Millie Garg1, Chee Y Ooi2,3. 1. School of Women's and Children's Health, Medicine, University of New South Wales, Randwick, NSW, 2031, Australia. 2. School of Women's and Children's Health, Medicine, University of New South Wales, Randwick, NSW, 2031, Australia. keith.ooi@unsw.edu.au. 3. Department of Paediatric Gastroenterology, Sydney Children's Hospital, Randwick, NSW, 2031, Australia. keith.ooi@unsw.edu.au.
Abstract
PURPOSE OF REVIEW: Intestinal inflammation, dysbiosis, and increased gastrointestinal malignancy risks are well-described in patients with cystic fibrosis (CF). However, there is limited understanding of their pathophysiology. This review aims to discuss these issues and assess potential links between them. RECENT FINDINGS: Evidence of links between intestinal inflammation and dysbiosis (an imbalance in intestinal microbial populations) exist. Recent studies have demonstrated reduction in intestinal inflammation with probiotic administration. Both bacterial dysbiosis and gut inflammation contribute to the suboptimal nutritional status seen in children with CF. Short-chain fatty acids may be reduced in the gut lumen as a result of bacterial imbalances and may promote inflammation. Inflammation and bacterial dysbiosis in CF may also contribute to emerging adult complications such as gastrointestinal malignancy. An increase in carcinogenic microbes and reduction in microbes protective against cancer have been found in CF, linking bacterial dysbiosis and cancer. Murine studies suggest the CF gene, cystic fibrosis transmembrane conductance regulator (CFTR) gene, itself may be a tumour suppressor gene. The pathophysiology of interactions among intestinal inflammation, dysbiosis, and malignancy in CF is not clearly understood and requires further research.
PURPOSE OF REVIEW: Intestinal inflammation, dysbiosis, and increased gastrointestinal malignancy risks are well-described in patients with cystic fibrosis (CF). However, there is limited understanding of their pathophysiology. This review aims to discuss these issues and assess potential links between them. RECENT FINDINGS: Evidence of links between intestinal inflammation and dysbiosis (an imbalance in intestinal microbial populations) exist. Recent studies have demonstrated reduction in intestinal inflammation with probiotic administration. Both bacterial dysbiosis and gut inflammation contribute to the suboptimal nutritional status seen in children with CF. Short-chain fatty acids may be reduced in the gut lumen as a result of bacterial imbalances and may promote inflammation. Inflammation and bacterial dysbiosis in CF may also contribute to emerging adult complications such as gastrointestinal malignancy. An increase in carcinogenic microbes and reduction in microbes protective against cancer have been found in CF, linking bacterial dysbiosis and cancer. Murine studies suggest the CF gene, cystic fibrosis transmembrane conductance regulator (CFTR) gene, itself may be a tumour suppressor gene. The pathophysiology of interactions among intestinal inflammation, dysbiosis, and malignancy in CF is not clearly understood and requires further research.
Authors: Patrick Maisonneuve; Stacey C FitzSimmons; Joseph P Neglia; Preston W Campbell; Albert B Lowenfels Journal: J Natl Cancer Inst Date: 2003-03-05 Impact factor: 13.506
Authors: Janelle C Arthur; Ernesto Perez-Chanona; Marcus Mühlbauer; Sarah Tomkovich; Joshua M Uronis; Ting-Jia Fan; Barry J Campbell; Turki Abujamel; Belgin Dogan; Arlin B Rogers; Jonathan M Rhodes; Alain Stintzi; Kenneth W Simpson; Jonathan J Hansen; Temitope O Keku; Anthony A Fodor; Christian Jobin Journal: Science Date: 2012-08-16 Impact factor: 47.728
Authors: Mario Ollero; Omer Junaidi; Munir M Zaman; Iphigenia Tzameli; Adolfo A Ferrando; Charlotte Andersson; Paola G Blanco; Eldad Bialecki; Steven D Freedman Journal: J Cell Physiol Date: 2004-08 Impact factor: 6.384
Authors: Aliye Uc; Alicia K Olivier; Michelle A Griffin; David K Meyerholz; Jianrong Yao; Maisam Abu-El-Haija; Katherine M Buchanan; Oriana G Vanegas Calderón; Marwa Abu-El-Haija; Alejandro A Pezzulo; Leah R Reznikov; Mark J Hoegger; Michael V Rector; Lynda S Ostedgaard; Peter J Taft; Nick D Gansemer; Paula S Ludwig; Emma E Hornick; David A Stoltz; Katie L Ode; Michael J Welsh; John F Engelhardt; Andrew W Norris Journal: Clin Sci (Lond) Date: 2015-01 Impact factor: 6.124
Authors: Samuel L Neff; Thomas H Hampton; Charles Puerner; Liviu Cengher; Georgia Doing; Alexandra J Lee; Katja Koeppen; Ambrose L Cheung; Deborah A Hogan; Robert A Cramer; Bruce A Stanton Journal: Sci Data Date: 2022-06-16 Impact factor: 8.501
Authors: Kathrin Krause; Benjamin T Kopp; Mia F Tazi; Kyle Caution; Kaitlin Hamilton; Asmaa Badr; Chandra Shrestha; Dmitry Tumin; Don Hayes; Frank Robledo-Avila; Luanne Hall-Stoodley; Brett G Klamer; Xiaoli Zhang; Santiago Partida-Sanchez; Narasimham L Parinandi; Stephen E Kirkby; Duaa Dakhlallah; Karen S McCoy; Estelle Cormet-Boyaka; Amal O Amer Journal: J Cyst Fibros Date: 2017-12-11 Impact factor: 5.482
Authors: Ashlee M Strubberg; Jinghua Liu; Nancy M Walker; Casey D Stefanski; R John MacLeod; Scott T Magness; Lane L Clarke Journal: Cell Mol Gastroenterol Hepatol Date: 2017-12-07