MCID: BRN022
MIFTS: 62

Bronchiectasis malady

Respiratory diseases category
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Summaries for Bronchiectasis

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Disease Ontology:8 A bronchial disease that is a chronic inflammatory condition of one or more bronchi or bronchioles marked by dilatation and loss of elasticity of the walls resulting from damage to the airway wall leading to the formation of small sacs on the bronchial wall and impairment of cilia mobility in the lung. inflammation of the bronchial wall increases mucus secretion which serves as a breeding ground for bacteria. bronchiectasis is caused by repeated respiratory infections, immune deficiency disorders, hereditary disorders (cystic fibrosis or primary ciliary dyskinesia), mechanical factors (inhaled object or a lung tumor) or inhaling toxic substances.

MalaCards based summary: Bronchiectasis, also known as polynesian bronchiectasis, is related to aspergillosis and cystic fibrosis. An important gene associated with Bronchiectasis is CFTR (cystic fibrosis transmembrane conductance regulator (ATP-binding cassette sub-family C, member 7)), and among its related pathways are ABC-family proteins mediated transport and ER to Golgi Transport. The drug acetylcysteine and the compounds triamterene and phosphonate have been mentioned in the context of this disorder. Affiliated tissues include lung, neutrophil and testes, and related mouse phenotypes are respiratory system and immune system.

Wikipedia:65 Bronchiectasis is a disease in which there is permanent enlargement of parts of the airways of the lung.... more...

Description from OMIM:46 211400

Aliases & Classifications for Bronchiectasis

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Sources:
8Disease Ontology, 22GTR, 10DISEASES, 44Novoseek, 62UMLS, 39NCIt, 57SNOMED-CT, 34MeSH, 46OMIM, 27ICD9CM
See all sources

Bronchiectasis, Aliases & Descriptions:

Name: Bronchiectasis 8 22 10 44 62
 
Polynesian Bronchiectasis 8 62


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Respiratory diseases


External Ids:

Disease Ontology8 DOID:9563
NCIt39 C84475
MeSH34 D001987
OMIM46 211400
ICD9CM27 494

Related Diseases for Bronchiectasis

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Diseases related to Bronchiectasis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 170)
idRelated DiseaseScoreTop Affiliating Genes
1aspergillosis30.9ELANE
2cystic fibrosis30.8CFTR, ELANE, CTSG, BPI
3bronchitis30.6CFTR, ELANE
4cholangitis30.6CFTR
5hypertension30.4SCNN1G, SCNN1B, SCNN1A
6asthma30.2CTSG, ELANE, MMP8
7respiratory failure30.2ELANE, CFTR
8chronic obstructive pulmonary disease30.1ELANE, MMP8, CFTR, CTSG
9arthritis30.1MMP8, CTSG, TAP2
10aneurysm30.0MMP8, ELANE
11ulcerative colitis29.9CTSG, BPI
12vasculitis29.8BPI, ELANE, CTSG
13pneumonia29.7CTSG, CFTR, MMP8, ELANE, BPI
14rheumatoid arthritis29.1CFTR, MMP8, ELANE, CTSG, TAP2, BPI
15primary ciliary dyskinesia10.4
16pseudohypoaldosteronism type 110.3SCNN1A, SCNN1B
17sinusitis10.3
18allergic bronchopulmonary aspergillosis10.3
19dextrocardia10.3
20tuberculosis10.3
21bronchiectasis with or without elevated sweat chloride 210.3
22emphysema due to aat deficiency10.3ELANE
23common variable immunodeficiency10.3
24yellow nail syndrome10.3
25periodontal disease10.2ELANE
26alpha 1-antitrypsin deficiency10.2
27influenza10.2
28ciliary dyskinesia-bronchiectasis10.2
29pulmonary function10.2
30liddle syndrome10.2SCNN1B, SCNN1A, SCNN1G
31alcoholic pancreatitis10.1CFTR, ELANE
32exocrine pancreatic insufficiency10.1CFTR, ELANE
33steatorrhea10.1ELANE, CFTR
34pancreas disease10.1CFTR, ELANE
35keratoconus10.1CTSG, MMP8
36situs inversus10.1
37agammaglobulinemia10.1
38amyloidosis10.1
39aspiration pneumonia10.1
40esophagitis10.1
41obstructive lung disease10.1
42thyroiditis10.1
43bronchiectasis oligospermia10.1
44haemophilus influenzae10.1
45bronchiectasis with or without elevated sweat chloride 310.1
46williams-campbell syndrome10.1
47peritonitis10.1CTSG, MMP8
48chorioamnionitis10.1ELANE, MMP8
49abdominal aortic aneurysm10.1MMP8, ELANE
50pseudohypoaldosteronism10.0SCNN1B, SCNN1G, SCNN1A, CFTR

