Caley L R, White H, de Goffau M C, Floto R A, Parkhill J, Marsland B, Peckham D G
Leeds Institute of Medical Research, St James's University Hospital, Clinical Sciences Building, Leeds, LS9 7TF, UK.
Nutrition, Health & Environment, Leeds Beckett University, Leeds, UK.
Dig Dis Sci. 2023 May;68(5):1797-1814. doi: 10.1007/s10620-022-07812-1. Epub 2023 Jan 4.
Cystic Fibrosis (CF) is associated with gut dysbiosis, local and systemic inflammation, and impaired immune function. Gut microbiota dysbiosis results from changes in the complex gut milieu in response to CF transmembrane conductance regulator (CFTR) dysfunction, pancreatic malabsorption, diet, medications, and environmental influences. In several diseases, alteration of the gut microbiota influences local and systemic inflammation and disease outcomes. We conducted a systematic review of the gut microbiota in CF and explored factors influencing dysbiosis.
An electronic search of three databases was conducted in January 2019, and re-run in June 2021. Human, animal, and in vitro studies were included. The primary outcome was differences in the gut microbiota between people with CF (pwCF) and healthy controls. Secondary outcomes included the relationship between the gut microbiota and other factors, including diet, medication, inflammation, and pulmonary function in pwCF.
Thirty-eight studies were identified. The literature confirmed the presence of CF-related gut dysbiosis, characterized by reduced diversity and several taxonomic changes. There was a relative increase of bacteria associated with a pro-inflammatory response coupled with a reduction of those considered anti-inflammatory. However, studies linking gut dysbiosis to systemic and lung inflammation were limited. Causes of gut dysbiosis were multifactorial, and findings were variable. Data on the impact of CFTR modulators on the gut microbiota were limited.
CF-related gut dysbiosis is evident in pwCF. Whether this influences local and systemic disease and is amenable to interventions with diet and drugs, such as CFTR modulators, requires further investigation.
囊性纤维化（CF）与肠道菌群失调、局部和全身炎症以及免疫功能受损有关。肠道微生物群失调是由于复杂的肠道环境因CF跨膜传导调节因子（CFTR）功能障碍、胰腺吸收不良、饮食、药物和环境影响而发生变化所致。在一些疾病中，肠道微生物群的改变会影响局部和全身炎症以及疾病转归。我们对CF患者的肠道微生物群进行了系统综述，并探讨了影响菌群失调的因素。
于2019年1月对三个数据库进行了电子检索，并于2021年6月重新检索。纳入了人体、动物和体外研究。主要结局是CF患者（pwCF）与健康对照者之间肠道微生物群的差异。次要结局包括肠道微生物群与其他因素之间的关系，这些因素包括pwCF患者的饮食、药物、炎症和肺功能。
共确定了38项研究。文献证实存在与CF相关的肠道菌群失调，其特征为多样性降低和一些分类学变化。与促炎反应相关的细菌相对增加，而被认为具有抗炎作用的细菌减少。然而，将肠道菌群失调与全身和肺部炎症联系起来的研究有限。肠道菌群失调的原因是多因素的，研究结果存在差异。关于CFTR调节剂对肠道微生物群影响的数据有限。
pwCF中与CF相关的肠道菌群失调很明显。这是否会影响局部和全身疾病，以及是否适合通过饮食和药物（如CFTR调节剂）进行干预，需要进一步研究。
