title: Disrupted progression of the intestinal microbiota with age in children with cystic fibrosis.
abstract: Cystic fibrosis (CF) is a genetic disorder that leads to formation of thick epithelial secretions in affected organs. Chronic microbial infections associated with thick mucus secretions are the hallmarks of lung disease in CF. Despite similar conditions existing in the gastrointestinal tract, it is much less studied. We therefore examined the microbial communities within the gastrointestinal tract of children with and without CF (either pancreatic sufficient or insufficient) across a range of childhood ages (0.87-17 years). We observed a substantial reduction in the richness and diversity of gut bacteria associated with CF from early childhood (2 years) until late adolescence (17 years). A number of bacteria that establish themselves in the gut of healthy children were unable to do so in children with CF. In contrast, a few bacteria dominated the gut microbiota in children with CF and are unlikely to be beneficial for the metabolic function of the gut. A functioning pancreas (pancreatic sufficient) under a CF lifestyle showed little effect on microbial communities. Our results argue that any attempts to rectify the loss of bacterial diversity and provide normal bacterial function in the gut of CF patients should be conducted no later than early childhood.
authors.affiliation: Centre for Marine Bio-innovation, University of New South Wales, Sydney, 2052, Australia.|School of Biotechnology and Biomolecular Sciences, and University of New South Wales, Sydney, 2052, Australia.
authors.forename: Shaun
authors.initials: S
authors.lastname: Nielsen
authors.affiliation: School of Biotechnology and Biomolecular Sciences, and University of New South Wales, Sydney, 2052, Australia.
authors.forename: Bronwen
authors.initials: B
authors.lastname: Needham
authors.affiliation: Discipline of Paediatrics, School of Women's and Children's Health, Medicine, University of New South Wales, Sydney, 2052, Australia.
authors.forename: Steven T
authors.initials: ST
authors.lastname: Leach
authors.affiliation: Department of Paediatrics, University of Otago, Christchurch, 8011, New Zealand.
authors.forename: Andrew S
authors.initials: AS
authors.lastname: Day
authors.affiliation: Discipline of Paediatrics, School of Women's and Children's Health, Medicine, University of New South Wales, Sydney, 2052, Australia.|Department of Paediatric Respiratory and Sydney Children's Hospital, Randwick, 2031, Australia.
authors.forename: Adam
authors.initials: A
authors.lastname: Jaffe
authors.affiliation: Centre for Marine Bio-innovation, University of New South Wales, Sydney, 2052, Australia.|School of Biotechnology and Biomolecular Sciences, and University of New South Wales, Sydney, 2052, Australia.
authors.forename: Torsten
authors.initials: T
authors.lastname: Thomas
authors.affiliation: Discipline of Paediatrics, School of Women's and Children's Health, Medicine, University of New South Wales, Sydney, 2052, Australia.|Department of Paediatric Gastroenterology, Sydney Children's Hospital, Randwick, 2031, Australia.
authors.forename: Chee Y
authors.initials: CY
authors.lastname: Ooi
mesh_terms: D000367:Age Factors; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications*; D064806:Dysbiosis; D000069196:Gastrointestinal Microbiome; D006801:Humans; D064307:Microbiota