Graphical network of the top 20 diseases related to Bronchiectasis:



Diseases related to bronchiectasis

Symptoms for Bronchiectasis

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Clinical features from OMIM:

211400

Drugs & Therapeutics for Bronchiectasis

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Drug clinical trials:

Search ClinicalTrials for Bronchiectasis

Search NIH Clinical Center for Bronchiectasis

Inferred drug relations via UMLS62/NDF-RT40:

Genetic Tests for Bronchiectasis

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Genetic tests related to Bronchiectasis:

id Genetic test Affiliating Genes
1 Bronchiectasis22

Anatomical Context for Bronchiectasis

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MalaCards organs/tissues related to Bronchiectasis:

32
Lung, Neutrophil, Testes, Colon, Thyroid, Bone, Kidney, Bone marrow, Lymph node, Brain, Smooth muscle, Breast, Myeloid, Monocytes, T cells, B cells

Animal Models for Bronchiectasis or affiliated genes

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MGI Mouse Phenotypes related to Bronchiectasis:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053888.7SCNN1A, SCNN1G, CFTR, SCNN1B
2MP:00053877.7CTSG, TAP2, ELANE, MMP8, CFTR
3MP:00028737.6CTSG, CFTR, SCNN1B, SCNN1A, SCNN1G, ELANE
4MP:00107687.5CTSG, SCNN1G, ELANE, SCNN1A, CFTR, SCNN1B

Publications for Bronchiectasis

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Articles related to Bronchiectasis:

(show top 50)    (show all 634)
idTitleAuthorsYear
1
Impact of Different Etiologies of Bronchiectasis on the Pulmonary Function Tests. (25380609)
2014
2
The prevalence of bronchiectasis in patients with Marfan syndrome. (25199019)
2014
3
Expression of soluble triggering receptor expressed on myeloid cells-1 in childhood CF and Non-CF bronchiectasis. (25348906)
2014
4
Quality of Life Questionnaire-Bronchiectasis: final psychometric analyses and determination of minimal important difference scores. (25323621)
2014
5
Epicardially originating ventricular tachycardia: an unusual presentation of bronchiectasis. (23365070)
2013
6
Validation of a Quality of Life Questionnaire for Bronchiectasis: psychometric analyses of the Spanish QOL-B-V3.0. (24142190)
2013
7
Molecular epidemiological analysis suggests cross infection with Pseudomonas aeruginosa is rare in Non-Cystic Fibrosis bronchiectasis. (24176999)
2013
8
Depression and anxiety symptoms in bronchiectasis: associations with health-related quality of life. (22544417)
2013
9
Surgical treatment of bronchiectasis: a retrospective observational study of 138 patients. (23825752)
2013
10
Lung transplantation for cystic fibrosis and bronchiectasis. (23821505)
2013
11
Indigenous children from three countries with non-cystic fibrosis chronic suppurative lung disease/bronchiectasis. (23401398)
2013
12
Mannose-binding lectin and innate immunity in bronchiectasis. (24429113)
2013
13
Bronchiectasis exacerbation study on azithromycin and amoxycillin-clavulanate for respiratory exacerbations in children (BEST-2): study protocol for a randomized controlled trial. (23421781)
2013
14
Microbes in bronchiectasis: the forest or the trees? (23675713)
2013
15
Quantitative and qualitative computed tomographic characteristics of bronchiectasis in 12 dogs. (23578226)
2013
16
Pulmonary rehabilitation for patients with bronchiectasis: case reports. (22510678)
2012
17
Focal bronchiectasis causing abnormal pulmonary radioiodine uptake in a patient with well-differentiated papillary thyroid carcinoma. (23097725)
2012
18
Bronchiectasis in chronic pulmonary aspiration: risk factors and clinical implications. (22028069)
2012
19
Immune regulation in idiopathic bronchiectasis. (23231716)
2012
20
Inhaled gentamicin in non-cystic fibrosis bronchiectasis: effects of long-term therapy. (21470074)
2011
21
Risk of infections in bronchiectasis during disease-modifying treatment and biologics for rheumatic diseases. (22046967)
2011
22
Infection and remodelling: a 21st century model of bronchiectasis? (21965500)
2011
23
Lady Windermere revisited: treatment with thoracoscopic lobectomy/segmentectomy for right middle lobe and lingular bronchiectasis associated with non-tuberculous mycobacterial disease. (21324708)
2011
24
WITHDRAWN: Bronchopulmonary hygiene physical therapy for chronic obstructive pulmonary disease and bronchiectasis. (21735379)
2011
25
Non-cystic fibrosis bronchiectasis: diagnosis and management in 21st century. (20709772)
2010
26
Pneumatosis intestinalis presenting with a pneumoperitoneum in a patient with chronic bronchiectasis: a delayed diagnosis of superior mesenteric artery ischaemia. (22767661)
2010
27
British Thoracic Society guideline for non-CF bronchiectasis. (20627912)
2010
28
Cardiac herniation following completion pneumonectomy for bronchiectasis. (20826968)
2010
29
Chronic suppurative lung disease and bronchiectasis in children and adults in Australia and New Zealand. (20854242)
2010
30
A decade of non-cystic fibrosis bronchiectasis 1996-2006. (20666070)
2010
31
Respiratory bacterial pathogens in the nasopharynx and lower airways of Australian indigenous children with bronchiectasis. (20656297)
2010
32
Stable bronchiectasis is associated with low serum L-ficolin concentrations. (20298369)
2009
33
Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening. (19616787)
2009
34
Exhaled breath condensate MMP-9 levels in children with bronchiectasis. (19725099)
2009
35
Bronchiectasis and immune deficiency in an adult patient with deletion 2q37 due to an unbalanced translocation t(2;10). (19272308)
2009
36
Bronchiectasis: pathophysiology, presentation and management. (18837280)
2008
37
Congenital lymphoedema, bronchiectasis and seizure: case report. (18663889)
2008
38
Bronchiectasis, part 2: Management. (20046814)
2008
39
Bronchiectasis in adults: a review. (18824830)
2008
40
Upregulation of ICAM-1 expression in bronchial epithelial cells by airway secretions in bronchiectasis. (17931847)
2008
41
Bronchiectasis and sino-nasal disease: a review. (17592654)
2008
42
Re: A retrospective study of the relationship between tracheal collapse and bronchiectasis in dogs. (18018738)
2007
43
IFN gamma and CXCR-1 gene polymorphisms in idiopathic bronchiectasis. (17026468)
2006
44
Bronchiectasis worsening by p-ANCA (bactericidal/permeability-increasing protein) positive vasculitis. A case report and review of the literature]. (16610259)
2005
45
Neutrophil apoptosis, proinflammatory mediators and cell counts in bronchiectasis. (14985560)
2004
46
CFTR gene mutations in adults with disseminated bronchiectasis. (9272738)
1997
47
Bronchiectasis and homozygous (P1ZZ) alpha 1-antitrypsin deficiency. (8711667)
1996
48
Sputum sol phase proteins and elastase activity in patients with clinically stable bronchiectasis. (1372451)
1992
49
Simulated dextrocardia associated with bronchiectasis; report of case. (20280493)
1946
50
Dextrocardia and bronchiectasis, a review of the literature and a report of two cases. (20996259)
1946

