The role of endoscopy and biopsy in the management of severe gastrointestinal disease in cystic fibrosis (CF) patients

Cystic fibrosis (CF) is a genetic, life-limiting disease without a cure; treatment is complex and lifelong. Respiratory failure is the most common cause of death; however, gastrointestinal disease, diabetes and liver disease are common comorbidities. Coronavirus disease (COVID-19) rapidly changed healthcare services across the globe, including redeployment of healthcare professionals. This adult CF service was challenged to continue a patient facing service within severe staffing and structural limitations.Not only were many members of the CF multidisciplinary team (MDT) redeployed at the start of the first wave, but also both the CF and ambulatory care wards were closed. Fortunately, the CF clinical nurse specialists (CF-CNSs) remained in their role. Rapid change and adaptation of the CF service was required to ensure that patients did not feel abandoned and access to treatment remained available. The role of the CF-CNS was therefore pivotal in this change.The aim of this project was to use quality improvement methodology to plan an emergency service allowing a reintroduction of ambulatory care services. Success was measured by the number of patients clinically reviewed with or without intervention, and the reasons for patients contacting the CF-CNS via email and phone were recorded.In weeks 1 and 2 of the emergency service, the CF-CNSs triaged patients by phone, then reviewed face-to-face when necessary. This first step allowed the CF-CNSs to start two patients on home intravenous antibiotics. This service continued to be developed over the following 12 weeks, leading to a total of 36 patient attendances. In March 2020, n=1187 patients made contact (mostly COVID-19, unwell and medication related), in April n=904 and May n=870 (blood test results, unwell and medication related).The motivation of the CF-CNSs was pivotal to the success of this initiative with the CF MDT available to provide some support and advice. It concluded at week 12, which then saw the opening of the formal ambulatory care ward and returning redeployed ward staff.

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Quality of life and psychological distress in patients with cystic fibrosis: The role of (un)favourable illness representations

PsycEXTRA Dataset ◽

10.1037/e524362011-063 ◽

2008 ◽

Author(s):

A. Casier ◽

L. Goubert ◽

D. Huse ◽

M. Theunis ◽

G. Crombez

Keyword(s):

Quality Of Life ◽

Cystic Fibrosis ◽

Psychological Distress ◽

Illness Representations

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Systems Biology and Bile Acid Signalling in Microbiome-Host Interactions in the Cystic Fibrosis Lung

Antibiotics ◽

10.3390/antibiotics10070766 ◽

2021 ◽

Vol 10 (7) ◽

pp. 766

Author(s):

David F. Woods ◽

Stephanie Flynn ◽

Jose A. Caparrós-Martín ◽

Stephen M. Stick ◽

F. Jerry Reen ◽

...

Keyword(s):

Cystic Fibrosis ◽

Bile Acids ◽

Host Response ◽

Signal Molecules ◽

Therapeutic Approaches ◽

Related Factors ◽

Host Interactions ◽

Important Host ◽

Respiratory Microbiota

The study of the respiratory microbiota has revealed that the lungs of healthy and diseased individuals harbour distinct microbial communities. Imbalances in these communities can contribute to the pathogenesis of lung disease. How these imbalances occur and establish is largely unknown. This review is focused on the genetically inherited condition of Cystic Fibrosis (CF). Understanding the microbial and host-related factors that govern the establishment of chronic CF lung inflammation and pathogen colonisation is essential. Specifically, dissecting the interplay in the inflammation–pathogen–host axis. Bile acids are important host derived and microbially modified signal molecules that have been detected in CF lungs. These bile acids are associated with inflammation and restructuring of the lung microbiota linked to chronicity. This community remodelling involves a switch in the lung microbiota from a high biodiversity/low pathogen state to a low biodiversity/pathogen-dominated state. Bile acids are particularly associated with the dominance of Proteobacterial pathogens. The ability of bile acids to impact directly on both the lung microbiota and the host response offers a unifying principle underpinning the pathogenesis of CF. The modulating role of bile acids in lung microbiota dysbiosis and inflammation could offer new potential targets for designing innovative therapeutic approaches for respiratory disease.

