The clinical significance of the gut microbiota in cystic fibrosis and the potential for dietary therapies

Li, Li; Somerset, Shawn

doi:10.1016/j.clnu.2014.04.004

Cited by 54 publications

(38 citation statements)

References 112 publications

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“…Antibiotic treatment reduced the expression of pro-inflammatory genes in the intestine of CF mice, supporting the hypothesis that SIBO represents a significant factor for intestinal inflammation in CF mice [92]. As an important modifiable factor, also treatment with probiotics and prebiotics have shown promise in improving CF gastrointestinal disease [95]. A recent prospective double blind cross over study in CF patients could demonstrate a positive effect of Lactobacillus reuteri on the gut microbiota, thereby significantly decreasing gastrointestinal inflammation and reducing digestive discomfort [96].…”

Section: The Pathomechanism Of Cfldmentioning

confidence: 72%

Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology

Staufer

Halilbasic

Trauner

et al. 2014

IJMS

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Due to improved medical care, life expectancy in patients with cystic fibrosis (CF) has veritably improved over the last decades. Importantly, cystic fibrosis related liver disease (CFLD) has become one of the leading causes of morbidity and mortality in CF patients. However, CFLD might be largely underdiagnosed and diagnostic criteria need to be refined. The underlying pathomechanisms are largely unknown, and treatment strategies with proven efficacy are lacking. This review focuses on current invasive and non-invasive diagnostic standards, the current knowledge on the pathophysiology of CFLD, treatment strategies, and possible future developments.

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Section: The Pathomechanism Of Cfldmentioning

confidence: 72%

Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology

Staufer

Halilbasic

Trauner

et al. 2014

IJMS

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“…7,8 A cross-sectional and longitudinal comparison of the microbiota of children with CF with their healthy siblings identified instability and decreased microbiome diversity with a decrease in Clostridia, Bifidobacterium , and Veillonella in patients with CF. 38 CFTR allelic variants correlate with patterns of GI dysbiosis, and patients with homozygous ΔF508 deletion have more significant alteration in gut microbiome patterns.…”

Section: Evidence For An Altered Gut Microbiome In Cfmentioning

confidence: 99%

Neonatal Gastrointestinal and Respiratory Microbiome in Cystic Fibrosis: Potential Interactions and Implications for Systemic Health

Madan

2016

Clinical Therapeutics

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Purpose The gastrointestinal microbiome plays a critical role in nutrition and metabolic and immune functions in infants and young children and has implications for lifelong health. Cystic fibrosis (CF) transmembrane conductance regulator (CFTR) mutations in CF result in viscous mucous production, frequent exposure to antibiotics, and atypical colonization patterns, resulting in an evolving dysbiosis of the gastrointestinal and respiratory microsystems; dysbiosis in CF results in systemic inflammation, chronic infection, and dysregulation of immune function. Dysbiosis in both the respiratory system and gut contributes to undernutrition, growth failure, and long-term respiratory and systemic morbidity in infants and children with CF. Understanding the role that the gut and respiratory microbiome plays in health or disease progression in CF will afford opportunities to better identify interventions to affect clinical changes. Methods Summary was done of the pertinent literature in CF and the study of the microbiome and probiotics. Findings New studies have identified bacteria in the respiratory tract in CF that are typically members of the intestinal microbiota, and enteral exposures to breast milk and probiotics are associated with prolonged periods of respiratory stability in CF. Implications Understanding the complex interactions between the CFTR mutations, microbial colonization, and mucosal and systemic immunity is of major importance to inform new treatment strategies (such as restoring a healthier microbiome with probiotics or dietary interventions) to improve nutritional status and immune competence and to decrease morbidity and mortality in CF.

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“…Cystic fibrosis (CF) frequently involves the gastrointestinal tract, with one of the most common complications being chronic inflammation (Li and Somerset 2014). Decreased bacterial diversity is associated with overgrowth of particular groups of resident bacteria that can induce inflammatory responses, which could be a factor leading to intestinal inflammation in CF.…”

Section: Introductionmentioning

confidence: 99%

“…2013). In this context, the gut microbiota could be one of the factors affecting nutrient metabolism and energy balance (Li and Somerset 2014). …”

Section: Introductionmentioning

confidence: 99%

Impact of cystic fibrosis disease on archaea and bacteria composition of gut microbiota

Miragoli

Federici

Ferrari

et al. 2016

FEMS Microbiology Ecology

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Cystic fibrosis is often associated with intestinal inflammation due to several factors, including altered gut microbiota composition. In this study, we analyzed the fecal microbiota among patients with cystic fibrosis of 10–22 years of age, and compared the findings with age-matched healthy subjects. The participating patients included 14 homozygotes and 14 heterozygotes with the delF508 mutation, and 2 heterozygotes presenting non-delF508 mutations. We used PCR-DGGE and qPCR to analyze the presence of bacteria, archaea and sulfate-reducing bacteria. Overall, our findings confirmed disruption of the cystic fibrosis gut microbiota. Principal component analysis of the qPCR data revealed no differences between homozygotes and heterozygotes, while both groups were distinct from healthy subjects who showed higher biodiversity. Archaea were under the detection limit in all homozygotes subjects, whereas methanogens were detected in 62% of both cystic fibrosis heterozygotes and healthy subjects. Our qPCR results revealed a low frequency of sulfate-reducing bacteria in the homozygote (13%) and heterozygote (13%) patients with cystic fibrosis compared with healthy subjects (87.5%). This is a pioneer study showing that patients with cystic fibrosis exhibit significant reduction of H2-consuming microorganisms, which could increase hydrogen accumulation in the colon and the expulsion of this gas through non-microbial routes.

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The clinical significance of the gut microbiota in cystic fibrosis and the potential for dietary therapies

Cited by 54 publications

References 112 publications

Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology

Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology

Neonatal Gastrointestinal and Respiratory Microbiome in Cystic Fibrosis: Potential Interactions and Implications for Systemic Health

Impact of cystic fibrosis disease on archaea and bacteria composition of gut microbiota

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