Review of Gastrointestinal Motility in Cystic Fibrosis

De Lisle R.C. Borowitz D.

The cystic fibrosis intestine.

Cold Spring Harb Perspect Med. 3 ()

Cystic Fibrosis Disease Modifiers: Complex Genetics Defines the Phenotypic Diversity in a Monogenic Disease.

Annu Rev Genomics Hum Genet. 19: 201-222

Nutritional Status in Childhood as a Prognostic Factor in Patients with Cystic Fibrosis.

Lung. 197: 371-376

Improved prognosis in CF patients with normal fat absorption.

J Pediatr Gastroenterol Nutr. : S99-105

Early Life Growth Trajectories in Cystic Fibrosis are Associated with Pulmonary Function at Age 6 Years.

J Pediatr. 167 ()

Longitudinal trends in nutritional status and the relation between lung function and BMI in cystic fibrosis: a population-based cohort study.

Am J Clin Nutr. 97: 872-877Yen E.H. Quinton H. Borowitz D.

Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis.

J Pediatr. 162 (): 530-535

https://www.cff.org/About-Us/Reports-and-Financials/Our-Strategic-Plan/Cure/.

Bile acids and bile salts: ionization and solubility properties.

Hepatology. 4 ()

Altered transit and bacterial overgrowth in the cystic fibrosis mouse small intestine.

Am J Physiol Gastrointest Liver Physiol. 293: G104-G111

Gastrointestinal transit times and motility in patients with cystic fibrosis.

Scand J Gastroenterol. 47: 920-926Strong T.V. Boehm K. Collins F.S.

Localization of cystic fibrosis transmembrane conductance regulator mRNA in the human gastrointestinal tract by in situ hybridization.

J Clin Invest. 93: 347-354

Bali, A., D.E. Stableforth, and P. Asquith, Prolonged small-intestinal transit time in cystic fibrosis. Br Med J (Clin Res Ed), 1983. 287(6398): p. 1011-3.

Gastric emptying time is faster in cystic fibrosis.

J Pediatr Gastroenterol Nutr. 25: 492-498

Corral, J.E., et al., Is Gastroparesis Found More Frequently in Patients with Cystic Fibrosis? A Systematic Review. Scientifica (Cairo), 2016. 2016: p. 2918139.

Gastrointestinal pH, motility/transit and permeability in cystic fibrosis.

J Pediatr Gastroenterol Nutr. 23: 513-523

Delayed small bowel transit in children with cystic fibrosis and pancreatic insufficiency.

J Pediatr Gastroenterol Nutr. 57: 81-84

Future directions in esophageal motility and function - new technology and methodology.

Neurogastroenterol Motil. : 48-56

Mechanisms of increased gastroesophageal reflux in patients with cystic fibrosis.

Am J Gastroenterol. 107: 1346-1353

Gastric secretion in cystic fibrosis in relation to the migrating motor complex.

Scand J Gastroenterol. 36: 121-127Maqbool S. Parkman H.P. Friedenberg F.K.

Wireless capsule motility: comparison of the SmartPill GI monitoring system with scintigraphy for measuring whole gut transit.

Dig Dis Sci. 54: 2167-2174

Gut hormones and gastrointestinal motility in children with cystic fibrosis.

Dig Dis Sci. 37: 187-192

Gastric transit and small intestinal transit time and motility assessed by a magnet tracking system.

BMC Gastroenterol. 11: 145

Postprandial changes in gastrointestinal function and transit in cystic fibrosis assessed by Magnetic Resonance Imaging.

J Cyst Fibros. Bordoni B. Simonelli M. Morabito B. The Other Side of the Fascia: The Smooth Muscle Part 1. Cureus. 11: e4651

Cystic Fibrosis Transmembrane Conductance Regulator in Sarcoplasmic Reticulum of Airway Smooth Muscle. Implications for Airway Contractility.

Am J Respir Crit Care Med. 193: 417-426

Ileal smooth muscle dysfunction and remodeling in cystic fibrosis.

Am J Physiol Gastrointest Liver Physiol. 303: G1-G8De Lisle R.C. Meldi L. Mueller R.

Intestinal smooth muscle dysfunction develops postnatally in cystic fibrosis mice.

J Pediatr Gastroenterol Nutr. 55: 689-694de Lisle R.C. Sewell R. Meldi L.

Enteric circular muscle dysfunction in the cystic fibrosis mouse small intestine.

Neurogastroenterol Motil. 22 ()

Expression and distribution of cystic fibrosis transmembrane conductance regulator in neurons of the human brain.

J Histochem Cytochem. 57: 1113-1120

Cystic fibrosis transmembrane conductance regulator expression in human hypothalamus.

Neuroreport. 9: 141-144

Cystic fibrosis transmembrane conductance regulator modulates enteric cholinergic activities and is abnormally expressed in the enteric ganglia of patients with slow transit constipation.

