MCID: BRN022
MIFTS: 61

Bronchiectasis malady

Respiratory diseases category

Summaries for Bronchiectasis

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Sources:
9Disease Ontology, 66Wikipedia, 48OMIM, 34MalaCards
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Disease Ontology:9 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: Bronchiectasis, also known as polynesian bronchiectasis, is related to cystic fibrosis and aspergillosis. 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, testes and colon, and related mouse phenotypes are respiratory system and immune system.

Wikipedia:66 Bronchiectasis is a disease where there is permanent enlargement of parts of the airways of the lung.... more...

Description from OMIM:48 211400

Aliases & Classifications for Bronchiectasis

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Sources:
9Disease Ontology, 23GTR, 11DISEASES, 46Novoseek, 63UMLS, 59SNOMED-CT, 36MeSH, 41NCIt, 48OMIM, 28ICD9CM
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Classifications:

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


Aliases & Descriptions:

bronchiectasis 9 23 11 46 63
polynesian bronchiectasis 9


External Ids:

Disease Ontology9 DOID:9563
MeSH36 D001987
NCIt41 C84475
OMIM48 211400
ICD9CM28 494

Related Diseases for Bronchiectasis

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Sources:
18GeneCards, 19GeneDecks
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Diseases related to Bronchiectasis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 158)
idRelated DiseaseScoreTop Affiliating Genes
1cystic fibrosis31.2CFTR, ELANE, CTSG, BPI
2aspergillosis30.6ELANE
3asthma30.6CTSG, ELANE, MMP8
4bronchitis30.5CFTR, ELANE
5pneumonia30.3CTSG, CFTR, MMP8, ELANE, BPI
6hypertension30.2SCNN1G, SCNN1B, SCNN1A
7arthritis30.1MMP8, CTSG, TAP2
8vasculitis30.1BPI, ELANE, CTSG
9respiratory failure30.1ELANE, CFTR
10aneurysm30.0MMP8, ELANE
11cholangitis30.0CFTR
12rheumatoid arthritis29.9CFTR, MMP8, ELANE, CTSG, TAP2, BPI
13ulcerative colitis29.8CTSG, BPI
14primary ciliary dyskinesia10.3
15dextrocardia10.3
16allergic bronchopulmonary aspergillosis10.3
17tuberculosis10.3
18sinusitis10.3
19yellow nail syndrome10.3
20common variable immunodeficiency10.2
21bronchiectasis with or without elevated sweat chloride 1, modifier of10.2
22alpha 1-antitrypsin deficiency10.2
23influenza10.2
24ciliary dyskinesia-bronchiectasis10.2
25bronchiectasis with or without elevated sweat chloride 210.2
26situs inversus10.1
27agammaglobulinemia10.1
28amyloidosis10.1
29aspiration pneumonia10.1
30esophagitis10.1
31obstructive lung disease10.1
32thyroiditis10.1
33pulmonary function10.1
34bronchiectasis with or without elevated sweat chloride 310.1
35blindness10.1
36autosomal recessive pseudohypoaldosteronism type 110.1SCNN1A, SCNN1B
37emphysema due to aat deficiency10.1ELANE
38periodontal disease10.1ELANE
39pseudohypoaldosteronism type 110.0SCNN1A, SCNN1G, SCNN1B
40liddle syndrome10.0SCNN1B, SCNN1A, SCNN1G
41alcoholic pancreatitis10.0CFTR, ELANE
42exocrine pancreatic insufficiency10.0CFTR, ELANE
43steatorrhea10.0ELANE, CFTR
44pancreas disease10.0CFTR, ELANE
45keratoconus10.0CTSG, MMP8
46peritonitis10.0CTSG, MMP8
47polycystic kidney disease10.0
48nasopharyngitis10.0
49oligospermia10.0
50crescentic glomerulonephritis10.0

Graphical network of the top 20 diseases related to Bronchiectasis:



Diseases related to bronchiectasis

Symptoms for Bronchiectasis

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48OMIM
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Clinical features from OMIM:

211400

Drugs & Therapeutics for Bronchiectasis

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
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Approved drugs:

Search CenterWatch for Bronchiectasis

Drug clinical trials:

