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Bronchiectasis with or without elevated sweat chloride 3(BESC3)

MedGen UID:
414351
•Concept ID:
C2751324
•
Disease or Syndrome
Synonym: BESC3
 
Gene (location): SCNN1G (16p12.2)
 
Monarch Initiative: MONDO:0013112
OMIM®: 613071

Definition

Bronchiectasis with or without elevated sweat chloride-3 (BESC3) is characterized by dilation of the airways arising from chronic bronchial inflammation accompanied by chronic cough, purulent sputum, and recurrent lower respiratory tract infections. Most patients show some degree of airflow obstruction (Fajac et al., 2008). [from OMIM]

Clinical features

From HPO
Bronchiectasis
MedGen UID:
14234
•Concept ID:
C0006267
•
Disease or Syndrome
Persistent abnormal dilatation of the bronchi owing to localized and irreversible destruction and widening of the large airways.
See: Feature record | Search on this feature
Chronic bronchitis
MedGen UID:
3084
•Concept ID:
C0008677
•
Disease or Syndrome
Chronic inflammation of the bronchi.
See: Feature record | Search on this feature

Professional guidelines

PubMed

The Epidemiology and Pathogenesis and Treatment of Pseudomonas aeruginosa Infections: An Update.
Reynolds D, Kollef M
Drugs 2021 Dec;81(18):2117-2131. Epub 2021 Nov 7 doi: 10.1007/s40265-021-01635-6. PMID: 34743315Free PMC Article
Rheumatoid arthritis-interstitial lung disease: manifestations and current concepts in pathogenesis and management.
Kadura S, Raghu G
Eur Respir Rev 2021 Jun 30;30(160) Epub 2021 Jun 23 doi: 10.1183/16000617.0011-2021. PMID: 34168062Free PMC Article
Bronchiectasis in children: diagnosis and treatment.
Chang AB, Bush A, Grimwood K
Lancet 2018 Sep 8;392(10150):866-879. doi: 10.1016/S0140-6736(18)31554-X. PMID: 30215382

Recent clinical studies

Etiology

Bronchiectasis.
Chalmers JD, Chang AB, Chotirmall SH, Dhar R, McShane PJ
Nat Rev Dis Primers 2018 Nov 15;4(1):45. doi: 10.1038/s41572-018-0042-3. PMID: 30442957
Advances in bronchiectasis: endotyping, genetics, microbiome, and disease heterogeneity.
Flume PA, Chalmers JD, Olivier KN
Lancet 2018 Sep 8;392(10150):880-890. doi: 10.1016/S0140-6736(18)31767-7. PMID: 30215383Free PMC Article
Bronchiectasis in children: diagnosis and treatment.
Chang AB, Bush A, Grimwood K
Lancet 2018 Sep 8;392(10150):866-879. doi: 10.1016/S0140-6736(18)31554-X. PMID: 30215382
Management of bronchiectasis in adults.
Visser SK, Bye P, Morgan L
Med J Aust 2018 Aug 20;209(4):177-183. doi: 10.5694/mja17.01195. PMID: 30107772
European Respiratory Society guidelines for the management of adult bronchiectasis.
Polverino E, Goeminne PC, McDonnell MJ, Aliberti S, Marshall SE, Loebinger MR, Murris M, Cantón R, Torres A, Dimakou K, De Soyza A, Hill AT, Haworth CS, Vendrell M, Ringshausen FC, Subotic D, Wilson R, Vilaró J, Stallberg B, Welte T, Rohde G, Blasi F, Elborn S, Almagro M, Timothy A, Ruddy T, Tonia T, Rigau D, Chalmers JD
Eur Respir J 2017 Sep;50(3) Epub 2017 Sep 9 doi: 10.1183/13993003.00629-2017. PMID: 28889110

Diagnosis

Diagnosis and management of non-cystic fibrosis bronchiectasis.
Macfarlane L, Kumar K, Scoones T, Jones A, Loebinger MR, Lord R
Clin Med (Lond) 2021 Nov;21(6):e571-e577. doi: 10.7861/clinmed.2021-0651. PMID: 34862215Free PMC Article
Bronchiectasis.
Chalmers JD, Chang AB, Chotirmall SH, Dhar R, McShane PJ
Nat Rev Dis Primers 2018 Nov 15;4(1):45. doi: 10.1038/s41572-018-0042-3. PMID: 30442957
Bronchiectasis in children: diagnosis and treatment.
Chang AB, Bush A, Grimwood K
Lancet 2018 Sep 8;392(10150):866-879. doi: 10.1016/S0140-6736(18)31554-X. PMID: 30215382
Management of bronchiectasis in adults.
Visser SK, Bye P, Morgan L
Med J Aust 2018 Aug 20;209(4):177-183. doi: 10.5694/mja17.01195. PMID: 30107772
Bronchiectasis update.
O'Donnell AE
Curr Opin Infect Dis 2018 Apr;31(2):194-198. doi: 10.1097/QCO.0000000000000445. PMID: 29489526

Therapy

Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes.
Barry PJ, Mall MA, Álvarez A, Colombo C, de Winter-de Groot KM, Fajac I, McBennett KA, McKone EF, Ramsey BW, Sutharsan S, Taylor-Cousar JL, Tullis E, Ahluwalia N, Jun LS, Moskowitz SM, Prieto-Centurion V, Tian S, Waltz D, Xuan F, Zhang Y, Rowe SM, Polineni D; VX18-445-104 Study Group
N Engl J Med 2021 Aug 26;385(9):815-825. doi: 10.1056/NEJMoa2100665. PMID: 34437784Free PMC Article
Bronchiectasis.
Chalmers JD, Chang AB, Chotirmall SH, Dhar R, McShane PJ
Nat Rev Dis Primers 2018 Nov 15;4(1):45. doi: 10.1038/s41572-018-0042-3. PMID: 30442957
Bronchiectasis in children: diagnosis and treatment.
Chang AB, Bush A, Grimwood K
Lancet 2018 Sep 8;392(10150):866-879. doi: 10.1016/S0140-6736(18)31554-X. PMID: 30215382
Management of bronchiectasis in adults.
Visser SK, Bye P, Morgan L
Med J Aust 2018 Aug 20;209(4):177-183. doi: 10.5694/mja17.01195. PMID: 30107772
European Respiratory Society guidelines for the management of adult bronchiectasis.
Polverino E, Goeminne PC, McDonnell MJ, Aliberti S, Marshall SE, Loebinger MR, Murris M, Cantón R, Torres A, Dimakou K, De Soyza A, Hill AT, Haworth CS, Vendrell M, Ringshausen FC, Subotic D, Wilson R, Vilaró J, Stallberg B, Welte T, Rohde G, Blasi F, Elborn S, Almagro M, Timothy A, Ruddy T, Tonia T, Rigau D, Chalmers JD
Eur Respir J 2017 Sep;50(3) Epub 2017 Sep 9 doi: 10.1183/13993003.00629-2017. PMID: 28889110

Prognosis

Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes.
Barry PJ, Mall MA, Álvarez A, Colombo C, de Winter-de Groot KM, Fajac I, McBennett KA, McKone EF, Ramsey BW, Sutharsan S, Taylor-Cousar JL, Tullis E, Ahluwalia N, Jun LS, Moskowitz SM, Prieto-Centurion V, Tian S, Waltz D, Xuan F, Zhang Y, Rowe SM, Polineni D; VX18-445-104 Study Group
N Engl J Med 2021 Aug 26;385(9):815-825. doi: 10.1056/NEJMoa2100665. PMID: 34437784Free PMC Article
Bronchiectasis.
McShane PJ, Tino G
Chest 2019 Apr;155(4):825-833. Epub 2018 Nov 4 doi: 10.1016/j.chest.2018.10.027. PMID: 30403962
Geographic variation in the aetiology, epidemiology and microbiology of bronchiectasis.
Chandrasekaran R, Mac Aogáin M, Chalmers JD, Elborn SJ, Chotirmall SH
BMC Pulm Med 2018 May 22;18(1):83. doi: 10.1186/s12890-018-0638-0. PMID: 29788932Free PMC Article
Cough and bronchiectasis.
McCallion P, De Soyza A
Pulm Pharmacol Ther 2017 Dec;47:77-83. Epub 2017 Jun 7 doi: 10.1016/j.pupt.2017.04.010. PMID: 28602999
The bronchiectasis severity index. An international derivation and validation study.
Chalmers JD, Goeminne P, Aliberti S, McDonnell MJ, Lonni S, Davidson J, Poppelwell L, Salih W, Pesci A, Dupont LJ, Fardon TC, De Soyza A, Hill AT
Am J Respir Crit Care Med 2014 Mar 1;189(5):576-85. doi: 10.1164/rccm.201309-1575OC. PMID: 24328736Free PMC Article