J Gastroenterol. 54: 994-1006

Xue, R., et al., Expression of Cystic Fibrosis Transmembrane Conductance Regulator in Ganglia of Human Gastrointestinal Tract. Sci Rep, 2016. 6: p. 30926.

De Lisle R.C. Roach E. Jansson K.

Effects of laxative and N-acetylcysteine on mucus accumulation, bacterial load, transit, and inflammation in the cystic fibrosis mouse small intestine.

Am J Physiol Gastrointest Liver Physiol. 293: G577-G584

Oral antibiotic therapy improves fat absorption in cystic fibrosis patients with small intestine bacterial overgrowth.

J Cyst Fibros. 10: 418-421

Molecular consequences of cystic fibrosis transmembrane regulator (CFTR) gene mutations in the exocrine pancreas.

Gut. 52: 1159-1164

Gastrointestinal motility, dysbiosis and opioid-induced tolerance: is there a link?.

Nat Rev Gastroenterol Hepatol. 16: 323-324

Gut Microbial Dysbiosis in the Pathogenesis of Gastrointestinal Dysmotility and Metabolic Disorders.

J Neurogastroenterol Motil. 27: 19-34

A novel guluronate oligomer improves intestinal transit and survival in cystic fibrosis mice.

J Cyst Fibros. 15: 745-751

Risk factors for small bowel bacterial overgrowth in cystic fibrosis.

J Pediatr Gastroenterol Nutr. 44: 212-218Lisowska A. Wojtowicz J. Walkowiak J.

Small intestine bacterial overgrowth is frequent in cystic fibrosis: combined hydrogen and methane measurements are required for its detection.

Acta Biochim Pol. 56: 631-634

Enteric neuroplasticity and dysmotility in inflammatory disease: key players and possible therapeutic targets.

Am J Physiol Gastrointest Liver Physiol. 317 ()

Severe disease in Cystic Fibrosis and fecal calprotectin levels.

Immunobiology. 222: 582-586

Intestinal Inflammation in Children with Cystic Fibrosis Is Associated with Crohn's-Like Microbiota Disturbances.

J Clin Med. 8

Intestinal pH and gastrointestinal transit profiles in cystic fibrosis patients measured by wireless motility capsule.

Dig Dis Sci. 58: 2275-2281Feigelson J. Sauvegrain J.

[Letter: Gastro-esophageal reflux in mucoviscidosis].

Nouv Presse Med. 4: 2729-2730

Gastroesophageal reflux disease in lung transplant patients with cystic fibrosis.

Am J Surg. 204: e21-e26Scott R.B. O'Loughlin E.V. Gall D.G.

Gastroesophageal reflux in patients with cystic fibrosis.

J Pediatr. 106: 223-227

Gastroesophageal reflux in cystic fibrosis: current understandings of mechanisms and management.

Curr Gastroenterol Rep. 14: 226-235

Gastroesophageal reflux: a primary defect in cystic fibrosis?.

Scand J Gastroenterol Suppl. 143: 125-131

Gastroesophageal reflux in cystic fibrosis across the age spectrum.

Transl Gastroenterol Hepatol. 4: 69

Gastric emptying and different types of reflux in adult patients with cystic fibrosis.

Aliment Pharmacol Ther. 34: 799-807

Pharmacologic Treatment for Pediatric Gastroparesis: A Review of the Literature.

J Pediatr Pharmacol Ther. 21: 120-132

Gastroesophageal reflux (symptomatic and silent): a potentially significant problem in patients with cystic fibrosis before and after lung transplantation.

J Heart Lung Transplant. 24: 1522-1529

Gastro-oesophageal reflux and severity of pulmonary disease in cystic fibrosis.

Scand J Gastroenterol. 26: 449-456

The association of cystic fibrosis, gastroesophageal reflux, and reduced pulmonary function.

Can Assoc Radiol J. 39: 100-102Johnson L.F. Demeester T.R.

Twenty-four-hour pH monitoring of the distal esophagus. A quantitative measure of gastroesophageal reflux.

Am J Gastroenterol. 62: 325-332

Bile acids in sputum and increased airway inflammation in patients with cystic fibrosis.

Chest. 141: 1568-1574

J. Maxwell Chamberlain Memorial Paper. Early fundoplication prevents chronic allograft dysfunction in patients with gastroesophageal reflux disease.

Ann Thorac Surg. 78 (): 1142-1151Davis C.S. Jellish W.S. Fisichella P.M.

Laparoscopic fundoplication with or without pyloroplasty in patients with gastroesophageal reflux disease after lung transplantation: how I do it.

J Gastrointest Surg. 14: 1434-1441

Improved lung allograft function after fundoplication in patients with gastroesophageal reflux disease undergoing lung transplantation.

J Thorac Cardiovasc Surg. 125: 533-542

The protective role of laparoscopic antireflux surgery against aspiration of pepsin after lung transplantation.