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Understanding the expanding role of pharmacy services in outpatient cystic fibrosis care

Pediatric Pulmonology ◽

10.1002/ppul.25283 ◽

2021 ◽

Author(s):

Jeffery T. Zobell ◽

Justin Moss ◽

Stephanie Heuser ◽

Lynda Roe ◽

David C. Young

Keyword(s):

Cystic Fibrosis ◽

Pharmacy Services

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Oral–Gut Microbiome Axis in Gastrointestinal Disease and Cancer

Cancers ◽

10.3390/cancers13092124 ◽

2021 ◽

Vol 13 (9) ◽

pp. 2124

Author(s):

Se-Young Park ◽

Byeong-Oh Hwang ◽

Mihwa Lim ◽

Seung-Ho Ok ◽

Sun-Kyoung Lee ◽

...

Keyword(s):

Oral Cavity ◽

Gut Microbiome ◽

Gastrointestinal Disease ◽

Oral Microbiota ◽

Microbial Interaction ◽

Barrier Dysfunction ◽

Gut Barrier ◽

Microbial Ecosystem ◽

Precise Diagnosis

It is well-known that microbiota dysbiosis is closely associated with numerous diseases in the human body. The oral cavity and gut are the two largest microbial habitats, playing a major role in microbiome-associated diseases. Even though the oral cavity and gut are continuous regions connected through the gastrointestinal tract, the oral and gut microbiome profiles are well-segregated due to the oral–gut barrier. However, the oral microbiota can translocate to the intestinal mucosa in conditions of the oral–gut barrier dysfunction. Inversely, the gut-to-oral microbial transmission occurs as well in inter- and intrapersonal manners. Recently, it has been reported that oral and gut microbiomes interdependently regulate physiological functions and pathological processes. Oral-to-gut and gut-to-oral microbial transmissions can shape and/or reshape the microbial ecosystem in both habitats, eventually modulating pathogenesis of disease. However, the oral–gut microbial interaction in pathogenesis has been underappreciated to date. Here, we will highlight the oral–gut microbiome crosstalk and its implications in the pathogenesis of the gastrointestinal disease and cancer. Better understanding the role of the oral–gut microbiome axis in pathogenesis will be advantageous for precise diagnosis/prognosis and effective treatment.

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Modeling Gastrointestinal Diseases Using Organoids to Understand Healing and Regenerative Processes

Cells ◽

10.3390/cells10061331 ◽

2021 ◽

Vol 10 (6) ◽

pp. 1331

Author(s):

Alexane Ollivier ◽

Maxime M. Mahe ◽

Géraldine Guasch

Keyword(s):

Molecular Mechanisms ◽

Gastrointestinal Disease ◽

3D Culture ◽

Gastrointestinal Diseases ◽

Continuous Series ◽

Regenerative Therapy ◽

Disease Mechanisms ◽

Epithelial Wound Healing ◽

Potential Applications

The gastrointestinal tract is a continuous series of organs from the mouth to the esophagus, stomach, intestine and anus that allows digestion to occur. These organs are frequently associated with chronic stress and injury during life, subjecting these tissues to frequent regeneration and to the risk of developing disease-associated cancers. The possibility of generating human 3D culture systems, named organoids, that resemble histologically and functionally specific organs, has opened up potential applications in the analysis of the cellular and molecular mechanisms involved in epithelial wound healing and regenerative therapy. Here, we review how during normal development homeostasis takes place, and the role of the microenvironmental niche cells in the intestinal stem cell crypt as an example. Then, we introduce the notion of a perturbed niche during disease conditions affecting the esophageal–stomach junction and the colon, and describe the potential applications of organoid models in the analysis of human gastrointestinal disease mechanisms. Finally, we highlight the perspectives of organoid-based regenerative therapy to improve the repair of the epithelial barrier.

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