Clinical prediction guides

A Double-Blind Randomized Placebo-Controlled Phase 3 Trial of Tobramycin Inhalation Solution in Adults With Bronchiectasis With Pseudomonas aeruginosa Infection.
Guan WJ, Xu JF, Luo H, Xu XX, Song YL, Ma WL, Liang ZA, Liu XD, Zhang GJ, Zhang XJ, Li RK, Zhu SY, Zhang YJ, Cai XJ, Wei LP, Tian DB, Zhao H, Chen PY, Qu JM, Zhong NS; TORNASOL Study Group
Chest 2023 Jan;163(1):64-76. Epub 2022 Jul 19 doi: 10.1016/j.chest.2022.07.007. PMID: 35863486
Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes.
Barry PJ, Mall MA, Álvarez A, Colombo C, de Winter-de Groot KM, Fajac I, McBennett KA, McKone EF, Ramsey BW, Sutharsan S, Taylor-Cousar JL, Tullis E, Ahluwalia N, Jun LS, Moskowitz SM, Prieto-Centurion V, Tian S, Waltz D, Xuan F, Zhang Y, Rowe SM, Polineni D; VX18-445-104 Study Group
N Engl J Med 2021 Aug 26;385(9):815-825. doi: 10.1056/NEJMoa2100665. PMID: 34437784Free PMC Article
Exercise training for bronchiectasis.
Lee AL, Gordon CS, Osadnik CR
Cochrane Database Syst Rev 2021 Apr 6;4(4):CD013110. doi: 10.1002/14651858.CD013110.pub2. PMID: 33822364Free PMC Article
Advances in bronchiectasis: endotyping, genetics, microbiome, and disease heterogeneity.
Flume PA, Chalmers JD, Olivier KN
Lancet 2018 Sep 8;392(10150):880-890. doi: 10.1016/S0140-6736(18)31767-7. PMID: 30215383Free PMC Article
European Respiratory Society guidelines for the management of adult bronchiectasis.
Polverino E, Goeminne PC, McDonnell MJ, Aliberti S, Marshall SE, Loebinger MR, Murris M, Cantón R, Torres A, Dimakou K, De Soyza A, Hill AT, Haworth CS, Vendrell M, Ringshausen FC, Subotic D, Wilson R, Vilaró J, Stallberg B, Welte T, Rohde G, Blasi F, Elborn S, Almagro M, Timothy A, Ruddy T, Tonia T, Rigau D, Chalmers JD
Eur Respir J 2017 Sep;50(3) Epub 2017 Sep 9 doi: 10.1183/13993003.00629-2017. PMID: 28889110

Recent systematic reviews

The effectiveness of the active cycle of breathing technique in patients with chronic respiratory diseases: A systematic review.
Zisi D, Chryssanthopoulos C, Nanas S, Philippou A
Heart Lung 2022 May-Jun;53:89-98. Epub 2022 Feb 27 doi: 10.1016/j.hrtlng.2022.02.006. PMID: 35235877
Telerehabilitation for chronic respiratory disease.
Cox NS, Dal Corso S, Hansen H, McDonald CF, Hill CJ, Zanaboni P, Alison JA, O'Halloran P, Macdonald H, Holland AE
Cochrane Database Syst Rev 2021 Jan 29;1(1):CD013040. doi: 10.1002/14651858.CD013040.pub2. PMID: 33511633Free PMC Article
Bronchial artery embolization in hemoptysis: a systematic review.
Panda A, Bhalla AS, Goyal A
Diagn Interv Radiol 2017 Jul-Aug;23(4):307-317. doi: 10.5152/dir.2017.16454. PMID: 28703105Free PMC Article
Airway clearance techniques for bronchiectasis.
Lee AL, Burge AT, Holland AE
Cochrane Database Syst Rev 2015 Nov 23;2015(11):CD008351. doi: 10.1002/14651858.CD008351.pub3. PMID: 26591003Free PMC Article
Bronchiectasis.
Magis-Escurra C, Reijers MH
BMJ Clin Evid 2015 Feb 25;2015 PMID: 25715965Free PMC Article

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