare coexistence]. 38
12522489 2002
24
[A case of pulmonary histiocytosis X]. 38
11899808 2002
25
[Pulmonary histiocytosis X. Report of a case and review of the literature]. 38
11989074 2002
26
Clinico-epidemiological features of pulmonary histiocytosis X. 38
11688843 2001
27
[Histiocytosis X of the lung after successfully treated pulmonary metastatic ovarian carcinoma]. 38
11458784 2001
28
Adult pulmonary Langerhans' cell histiocytosis. 38
10770823 2000
29
Streptococcus equinus endocarditis in a patient with pulmonary histiocytosis X. 38
10866592 2000
30
Severe pulmonary hypertension in histiocytosis X. 38
10619823 2000
31
Evidence that Langerhans cells in adult pulmonary Langerhans cell histiocytosis are mature dendritic cells: importance of the cytokine microenvironment. 38
10477625 1999
32
[Pneumothorax secondary to pulmonary histiocytosis X]. 38
10528489 1999
33
Pulmonary histiocytosis X. 38
11155889 1999
34
[Pathogenesis of adult pulmonary Langerhans-cell histiocytosis]. 38
10339758 1999
35
Streptococcus equinus endocarditis in a patient with pulmonary histiocytosis X. 38
10680994 1999
36
Recurrent spontaneous pneumothorax in pulmonary histiocytosis X. 38
9798765 1998
37
Relapsing nodular lesions in the course of adult pulmonary Langerhans cell histiocytosis. 38
9620939 1998
38
Pulmonary histiocytosis X. 38
17344722 1998
39
[Costal lysis caused by pulmonary histiocytosis X]. 38
9577209 1998
40
[An emergency operation in pulmonary histiocytosis X]. 38
9974042 1998
41
Pulmonary vascular involvement in pulmonary histiocytosis X. 38
9404788 1997
42
Group II phospholipase A2 and pulmonary histiocytosis X. 38
9266909 1997
43
Epithelioid haemangioendothelioma of the lung imitating clinical features of pulmonary histiocytosis X. 38
9401364 1997
44
Advanced pulmonary histiocytosis X is associated with severe pulmonary hypertension. 38
9106608 1997
45
Role of granulocyte-macrophage colony stimulating factor (GM-CSF) in the pathogenesis of adult pulmonary histiocytosis X. 38
8693443 1996
46
Pulmonary histiocytosis X: pulmonary function and exercise pathophysiology. 38
8542154 1996
47
[The variable course of pulmonary histiocytosis X]. 38
7610104 1995
48
[Pulmonary histiocytosis X in high resolution CT]. 38
7709288 1995
49
Alveolar macrophages from bronchoalveolar lavage of patients with pulmonary histiocytosis X: determination of phenotypic and functional changes. 38
7616760 1995
50
Nonproductive cough, weight loss and bilateral pulmonary infiltrates. 38
8087117 1994

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 EFO
18 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
Content
Loading form....