Review of Gastrointestinal Motility in Cystic Fibrosis |
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| Affiliation: | 1. Saint Louis University School of Medicine, SSM Health Cardinal Glennon Children''s Hospital, 1465 S Grand Blvd, St. Louis, MO 63104;2. Interim Chair, Department of Pediatrics, Professor of Pediatrics and Biochemistry, Saint Louis University School of Medicine, SSM Health Cardinal Glennon Children''s Hospital, 1465 S Grand BLVD, St. Louis, MO 63104;3. Emeritus Professor of Clinical Pediatrics, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, NY, 1001 Main Street, Buffalo, NY, 14203;4. Associate Professor and Medical Director, Neurogastroenterology and Motility Program, Department of Pediatrics, Saint Louis University School of Medicine, SSM Health Cardinal Glennon Children''s Hospital, 1465 S Grand Blvd, St. Louis, MO 63104;1. Saint Louis University School of Medicine, SSM Health Cardinal Glennon Children''s Hospital, 1465 S Grand Blvd, St. Louis, MO 63104;2. Interim Chair, Department of Pediatrics, Professor of Pediatrics and Biochemistry, Saint Louis University School of Medicine, SSM Health Cardinal Glennon Children''s Hospital, 1465 S Grand BLVD, St. Louis, MO 63104;3. Emeritus Professor of Clinical Pediatrics, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, NY, 1001 Main Street, Buffalo, NY, 14203;4. Associate Professor and Medical Director, Neurogastroenterology and Motility Program, Department of Pediatrics, Saint Louis University School of Medicine, SSM Health Cardinal Glennon Children''s Hospital, 1465 S Grand Blvd, St. Louis, MO 63104 |
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| Abstract: | Gastrointestinal manifestations in patients with cystic fibrosis (CF) are extremely common and have recently become a research focus. Gastrointestinal (GI) dysfunction is poorly understood in the CF population, despite many speculations including the role of luminal pH, bacterial overgrowth, and abnormal microbiome. Nevertheless, dysmotility is emerging as a possible key player in CF intestinal symptoms. Our review article aims to explore the sequelae of defective cystic fibrosis transmembrane conductance regulator (CFTR) genes on the GI tract as studied in both animals and humans, describe various presentations of intestinal dysmotility in CF, review newer diagnostic motility techniques including intraluminal manometry, and review the current literature regarding the potential role of dysmotility in CF-related intestinal pathologies. |
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