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Cystic Fibrosis Pulmonary Exacerbation

MedGen UID:
888330
Concept ID:
C1135342
Disease or Syndrome

Definition

An acute episode of worsening pulmonary symptoms related to cystic fibrosis. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCystic Fibrosis Pulmonary Exacerbation

Professional guidelines

PubMed

Gavioli EM, Guardado N, Haniff F, Deiab N, Vider E
Microb Drug Resist 2021 Dec;27(12):1726-1732. Epub 2021 Jun 1 doi: 10.1089/mdr.2020.0602. PMID: 34077286
Cogen JD, Faino AV, Onchiri F, Gibson RL, Hoffman LR, Kronman MP, Rosenfeld M, Nichols DP
Ann Am Thorac Soc 2021 Feb;18(2):266-272. doi: 10.1513/AnnalsATS.202002-176OC. PMID: 32810412Free PMC Article
Parkins MD, Elborn JS
J Antimicrob Chemother 2010 Sep;65(9):1853-61. Epub 2010 Jul 6 doi: 10.1093/jac/dkq245. PMID: 20605846

Recent clinical studies

Etiology

Waters V, Shaw M, Perrem L, Quon BS, Tullis E, Solomon M, Rayment JH, Lavoie A, Tse SM, Daigneault P, Bilodeau L, Price A, Nicholson M, Chin M, Parkins M, McKinney ML, Tam JS, Stanojevic S, Grasemann H, Ratjen F; (PIPE Study Investigators)
Eur Respir J 2024 Jun;63(6) Epub 2024 Jun 6 doi: 10.1183/13993003.02278-2023. PMID: 38697648
Wojsyk-Banaszak I, Więckowska B, Stachowiak Z, Kycler M, Szczepankiewicz A
Pediatr Pulmonol 2022 Jun;57(6):1466-1474. Epub 2022 Apr 8 doi: 10.1002/ppul.25891. PMID: 35293155
Cogen JD, Faino AV, Onchiri F, Gibson RL, Hoffman LR, Kronman MP, Rosenfeld M, Nichols DP
Ann Am Thorac Soc 2021 Feb;18(2):266-272. doi: 10.1513/AnnalsATS.202002-176OC. PMID: 32810412Free PMC Article
Laguna TA, Reilly CS, Williams CB, Welchlin C, Wendt CH
Pediatr Pulmonol 2015 Sep;50(9):869-77. Epub 2015 Jun 26 doi: 10.1002/ppul.23225. PMID: 26115542Free PMC Article
Briggs EC, Nguyen T, Wall MA, MacDonald KD
Clin Respir J 2012 Jan;6(1):56-64. Epub 2011 Aug 9 doi: 10.1111/j.1752-699X.2011.00246.x. PMID: 21595857Free PMC Article

Diagnosis

Hassanzad M, Kiani A, Abedini A, Ghaffaripour H, Emami H, Alizadeh N, Zoghi G, Hashemi S, Velayati AA
BMC Pulm Med 2021 Nov 8;21(1):353. doi: 10.1186/s12890-021-01728-8. PMID: 34743707Free PMC Article
Goss CH, Heltshe SL, West NE, Skalland M, Sanders DB, Jain R, Barto TL, Fogarty B, Marshall BC, VanDevanter DR, Flume PA; STOP2 Investigators
Am J Respir Crit Care Med 2021 Dec 1;204(11):1295-1305. doi: 10.1164/rccm.202102-0461OC. PMID: 34469706Free PMC Article
Pallin M, Kumar S, Daley C, Dawadi S, Leong P, Carr E, Soldatos G
J Cyst Fibros 2021 Sep;20(5):785-791. Epub 2021 Mar 26 doi: 10.1016/j.jcf.2021.03.004. PMID: 33781701
Scott LK, Toner R
Lung 2017 Aug;195(4):397-401. Epub 2017 Jun 16 doi: 10.1007/s00408-017-0024-3. PMID: 28623538
Rogers GB, Hoffman LR, Johnson MW, Mayer-Hamblett N, Schwarze J, Carroll MP, Bruce KD
Expert Rev Mol Diagn 2011 Mar;11(2):197-206. doi: 10.1586/erm.10.117. PMID: 21405970Free PMC Article

