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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.
Chronic bronchitis
MedGen UID:
3084
Concept ID:
C0008677
Disease or Syndrome
Chronic inflammation of the bronchi.

Professional guidelines

PubMed

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
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
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

Metersky ML, Barker AF
Clin Chest Med 2022 Mar;43(1):35-46. doi: 10.1016/j.ccm.2021.11.003. PMID: 35236559
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
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
Chang AB, Bush A, Grimwood K
Lancet 2018 Sep 8;392(10150):866-879. doi: 10.1016/S0140-6736(18)31554-X. PMID: 30215382
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

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
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
Chang AB, Bush A, Grimwood K
Lancet 2018 Sep 8;392(10150):866-879. doi: 10.1016/S0140-6736(18)31554-X. PMID: 30215382
Visser SK, Bye P, Morgan L
Med J Aust 2018 Aug 20;209(4):177-183. doi: 10.5694/mja17.01195. PMID: 30107772
O'Donnell AE
Curr Opin Infect Dis 2018 Apr;31(2):194-198. doi: 10.1097/QCO.0000000000000445. PMID: 29489526

Therapy

O'Neill K, O'Donnell AE, Bradley JM
Respirology 2019 Mar;24(3):227-237. Epub 2019 Jan 16 doi: 10.1111/resp.13459. PMID: 30650472
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
Chang AB, Bush A, Grimwood K
Lancet 2018 Sep 8;392(10150):866-879. doi: 10.1016/S0140-6736(18)31554-X. PMID: 30215382
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
Magis-Escurra C, Reijers MH
BMJ Clin Evid 2015 Feb 25;2015 PMID: 25715965Free PMC Article

Prognosis

Barbosa M, Chalmers JD
Presse Med 2023 Sep;52(3):104174. Epub 2023 Sep 30 doi: 10.1016/j.lpm.2023.104174. PMID: 37778637
Martins M, Keir HR, Chalmers JD
Pulmonology 2023 Nov-Dec;29(6):505-517. Epub 2023 Apr 6 doi: 10.1016/j.pulmoe.2023.03.004. PMID: 37030997
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
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
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

Clinical prediction guides

Martins M, Keir HR, Chalmers JD
Pulmonology 2023 Nov-Dec;29(6):505-517. Epub 2023 Apr 6 doi: 10.1016/j.pulmoe.2023.03.004. PMID: 37030997
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
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
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
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

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
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
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
Bohannon RW, Crouch R
J Eval Clin Pract 2017 Apr;23(2):377-381. Epub 2016 Sep 4 doi: 10.1111/jep.12629. PMID: 27592691
Magis-Escurra C, Reijers MH
BMJ Clin Evid 2015 Feb 25;2015 PMID: 25715965Free PMC Article

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