Search ClinicalTrials for Bronchiectasis

Search NIH Clinical Center for Bronchiectasis

Search CenterWatch for Bronchiectasis

Inferred drug relations via UMLS63/NDF-RT42:

Genetic Tests for Bronchiectasis

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Sources:
23GTR
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Genetic tests related to Bronchiectasis:

id Genetic test Affiliating Genes
1 Bronchiectasis23

Anatomical Context for Bronchiectasis

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34MalaCards
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MalaCards organs/tissues related to Bronchiectasis:

34
Lung, Testes, Colon, Thyroid, Kidney, Bone, T cells, Monocytes, Breast, Brain, Smooth muscle, Bone marrow, Lymph node, B cells

Animal Models for Bronchiectasis or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Bronchiectasis:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053888.7CFTR, SCNN1B, SCNN1A, SCNN1G
2MP:00053877.7CTSG, TAP2, ELANE, MMP8, CFTR
3MP:00028737.6CTSG, ELANE, SCNN1G, SCNN1A, SCNN1B, CFTR
4MP:00107687.5CFTR, SCNN1B, SCNN1A, SCNN1G, ELANE, CTSG

Publications for Bronchiectasis

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Sources:
53PubMed
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Articles related to Bronchiectasis:

(show top 50)    (show all 604)
idTitleAuthorsYear
1
Epicardially originating ventricular tachycardia: an unusual presentation of bronchiectasis. (23365070)
2013
2
Validation of a Quality of Life Questionnaire for Bronchiectasis: psychometric analyses of the Spanish QOL-B-V3.0. (24142190)
2013
3
Molecular epidemiological analysis suggests cross infection with Pseudomonas aeruginosa is rare in Non-Cystic Fibrosis bronchiectasis. (24176999)
2013
4
Depression and anxiety symptoms in bronchiectasis: associations with health-related quality of life. (22544417)
2013
5
Surgical treatment of bronchiectasis: a retrospective observational study of 138 patients. (23825752)
2013
6
Lung transplantation for cystic fibrosis and bronchiectasis. (23821505)
2013
7
Indigenous children from three countries with non-cystic fibrosis chronic suppurative lung disease/bronchiectasis. (23401398)
2013
8
Mannose-binding lectin and innate immunity in bronchiectasis. (24429113)
2013
9
Bronchiectasis exacerbation study on azithromycin and amoxycillin-clavulanate for respiratory exacerbations in children (BEST-2): study protocol for a randomized controlled trial. (23421781)
2013
10
Microbes in bronchiectasis: the forest or the trees? (23675713)
2013
11
Quantitative and qualitative computed tomographic characteristics of bronchiectasis in 12 dogs. (23578226)
2013
12
Pulmonary rehabilitation for patients with bronchiectasis: case reports. (22510678)
2012
13
Focal bronchiectasis causing abnormal pulmonary radioiodine uptake in a patient with well-differentiated papillary thyroid carcinoma. (23097725)
2012
14
Bronchiectasis in chronic pulmonary aspiration: risk factors and clinical implications. (22028069)
2012
15
Pathogenesis of bronchiectasis. (22640841)
2012
16
Immune regulation in idiopathic bronchiectasis. (23231716)
2012
17
The role of macrolides in childhood non-cystic fibrosis-related bronchiectasis. (22570510)
2012
18
Imaging of bronchiectasis. (22640843)
2012
19
Inhaled gentamicin in non-cystic fibrosis bronchiectasis: effects of long-term therapy. (21470074)
2011
20
Risk of infections in bronchiectasis during disease-modifying treatment and biologics for rheumatic diseases. (22046967)
2011
21
Infection and remodelling: a 21st century model of bronchiectasis? (21965500)
2011
22