Therapy

Waters V, Shaw M, Perrem L, Quon BS, Tullis E, Solomon M, Rayment JH, Lavoie A, Tse SM, Daigneault P, Bilodeau L, Price A, Nicholson M, Chin M, Parkins M, McKinney ML, Tam JS, Stanojevic S, Grasemann H, Ratjen F; (PIPE Study Investigators)
Eur Respir J 2024 Jun;63(6) Epub 2024 Jun 6 doi: 10.1183/13993003.02278-2023. PMID: 38697648
Goss CH, Heltshe SL, West NE, Skalland M, Sanders DB, Jain R, Barto TL, Fogarty B, Marshall BC, VanDevanter DR, Flume PA; STOP2 Investigators
Am J Respir Crit Care Med 2021 Dec 1;204(11):1295-1305. doi: 10.1164/rccm.202102-0461OC. PMID: 34469706Free PMC Article
Gavioli EM, Guardado N, Haniff F, Deiab N, Vider E
Microb Drug Resist 2021 Dec;27(12):1726-1732. Epub 2021 Jun 1 doi: 10.1089/mdr.2020.0602. PMID: 34077286
Scott LK, Toner R
Lung 2017 Aug;195(4):397-401. Epub 2017 Jun 16 doi: 10.1007/s00408-017-0024-3. PMID: 28623538
Parkins MD, Elborn JS
J Antimicrob Chemother 2010 Sep;65(9):1853-61. Epub 2010 Jul 6 doi: 10.1093/jac/dkq245. PMID: 20605846

Prognosis

Waters V, Shaw M, Perrem L, Quon BS, Tullis E, Solomon M, Rayment JH, Lavoie A, Tse SM, Daigneault P, Bilodeau L, Price A, Nicholson M, Chin M, Parkins M, McKinney ML, Tam JS, Stanojevic S, Grasemann H, Ratjen F; (PIPE Study Investigators)
Eur Respir J 2024 Jun;63(6) Epub 2024 Jun 6 doi: 10.1183/13993003.02278-2023. PMID: 38697648
Hassanzad M, Kiani A, Abedini A, Ghaffaripour H, Emami H, Alizadeh N, Zoghi G, Hashemi S, Velayati AA
BMC Pulm Med 2021 Nov 8;21(1):353. doi: 10.1186/s12890-021-01728-8. PMID: 34743707Free PMC Article
Goss CH, Heltshe SL, West NE, Skalland M, Sanders DB, Jain R, Barto TL, Fogarty B, Marshall BC, VanDevanter DR, Flume PA; STOP2 Investigators
Am J Respir Crit Care Med 2021 Dec 1;204(11):1295-1305. doi: 10.1164/rccm.202102-0461OC. PMID: 34469706Free PMC Article
Scott LK, Toner R
Lung 2017 Aug;195(4):397-401. Epub 2017 Jun 16 doi: 10.1007/s00408-017-0024-3. PMID: 28623538
Parkins MD, Elborn JS
J Antimicrob Chemother 2010 Sep;65(9):1853-61. Epub 2010 Jul 6 doi: 10.1093/jac/dkq245. PMID: 20605846

Clinical prediction guides

Waters V, Shaw M, Perrem L, Quon BS, Tullis E, Solomon M, Rayment JH, Lavoie A, Tse SM, Daigneault P, Bilodeau L, Price A, Nicholson M, Chin M, Parkins M, McKinney ML, Tam JS, Stanojevic S, Grasemann H, Ratjen F; (PIPE Study Investigators)
Eur Respir J 2024 Jun;63(6) Epub 2024 Jun 6 doi: 10.1183/13993003.02278-2023. PMID: 38697648
Hassanzad M, Kiani A, Abedini A, Ghaffaripour H, Emami H, Alizadeh N, Zoghi G, Hashemi S, Velayati AA
BMC Pulm Med 2021 Nov 8;21(1):353. doi: 10.1186/s12890-021-01728-8. PMID: 34743707Free PMC Article
Goss CH, Heltshe SL, West NE, Skalland M, Sanders DB, Jain R, Barto TL, Fogarty B, Marshall BC, VanDevanter DR, Flume PA; STOP2 Investigators
Am J Respir Crit Care Med 2021 Dec 1;204(11):1295-1305. doi: 10.1164/rccm.202102-0461OC. PMID: 34469706Free PMC Article
Scott LK, Toner R
Lung 2017 Aug;195(4):397-401. Epub 2017 Jun 16 doi: 10.1007/s00408-017-0024-3. PMID: 28623538
Parkins MD, Elborn JS
J Antimicrob Chemother 2010 Sep;65(9):1853-61. Epub 2010 Jul 6 doi: 10.1093/jac/dkq245. PMID: 20605846

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