Variations for Bronchiectasis

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Expression for genes affiliated with Bronchiectasis

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Expression patterns in normal tissues for genes affiliated with Bronchiectasis

Search GEO for disease gene expression data for Bronchiectasis.

Pathways for genes affiliated with Bronchiectasis

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Pathways related to Bronchiectasis according to GeneCards/GeneDecks:

(show all 15)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.6TAP2, CFTR
2
Show member pathways
9.6TAP2, CFTR
39.3SCNN1B, SCNN1A, SCNN1G
4
Show member pathways
9.3SCNN1B, SCNN1G, SCNN1A
5
Show member pathways
9.3SCNN1B, SCNN1A, SCNN1G
6
Show member pathways
9.3SCNN1A, SCNN1G, SCNN1B
79.3SCNN1G, SCNN1A, SCNN1B
8
Show member pathways
9.3SCNN1G, SCNN1A, SCNN1B
99.0SCNN1B, SCNN1A, SCNN1G, CFTR
109.0SCNN1G, SCNN1A, SCNN1B, CFTR
11
Show member pathways
9.0SCNN1G, SCNN1A, SCNN1B, CFTR
12
Show member pathways
9.0CFTR, SCNN1B, SCNN1A, SCNN1G
13
Show member pathways
8.6MMP8, ELANE, CTSG
148.6MMP8, CTSG, ELANE
15
Show member pathways
8.6MMP8, CTSG, ELANE

Compounds for genes affiliated with Bronchiectasis

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Compounds related to Bronchiectasis according to GeneCards/GeneDecks:

(show all 44)
idCompoundScoreTop Affiliating Genes
1triamterene44 24 1111.8SCNN1G, SCNN1A, SCNN1B
2phosphonate449.7CTSG, MMP8
3trypsinogen449.7ELANE, CFTR
4dermatan sulfate449.6CFTR, ELANE
5xanthine44 2410.5CTSG, MMP8
6actinomycin d449.5BPI, SCNN1A, CFTR
7batimastat44 61 1111.5MMP8, ELANE
8polysulfone449.5ELANE, BPI
9amiloride44 28 1111.5SCNN1G, SCNN1A, SCNN1B, CFTR
104-nitroanilide449.5CTSG, ELANE
11elastatinal449.4CTSG, ELANE
12saccharin44 210.4CTSG, ELANE
13thermolysin449.4ELANE, CTSG
14chymostatin449.4ELANE, CTSG
15pmsf449.4CTSG, ELANE
16epsilon aminocaproic acid449.4ELANE, CTSG
17diisopropylfluorophosphate449.4CTSG, ELANE
18phosphoramidon44 1110.3CTSG, ELANE
19cytochalasin b449.3CTSG, ELANE
20urea44 24 1111.3CTSG, BPI, MMP8
21n-formylmethionyl-leucylphenylalanine449.2ELANE, CTSG
22doxycycline44 1110.2ELANE, MMP8
23kininogen449.1ELANE, CTSG
24cyclosporin a44 28 6111.1ELANE, MMP8, CFTR
25aspirin44 50 28 2412.1CTSG, ELANE, CFTR
26pge2449.1ELANE, MMP8, CFTR
27epinephrine44 24 1111.1CTSG, ELANE, CFTR
28aprotinin44 1110.0CTSG, ELANE
29glutamine449.0TAP2, ELANE, CFTR
30(4-aminophenyl)mercuric acetate449.0MMP8, ELANE, CTSG
31hocl449.0CTSG, ELANE, MMP8
32endotoxin448.9CTSG, BPI, ELANE
33fmlp448.9ELANE, BPI, CTSG
34fibrinogen448.9CTSG, ELANE, MMP8
35sodium44 249.8CFTR, SCNN1B, SCNN1A, SCNN1G, CTSG
36h2o2448.6CFTR, MMP8, ELANE, CTSG
37superoxide44 249.6CTSG, ELANE, MMP8
38adenylate448.5TAP2, ELANE, CFTR
39lactate448.5MMP8, ELANE, BPI, CTSG
40alanine448.4CFTR, MMP8, BPI, TAP2, CTSG
41heparin44 28 24 1111.4ELANE, BPI, CTSG
42retinoic acid44 249.1CFTR, MMP8, ELANE, BPI, CTSG
43serine448.1CFTR, MMP8, ELANE, BPI, CTSG
44cysteine447.7CFTR, SCNN1G, MMP8, ELANE, BPI, CTSG

