Literature DB >> 34147501

Pulmonary Complications in Cystic Fibrosis: Past, Present, and Future: Adult Cystic Fibrosis Series.

Christina M Mingora1, Patrick A Flume2.   

Abstract

1Cystic fibrosis (CF) is an autosomal recessive genetic condition with multisystemic disease manifestations, the most prominent of which occur in the respiratory system. Despite significant developments in disease understanding and therapeutics, each contributing to improved lung function and survival in patients with CF, several pulmonary complications, including pneumothorax, massive hemoptysis, and respiratory failure, continue to occur. In this review, we briefly describe each of these complications and their management and discuss how they impact the care and disease trajectory of individuals in whom they occur. Finally, we discuss the evolving role that palliative care and CF transmembrane conductance regular modulator therapies play in the natural disease course and care of patients with CF.
Copyright © 2021 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

Entities:  

Keywords:  CFTR modulator; complications; cystic fibrosis; hemoptysis; palliative care; pneumothorax; pulmonary hypertension; respiratory failure

Mesh:

Substances:

Year:  2021        PMID: 34147501      PMCID: PMC8727888          DOI: 10.1016/j.chest.2021.06.017

Source DB:  PubMed          Journal:  Chest        ISSN: 0012-3692            Impact factor:   10.262


  60 in total

1.  Cystic fibrosis.

Authors:  Steven M Rowe; Stacey Miller; Eric J Sorscher
Journal:  N Engl J Med       Date:  2005-05-12       Impact factor: 91.245

2.  Endobronchial Valves in the Treatment of Persistent Air Leaks.

Authors:  John M Hance; Jeremiah T Martin; Timothy W Mullett
Journal:  Ann Thorac Surg       Date:  2015-08-18       Impact factor: 4.330

Review 3.  Non-invasive ventilation for cystic fibrosis.

Authors:  Fidelma Moran; Judy M Bradley; Amanda J Piper
Journal:  Cochrane Database Syst Rev       Date:  2017-02-20

4.  Efficacy of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease.

Authors:  Kate M O'Shea; Orla M O'Carroll; Catherine Carroll; Brenda Grogan; Anna Connolly; Lynda O'Shaughnessy; Trevor T Nicholson; Charles G Gallagher; Edward F McKone
Journal:  Eur Respir J       Date:  2021-02-25       Impact factor: 16.671

5.  A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.

Authors:  Bonnie W Ramsey; Jane Davies; N Gerard McElvaney; Elizabeth Tullis; Scott C Bell; Pavel Dřevínek; Matthias Griese; Edward F McKone; Claire E Wainwright; Michael W Konstan; Richard Moss; Felix Ratjen; Isabelle Sermet-Gaudelus; Steven M Rowe; Qunming Dong; Sally Rodriguez; Karl Yen; Claudia Ordoñez; J Stuart Elborn
Journal:  N Engl J Med       Date:  2011-11-03       Impact factor: 91.245

6.  Outcomes of intensive care unit care in adults with cystic fibrosis.

Authors:  N Sood; L J Paradowski; J R Yankaskas
Journal:  Am J Respir Crit Care Med       Date:  2001-02       Impact factor: 21.405

7.  Pneumothorax in cystic fibrosis.

Authors:  Patrick A Flume; Charlie Strange; Xiaobu Ye; Myla Ebeling; Thomas Hulsey; Leslie L Clark
Journal:  Chest       Date:  2005-08       Impact factor: 9.410

8.  Haemodynamic definitions and updated clinical classification of pulmonary hypertension.

Authors:  Gérald Simonneau; David Montani; David S Celermajer; Christopher P Denton; Michael A Gatzoulis; Michael Krowka; Paul G Williams; Rogerio Souza
Journal:  Eur Respir J       Date:  2019-01-24       Impact factor: 16.671

9.  Prevalence of unmet palliative care needs in adults with cystic fibrosis.

Authors:  Elizabeth T Trandel; Joseph M Pilewski; Elisabeth P Dellon; Kwonho Jeong; Jonathan G Yabes; Laura T Moreines; Robert M Arnold; Zachariah P Hoydich; Dio Kavalieratos
Journal:  J Cyst Fibros       Date:  2019-12-18       Impact factor: 5.482

10.  Hemoptysis from the perspective of people with cystic fibrosis.

Authors:  Christina M Romàn; Holly C Loughlin; Enid Aliaj; Robert J Fay; Quynh T Tran; Drucy Borowitz
Journal:  Clin Respir J       Date:  2020-01-01       Impact factor: 2.570

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  1 in total

Review 1.  Role for animal models in understanding essential fatty acid deficiency in cystic fibrosis.

Authors:  Deanne H Hryciw; Courtney A Jackson; Nirajan Shrestha; David Parsons; Martin Donnelley; Andrew J McAinch
Journal:  Cell Mol Life Sci       Date:  2021-11-05       Impact factor: 9.261

  1 in total

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