Surgery. 150: 598-606

Antireflux surgery preserves lung function in patients with gastroesophageal reflux disease and end-stage lung disease before and after lung transplantation.

Arch Surg. 146: 1041-1047

Laparoscopic antireflux surgery in the lung transplant population.

Surg Endosc. 16: 1674-1678Fisichella P.M. Jalilvand A.

The role of impaired esophageal and gastric motility in end-stage lung diseases and after lung transplantation.

J Surg Res. 186: 201-206Van Citters G.W. Lin H.C.

Ileal brake: neuropeptidergic control of intestinal transit.

Curr Gastroenterol Rep. 8: 367-373

Disturbed gastric and small bowel transit in severe idiopathic constipation.

Dig Dis Sci. 38: 837-844

Gastric emptying, incretin hormone secretion, and postprandial glycemia in cystic fibrosis–effects of pancreatic enzyme supplementation.

J Clin Endocrinol Metab. 96: E851-E855

Gastric scintigraphy with a liquid-solid radiolabelled meal: performances of solid and liquid parameters.

Nucl Med Commun. 25: 1143-1150

Reproducibility of gastric emptying assessed with scintigraphy in patients with upper GI symptoms.

Neurogastroenterol Motil. 30: e13365

Consensus recommendations for gastric emptying scintigraphy: a joint report of the American Neurogastroenterology and Motility Society and the Society of Nuclear Medicine.

J Nucl Med Technol. 36: 44-54

Chronic intestinal pseudo-obstruction. Management with total parenteral nutrition and a venting enterostomy.

Arch Surg. 120: 614-618Rahnemai-Azar A.A. et al.

Percutaneous endoscopic gastrostomy: indications, technique, complications and management.

World J Gastroenterol. 20: 7739-7751

Domperidone to Treat Symptoms of Gastroparesis: Benefits and Side Effects from a Large Single-Center Cohort.

Dig Dis Sci. 61: 3545-3551Barney E.Z. Fuller M.E. Habib A.S.

Comparison of metoclopramide and promethazine for the treatment of postoperative nausea and vomiting in the post-anesthesia care unit: A retrospective database analysis.

J Clin Anesth. 60: 47-48

Erythromycin improves gastric emptying half-time in adult cystic fibrosis patients with gastroparesis.

J Cyst Fibros. 8: 193-197

The antibiotic azithromycin is a motilin receptor agonist in human stomach: comparison with erythromycin.

Br J Pharmacol. 168: 1859-1867

New life for macrolides.

Minerva Med. 104: 7-14

Effect of azithromycin on acid reflux, hiatus hernia and proximal acid pocket in the postprandial period.

Gut. 61: 1670-1677

Azithromycin reduces gastroesophageal reflux and aspiration in lung transplant recipients.

Dig Dis Sci. 54: 972-979

Assessment of gastric emptying using a low fat meal: establishment of international control values.

Am J Gastroenterol. 95: 1456-1462

Cystic Fibrosis & disorders of the large intestine: DIOS, constipation, and colorectal cancer.

J Cyst Fibros. ()

Bacterial overgrowth, dysbiosis, inflammation, and dysmotility in the Cystic Fibrosis intestine.

J Cyst Fibros. ()

Rate of improvement of CF life expectancy exceeds that of general population–observational death registration study.

J Cyst Fibros. 13: 410-415Rubinstein S. Moss R. Lewiston N.

Constipation and meconium ileus equivalent in patients with cystic fibrosis.

Pediatrics. 78: 473-479

Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients.

J Cyst Fibros. : S24-S28

Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristics, and treatment of DIOS.

J Pediatr Gastroenterol Nutr. 50: 38-42

Lavage treatment of distal intestinal obstruction syndrome in children with cystic fibrosis.

Pediatrics. 83: 727-733

Linaclotide improves gastrointestinal transit in cystic fibrosis mice by inhibiting sodium/hydrogen exchanger 3.

Am J Physiol Gastrointest Liver Physiol. 315 ()

Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis.

Am J Respir Crit Care Med. 190: 175-184

Sustained Benefit from ivacaftor demonstrated by combining clinical trial and cystic fibrosis patient registry data.

Am J Respir Crit Care Med. 192: 836-842

A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.

N Engl J Med. 365: 1663-1672

Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.

N Engl J Med. 373: 220-231

Assessment of safety and efficacy of long-term treatment with combination lumacaftor and ivacaftor therapy in patients with cystic fibrosis homozygous for the F508del-CFTR mutation (PROGRESS): a phase 3, extension study.

Lancet Respir Med. 5: 107-118

A Prospective Study to Evaluate Biological and Clinical Effects of Significantly Corrected CFTR Function. Available from: https://ClinicalTrials.gov/show/NCT04038047.

Twenty four hour manometric recording of colonic motor activity in healthy man.

Gut. 28: 17-25

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