Modulation, microbiota and inflammation in the adult CF gut: A prospective study

Ronan, N.J.; Einarsson, G.G.; Deane, Jennifer; Fouhy, Fiona; Rea, Mary C.; Hill, Colin; Shanahan, Fergus; Elborn, J. Stuart; Ross, R. Paul; McCarthy, M.; Murphy, D.M.; Eustace, JA; MM, Tunney; Stanton, Catherine; Plant, B.J.

doi:10.1016/j.jcf.2022.06.002

Cited by 13 publications

(19 citation statements)

References 32 publications

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“…Similar results were observed in the KIWI trial, where ivacaftor resulted in a significant number of children between the ages of 2−5 ending the study with FE >200 μg/g [25]. In contrast, exocrine pancreatic function in adult cohorts appears to be irreversibly lost [26]. It is highly likely that ETI will also have similar effects on exocrine pancreatic function in children with CF.…”

Section: Exocrine Pancreassupporting

confidence: 78%

Nutritional and metabolic management for cystic fibrosis in a post-cystic fibrosis transmembrane conductance modulator era

Wilschanski

Peckham

2022

Current Opinion in Pulmonary Medicine

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Purpose of reviewThe introduction of highly effective cystic fibrosis transmembrane conductance regulator modulators has resulted in a paradigm shift towards treating underlying cause of cystic fibrosis (CF) rather than the ensuing complications. In this review, we will describe the impact of these small molecules on growth, nutrition, and metabolic status in people with CF (pwCF). Recent findingResults of clinical trials and real world data demonstrate that these small molecules are having a significant impact of on augmenting body weight, improving nutritional status and reducing gastrointestinal symptom burden. Early treatment can also positively impact on pancreatic endocrine and exocrine function.

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Section: Exocrine Pancreassupporting

confidence: 78%

Nutritional and metabolic management for cystic fibrosis in a post-cystic fibrosis transmembrane conductance modulator era

Wilschanski

Peckham

2022

Current Opinion in Pulmonary Medicine

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“…Results for the frequently observed inflammation in the gut following modulator treatment are also contrasting. Some studies observed a reduction in faecal calprotectin following modulator usage [15,20,21], whilst others [16] and our own group [19], did not measure any notable reduction.…”

Section: Introductionmentioning

confidence: 58%

Impact of extended Elexacaftor/Tezacaftor/Ivacaftor therapy on the gut microbiome in cystic fibrosis

Marsh,

Dos Santos,

Yule

et al. 2024

Preprint

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BackgroundThere is a paucity of knowledge on the longer-term effects of CF transmembrane conductance regulator (CFTR) modulator therapies upon the gut microbiome and associated outcomes. In a pilot study, we investigated longitudinal Elexacaftor/Tezacaftor/Ivacaftor (ETI) therapy on the gut microbiota, metabolomic functioning, and clinical outcomes in people with CF (pwCF).Study designFaecal samples from 20 pwCF were acquired before and then following 3, 6, and 17+ months of ETI therapy. Samples were subjected to microbiota sequencing and targeted metabolomics to profile and quantify short-chain fatty acid composition. Ten healthy matched controls were included for comparison. Clinical data, including markers of intestinal function were integrated to investigate relationships.ResultsExtended ETI therapy increased core microbiota diversity and composition, which translated to gradual shifts in whole microbiota composition towards that observed in healthy controls. Despite becoming more similar over time, CF microbiota and functional metabolite compositions remained significantly different to healthy controls. Antibiotic treatment for pulmonary infection significantly explained a relatively large degree of variation within the whole microbiota and rarer satellite taxa. Clinical outcomes were not significantly different following ETI.ConclusionsA positive trajectory towards the microbiota observed in healthy controls was found. However, we posit that progression was predominately impeded by pulmonary antibiotics administration. We recommend future studies use integrated omics approaches within a combination of long-term longitudinal patient studies and model experimental systems. This will deepen our understanding of the impacts of CFTR modulator therapy and respiratory antibiotic interventions upon the gut microbiome and gastrointestinal pathophysiology in CF.

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“…Recent clinical investigations suggest that CFTR-targeted therapy is able to improve small intestinal alkalinity [39] as well as improve gut health [40] , but this issue is controversial [41] . In addition, this extremely expensive therapy is not available to all patients with CF, and not all CFTR mutations are amenable to rescue.…”

Section: Cftr-dependent and Independent Improvement Of Gut Fluidity A...mentioning

confidence: 99%

CFTR-beyond the airways: Recent findings on the role of the CFTR channel in the pancreas, the intestine and the kidneys

Hegyi

Seidler

Kunzelmann

2023

Journal of Cystic Fibrosis

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Modulation, microbiota and inflammation in the adult CF gut: A prospective study

Cited by 13 publications

References 32 publications

Nutritional and metabolic management for cystic fibrosis in a post-cystic fibrosis transmembrane conductance modulator era

Nutritional and metabolic management for cystic fibrosis in a post-cystic fibrosis transmembrane conductance modulator era

Impact of extended Elexacaftor/Tezacaftor/Ivacaftor therapy on the gut microbiome in cystic fibrosis

CFTR-beyond the airways: Recent findings on the role of the CFTR channel in the pancreas, the intestine and the kidneys

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