Lady Windermere revisited: treatment with thoracoscopic lobectomy/segmentectomy for right middle lobe and lingular bronchiectasis associated with non-tuberculous mycobacterial disease. (21324708)
2011
23
WITHDRAWN: Bronchopulmonary hygiene physical therapy for chronic obstructive pulmonary disease and bronchiectasis. (21735379)
2011
24
Non-cystic fibrosis bronchiectasis: diagnosis and management in 21st century. (20709772)
2010
25
Pneumatosis intestinalis presenting with a pneumoperitoneum in a patient with chronic bronchiectasis: a delayed diagnosis of superior mesenteric artery ischaemia. (22767661)
2010
26
British Thoracic Society guideline for non-CF bronchiectasis. (20627912)
2010
27
Cardiac herniation following completion pneumonectomy for bronchiectasis. (20826968)
2010
28
A decade of non-cystic fibrosis bronchiectasis 1996-2006. (20666070)
2010
29
Respiratory bacterial pathogens in the nasopharynx and lower airways of Australian indigenous children with bronchiectasis. (20656297)
2010
30
Stable bronchiectasis is associated with low serum L-ficolin concentrations. (20298369)
2009
31
Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening. (19616787)
2009
32
Exhaled breath condensate MMP-9 levels in children with bronchiectasis. (19725099)
2009
33
Bronchiectasis and immune deficiency in an adult patient with deletion 2q37 due to an unbalanced translocation t(2;10). (19272308)
2009
34
Bronchiectasis: pathophysiology, presentation and management. (18837280)
2008
35
Congenital lymphoedema, bronchiectasis and seizure: case report. (18663889)
2008
36
Bronchiectasis, part 2: Management. (20046814)
2008
37
Bronchiectasis in adults: a review. (18824830)
2008
38
Upregulation of ICAM-1 expression in bronchial epithelial cells by airway secretions in bronchiectasis. (17931847)
2008
39
Bronchiectasis and sino-nasal disease: a review. (17592654)
2008
40
Idiopathic CD4+ T-lymphocytopenia with bronchiectasis and hyperimmunoglobulin A. (18779633)
2008
41
Re: A retrospective study of the relationship between tracheal collapse and bronchiectasis in dogs. (18018738)
2007
42
IFN gamma and CXCR-1 gene polymorphisms in idiopathic bronchiectasis. (17026468)
2006
43
Bronchiectasis worsening by p-ANCA (bactericidal/permeability-increasing protein) positive vasculitis. A case report and review of the literature]. (16610259)
2005
44
Neutrophil apoptosis, proinflammatory mediators and cell counts in bronchiectasis. (14985560)
2004
45
CFTR gene mutations in adults with disseminated bronchiectasis. (9272738)
1997
46
Bronchiectasis and homozygous (P1ZZ) alpha 1-antitrypsin deficiency. (8711667)
1996
47
Sputum sol phase proteins and elastase activity in patients with clinically stable bronchiectasis. (1372451)
1992
48
A case of acquired agammaglobulinemia: its association with bronchiectasis. (14020684)
1962
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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Sources:
2BioGPS, 16Gene Expression Omnibus DataSets
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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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Sources:
51PathCards, 56Reactome, 31KEGG, 61Thomson Reuters, 13EMD Millipore, 52PharmGKB, 54QIAGEN, 39NCBI BioSystems Database
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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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Sources:
46Novoseek, 25HMDB, 12DrugBank, 62Tocris Bioscience, 30IUPHAR, 3BitterDB, 52PharmGKB
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Compounds related to Bronchiectasis according to GeneCards/GeneDecks:

(show all 44)
idCompoundScoreTop Affiliating Genes
1triamterene46 25 1211.8SCNN1G, SCNN1A, SCNN1B
2phosphonate469.7CTSG, MMP8
3trypsinogen469.7ELANE, CFTR
4dermatan sulfate469.6CFTR, ELANE
5xanthine46 2510.5CTSG, MMP8
6actinomycin d469.5BPI, SCNN1A, CFTR
7batimastat46 62 1211.5MMP8, ELANE
8polysulfone469.5ELANE, BPI
9amiloride46 30 1211.5SCNN1G, SCNN1A, SCNN1B, CFTR
104-nitroanilide469.5CTSG, ELANE
11elastatinal469.4CTSG, ELANE
12saccharin46 310.4CTSG, ELANE
13thermolysin469.4ELANE, CTSG
14chymostatin469.4ELANE, CTSG
15pmsf469.4CTSG, ELANE
16epsilon aminocaproic acid469.4ELANE, CTSG
17diisopropylfluorophosphate469.4CTSG, ELANE
18phosphoramidon46 1210.3CTSG, ELANE
19cytochalasin b469.3CTSG, ELANE
20urea46 25 1211.3CTSG, BPI, MMP8
21n-formylmethionyl-leucylphenylalanine469.2ELANE, CTSG
22doxycycline46 1210.2ELANE, MMP8
23kininogen469.1ELANE, CTSG
24cyclosporin a46 30 6211.1ELANE, MMP8, CFTR
25aspirin46 52 30 2512.1CTSG, ELANE, CFTR
26pge2469.1ELANE, MMP8, CFTR
27epinephrine46 25 1211.1CTSG, ELANE, CFTR
28aprotinin46 1210.0CTSG, ELANE
29glutamine469.0TAP2, ELANE, CFTR
30(4-aminophenyl)mercuric acetate469.0MMP8, ELANE, CTSG
31hocl469.0CTSG, ELANE, MMP8
32endotoxin468.9CTSG, BPI, ELANE
33fmlp468.9ELANE, BPI, CTSG
34fibrinogen468.9CTSG, ELANE, MMP8
35sodium46 259.8CFTR, SCNN1B, SCNN1A, SCNN1G, CTSG
36h2o2468.6CFTR, MMP8, ELANE, CTSG
37superoxide46 259.6CTSG, ELANE, MMP8
38adenylate468.5TAP2, ELANE, CFTR
39lactate468.5MMP8, ELANE, BPI, CTSG
40alanine468.4CFTR, MMP8, BPI, TAP2, CTSG
41heparin46 30 25 1211.4ELANE, BPI, CTSG
42retinoic acid46 259.1CFTR, MMP8, ELANE, BPI, CTSG
43serine468.1CFTR, MMP8, ELANE, BPI, CTSG
44cysteine467.7CFTR, SCNN1G, MMP8, ELANE, BPI, CTSG

GO Terms for genes affiliated with Bronchiectasis

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17Gene Ontology
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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:0098979.4SCNN1B, SCNN1A, SCNN1G
3apical plasma membraneGO:0163249.2CFTR, SCNN1B, SCNN1A, SCNN1G
4secretory granuleGO:0301419.0CTSG, ELANE
5integral component of plasma membraneGO:0058878.7SCNN1B, SCNN1A, SCNN1G, BPI
6cell surfaceGO:0099868.4CTSG, ELANE, CFTR
7plasma membraneGO:0058867.9CTSG, TAP2, SCNN1G, SCNN1A, SCNN1B, CFTR
8extracellular vesicular exosomeGO:0700627.5SCNN1B, SCNN1A, SCNN1G, ELANE, BPI, CTSG

Biological processes related to Bronchiectasis according to GeneCards/GeneDecks:

(show all 15)
idNameGO IDScoreTop Affiliating Genes
1multicellular organismal water homeostasisGO:0508919.6SCNN1G, SCNN1A, SCNN1B
2sodium ion homeostasisGO:0550789.6SCNN1B, SCNN1A, SCNN1G
3sensory perception of tasteGO:0509099.6SCNN1G, SCNN1A, SCNN1B
4excretionGO:0075889.6SCNN1G, SCNN1A, SCNN1B
5response to stimulusGO:0508969.6SCNN1G, SCNN1A, SCNN1B
6sodium ion transportGO:0068149.5SCNN1B, SCNN1A, SCNN1G
7sodium ion transmembrane transportGO:0357259.5SCNN1G, SCNN1A, SCNN1B
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:0441309.1CTSG, ELANE
12transmembrane transportGO:0550858.8CFTR, SCNN1B, SCNN1A, SCNN1G, TAP2
13extracellular matrix disassemblyGO:0226178.7MMP8, ELANE, CTSG
14proteolysisGO:0065088.5CTSG, ELANE, MMP8
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, SCNN1A, SCNN1B
2WW domain bindingGO:0506999.2SCNN1B, SCNN1A, SCNN1G
3peptidase activityGO:0082339.0CTSG, ELANE
4serine-type endopeptidase activityGO:0042528.3CTSG, ELANE, MMP8

Products for genes affiliated with Bronchiectasis

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  • Antibodies
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Sources for Bronchiectasis

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet