Factors associated with stress responses in staff caring for patients with cystic fibrosis

Objective: To ascertain major risk factors associated with pulmonary exacerbation and pulmonary function decline in cystic fibrosis. Method: The systematic review was conducted at Aga Khan University, Karachi, in September 2018, and comprised electronic search of PubMed, Ovid, Science Direct and Cumulative Index of Nursing and Allied Health Literature databases of studies conducted from January 1990 to September 2018 which were categorised into 3 sets; 1990-98, 1999-2007 and 2008-18. Studies included for review focussed on articles with pulmonary exacerbation as the health outcome indicator, and had diagnosis of cystic fibrosis as the inclusion criteria, while risk factors were the exposure terms used in the search process. References in bibliographies of the included studies were also systematically searched for relevant documents. Results: Of the 60 studies obtained, 31(51.7%) were selected; 2(6.45%) from 1990-98, 7(22.58%) from 1999-2007 and 22(70.96%) from 2008-18. Overall, 17(54.83%) were cohort studies, 7(22.5%) were cross-sectional studies, 3(9.6%) were case-control studies, 3(9.6%) were randomised controlled trials and 1(3.2%) was systematic review and meta-analysis. In terms of major risk factors, genetic mutations were cited by 4(12.9%) studies, infections and inflammatory biomarkers by 15(48.4%), nutritional deficiencies by 9(29%) and geographical and socioeconomic status by 3(9.6%) studies. Conclusion: Early identification and recognition of risk factors associated with pulmonary exacerbation can have an explicit impact on its management, leading to decreased morbidity and mortality burden in cystic fibrosis cases. Key Words: Pulmonary exacerbation, Cystic fibrosis, Risk factors, Systematic review. Continuous...

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P159 Factors associated with Mycobacterium abscessus group (MABS) infection type in people with cystic fibrosis

Journal of Cystic Fibrosis ◽

10.1016/s1569-1993(19)30453-9 ◽

2019 ◽

Vol 18 ◽

pp. S102-S103

Author(s):

R. Stockwell ◽

M. Wood ◽

R. Carter ◽

C. Tolson ◽

S. Pandey ◽

...

Keyword(s):

Cystic Fibrosis ◽

Infection Type ◽

Mycobacterium Abscessus ◽

Factors Associated

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P242 Factors associated with medication adherence in adult cystic fibrosis patients

Journal of Cystic Fibrosis ◽

10.1016/s1569-1993(18)30537-x ◽

2018 ◽

Vol 17 ◽

pp. S127-S128

Author(s):

N.Z. Uslu ◽

S. Olgun Yildizeli ◽

D. Kocakaya ◽

B. Ceyhan

Keyword(s):

Cystic Fibrosis ◽

Medication Adherence ◽

Factors Associated

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Stressors and Stress Responses in Cystic Fibrosis

Endoplasmic Reticulum Stress in Diseases ◽

10.1515/ersc-2018-0002 ◽

2018 ◽

Vol 5 (1) ◽

pp. 11-29 ◽

Cited By ~ 1

Author(s):

Zsuzsa Bebok ◽

Lianwu Fu

Keyword(s):

Cystic Fibrosis ◽

Stress Response ◽

Stress Responses ◽

Genetic Disorder ◽

Cellular Stress ◽

Cellular Stress Response ◽

Bicarbonate Transport ◽

Persistent Infections ◽

Human Disorders ◽

The Unfolded Protein Response

Abstract Cystic fibrosis (CF) is a life-shortening, genetic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR). The primary cause of CF is reduced CFTR-mediated chloride and bicarbonate transport, due to mutations in CFTR. However, inflammation and persistent infections influence clinical outcome. Cellular stress response pathways, such as the unfolded protein response (UPR) and the integrated stress response (ISR), referred to here as cellular stress response pathways (SRPs), contribute to the pathology of human disorders. Multiple studies have indicated activation of SRPs in CF tissues. We review our present understanding of how SRPs are activated in CF and their contribution to pathology. We conclude that reduced CFTR function in CF organs establishes a tissue environment in which internal or external insults activate SRPs. SRPs contribute to CF pathogenesis by reducing CFTR expression, enhancing inflammation with consequent tissue remodeling. Understanding the contribution of SRPs to CF pathogenesis is crucial even in the era of CFTR “modulators” that are designed to potentiate, correct or amplify CFTR function, since there is an urgent need for supportive treatments. Importantly, CF patients with established pathology could benefit from the targeted use of drugs that modulate SRPs to reduce the symptoms.

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