GO Terms for genes affiliated with Bronchiectasis

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Cellular components related to Bronchiectasis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1sodium channel complexGO:0347069.5SCNN1G, SCNN1A, SCNN1B
2external side of plasma membraneGO:0098979.4SCNN1G, SCNN1A, SCNN1B
3apical plasma membraneGO:0163249.2SCNN1A, SCNN1B, CFTR, SCNN1G
4secretory granuleGO:0301419.0ELANE, CTSG
5integral component of plasma membraneGO:0058878.7BPI, SCNN1G, SCNN1A, SCNN1B
6cell surfaceGO:0099868.4CTSG, ELANE, CFTR
7plasma membraneGO:0058867.9CTSG, TAP2, SCNN1G, SCNN1A, SCNN1B, CFTR
8extracellular vesicular exosomeGO:0700627.5CTSG, BPI, ELANE, SCNN1G, SCNN1A, SCNN1B

Biological processes related to Bronchiectasis according to GeneCards/GeneDecks:

(show all 15)
idNameGO IDScoreTop Affiliating Genes
1multicellular organismal water homeostasisGO:0508919.6SCNN1A, SCNN1B, SCNN1G
2sodium ion homeostasisGO:0550789.6SCNN1B, SCNN1A, SCNN1G
3sensory perception of tasteGO:0509099.6SCNN1B, SCNN1G, SCNN1A
4excretionGO:0075889.6SCNN1G, SCNN1A, SCNN1B
5response to stimulusGO:0508969.6SCNN1B, SCNN1A, SCNN1G
6sodium ion transportGO:0068149.5SCNN1A, SCNN1G, SCNN1B
7sodium ion transmembrane transportGO:0357259.5SCNN1G, SCNN1B, SCNN1A
8ion transmembrane transportGO:0342209.3SCNN1B, SCNN1A, SCNN1G
9collagen catabolic processGO:0305749.2ELANE, MMP8
10positive regulation of immune responseGO:0507789.2CTSG, ELANE
11negative regulation of growth of symbiont in hostGO:0441309.1ELANE, CTSG
12transmembrane transportGO:0550858.8TAP2, SCNN1G, SCNN1A, CFTR, SCNN1B
13extracellular matrix disassemblyGO:0226178.7MMP8, CTSG, ELANE
14proteolysisGO:0065088.5MMP8, CTSG, ELANE
15extracellular matrix organizationGO:0301988.3CTSG, ELANE, MMP8

Molecular functions related to Bronchiectasis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1ligand-gated sodium channel activityGO:0152809.3SCNN1G, SCNN1B, SCNN1A
2WW domain bindingGO:0506999.2SCNN1A, SCNN1G, SCNN1B
3peptidase activityGO:0082339.0ELANE, CTSG
4serine-type endopeptidase activityGO:0042528.3MMP8, ELANE, CTSG

Products for genes affiliated with Bronchiectasis

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Sources for Bronchiectasis

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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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet