Neurogastroenterology and Motility Disorders of the Gastrointestinal Tract in Cystic Fibrosis.
Constipation
Cystic Fibrosis
Gastroesophageal reflux disease
Gastrointestinal dysmotility
Gastroparesis
Small bowel dysmotility
Journal
Current gastroenterology reports
ISSN: 1534-312X
Titre abrégé: Curr Gastroenterol Rep
Pays: United States
ID NLM: 100888896
Informations de publication
Date de publication:
07 Dec 2023
07 Dec 2023
Historique:
accepted:
26
10
2023
medline:
7
12
2023
pubmed:
7
12
2023
entrez:
6
12
2023
Statut:
aheadofprint
Résumé
To discuss all the various motility disorders impacting people with Cystic Fibrosis (PwCF) and provide diagnostic and management approaches from a group of pediatric and adult CF and motility experts and physiologists with experience in the management of this disease. Gastrointestinal (GI) symptoms coexist with pulmonary symptoms in PwCF regardless of age and sex. The GI manifestations include gastroesophageal reflux disease, esophageal dysmotility gastroparesis, small bowel dysmotility, small intestinal bacterial overgrowth syndrome, distal idiopathic obstruction syndrome, constipation, and pelvic floor disorders. They are quite debilitating, limiting the patients' quality of life and affecting their nutrition and ability to socialize. This genetic disorder affects many organ systems and is chronic, potentially impacting fertility and future family planning, requiring a multidisciplinary approach. Our review discusses the treatments of motility disorders in CF, their prevalence and pathophysiology. We have provided a framework for clinicians who care for these patients that can help to guide their clinical management.
Identifiants
pubmed: 38057499
doi: 10.1007/s11894-023-00906-4
pii: 10.1007/s11894-023-00906-4
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : Cystic Fibrosis Foundation
ID : 00451A121
Informations de copyright
© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.
Références
Moshiree B, Freeman AJ, Vu PT, Khan U, Ufret-Vincenty C, Heltshe SL, et al. Multicenter prospective study showing a high gastrointestinal symptom burden in cystic fibrosis. J Cyst Fibros. 2023;22(2):266–74.
pubmed: 36319569
doi: 10.1016/j.jcf.2022.10.006
Palmer SM, Miralles AP, Howell DN, Brazer SR, Tapson VF, Davis RD. Gastroesophageal reflux as a reversible cause of allograft dysfunction after lung transplantation. Chest. 2000;118(4):1214–7.
pubmed: 11035701
doi: 10.1378/chest.118.4.1214
Sabati AA, Kempainen RR, Milla CE, Ireland M, Schwarzenberg SJ, Dunitz JM, et al. Characteristics of gastroesophageal reflux in adults with cystic fibrosis. J Cyst Fibros. 2010;9(5):365–70.
pubmed: 20674518
doi: 10.1016/j.jcf.2010.06.004
Schwarzenberg SJ, Vu PT, Skalland M, Hoffman LR, Pope C, Gelfond D, et al. Elexacaftor/tezacaftor/ivacaftor and gastrointestinal outcomes in cystic fibrosis: report of promise-GI. J Cyst Fibros. 2023;22(2):282–9.
pubmed: 36280527
doi: 10.1016/j.jcf.2022.10.003
Jaudszus A, Zeman E, Jans T, Pfeifer E, Tabori H, Arnold C, et al. Validity and reliability of a novel multimodal questionnaire for the assessment of abdominal symptoms in people with cystic fibrosis (CFAbd-Score). Patient. 2019;12(4):419–28.
pubmed: 30887269
doi: 10.1007/s40271-019-00361-2
Woodley FW, Machado RS, Hayes D Jr, Di Lorenzo C, Kaul A, Skaggs B, et al. Children with cystic fibrosis have prolonged chemical clearance of acid reflux compared to symptomatic children without cystic fibrosis. Dig Dis Sci. 2014;59(3):623–30.
pubmed: 24287640
doi: 10.1007/s10620-013-2950-0
Bongiovanni A, Manti S, Parisi GF, Papale M, Mule E, Rotolo N, et al. Focus on gastroesophageal reflux disease in patients with cystic fibrosis. World J Gastroenterol. 2020;26(41):6322–34.
pubmed: 33244195
pmcid: 7656210
doi: 10.3748/wjg.v26.i41.6322
Woodley FW, Hayes D Jr, Kopp BT, Moore-Clingenpeel M, Machado RS, Nemastil CJ, et al. Gastroesophageal reflux in cystic fibrosis across the age spectrum. Transl Gastroenterol Hepatol. 2019;4:69.
pubmed: 31620651
pmcid: 6789305
doi: 10.21037/tgh.2019.08.11
Kapnadak SG, Dimango E, Hadjiliadis D, Hempstead SE, Tallarico E, Pilewski JM, et al. Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease. J Cyst Fibros. 2020;19(3):344–54.
pubmed: 32115388
doi: 10.1016/j.jcf.2020.02.015
Rosen R, Vandenplas Y, Singendonk M, Cabana M, DiLorenzo C, Gottrand F, et al. Pediatric Gastroesophageal Reflux Clinical Practice Guidelines: Joint Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr. 2018;66(3):516–54.
pubmed: 29470322
pmcid: 5958910
doi: 10.1097/MPG.0000000000001889
Button BM, Roberts S, Kotsimbos TC, Levvey BJ, Williams TJ, Bailey M, et al. Gastroesophageal reflux (symptomatic and silent): a potentially significant problem in patients with cystic fibrosis before and after lung transplantation. J Heart Lung Transplant. 2005;24(10):1522–9.
pubmed: 16210125
doi: 10.1016/j.healun.2004.11.312
DeVault KR, Castell DO, American College of G. Updated guidelines for the diagnosis and treatment of gastroesophageal reflux disease. Am J Gastroenterol. 2005;100(1):190–200.
pubmed: 15654800
doi: 10.1111/j.1572-0241.2005.41217.x
Jobe BA, Richter JE, Hoppo T, Peters JH, Bell R, Dengler WC, et al. Preoperative diagnostic workup before antireflux surgery: an evidence and experience-based consensus of the Esophageal Diagnostic Advisory Panel. J Am Coll Surg. 2013;217(4):586–97.
pubmed: 23973101
doi: 10.1016/j.jamcollsurg.2013.05.023
Yadlapati R, Gyawali CP, Pandolfino JE, Participants CGCC. AGA clinical practice update on the personalized approach to the evaluation and management of GERD: expert review. Clin Gastroenterol Hepatol. 2022;20(5):984-94 e1.
pubmed: 35123084
pmcid: 9838103
doi: 10.1016/j.cgh.2022.01.025
Sheikh SI, Ryan-Wenger NA, McCoy KS. Outcomes of surgical management of severe GERD in patients with cystic fibrosis. Pediatr Pulmonol. 2013;48(6):556–62.
pubmed: 22949398
doi: 10.1002/ppul.22630
Sathe M, Moshiree B, Vu PT, Khan U, Heltshe SL, Romasco M, et al. Utilization of electronic patient-reported outcome measures in cystic fibrosis research: Application to the GALAXY study. J Cyst Fibros. 2021;20(4):605–11.
pubmed: 34305007
pmcid: 8403637
doi: 10.1016/j.jcf.2021.07.002
Sathe M, Moshiree B, Aliaj E, Lee M, Hudson J, Gifford A, et al. Need to study simplification of gastrointestinal medication regimen in cystic fibrosis in the era of highly effective modulators. Pediatr Pulmonol. 2023;58(3):811–8.
pubmed: 36448312
doi: 10.1002/ppul.26257
Mertens V, Blondeau K, Pauwels A, Farre R, Vanaudenaerde B, Vos R, et al. Azithromycin reduces gastroesophageal reflux and aspiration in lung transplant recipients. Dig Dis Sci. 2009;54(5):972–9.
pubmed: 19241165
doi: 10.1007/s10620-009-0725-4
Moshiree B, McDonald R, Hou W, Toskes PP. Comparison of the effect of azithromycin versus erythromycin on antroduodenal pressure profiles of patients with chronic functional gastrointestinal pain and gastroparesis. Dig Dis Sci. 2010;55(3):675–83.
pubmed: 19924535
doi: 10.1007/s10620-009-1038-3
Chini P, Toskes PP, Waseem S, Hou W, McDonald R, Moshiree B. Effect of azithromycin on small bowel motility in patients with gastrointestinal dysmotility. Scand J Gastroenterol. 2012;47(4):422–7.
pubmed: 22364597
doi: 10.3109/00365521.2012.654402
Jung HK, Choung RS, Locke GR 3rd, Schleck CD, Zinsmeister AR, Szarka LA, et al. The incidence, prevalence, and outcomes of patients with gastroparesis in Olmsted County, Minnesota, from 1996 to 2006. Gastroenterology. 2009;136(4):1225–33.
pubmed: 19249393
doi: 10.1053/j.gastro.2008.12.047
Corral JE, Dye CW, Mascarenhas MR, Barkin JS, Salathe M, Moshiree B. Is gastroparesis found more frequently in patients with cystic fibrosis? A systematic review. Scientifica (Cairo). 2016;2016:2918139.
pubmed: 27313953
Moshiree B, Potter M, Talley NJ. Epidemiology and Pathophysiology of Gastroparesis. Gastrointest Endosc Clin N Am. 2019;29(1):1–14.
pubmed: 30396519
doi: 10.1016/j.giec.2018.08.010
Sanaka M, Yamamoto T, Kuyama Y. Effects of proton pump inhibitors on gastric emptying: a systematic review. Dig Dis Sci. 2010;55(9):2431–40.
pubmed: 20012198
doi: 10.1007/s10620-009-1076-x
Grover M, Farrugia G, Lurken MS, Bernard CE, Faussone-Pellegrini MS, Smyrk TC, et al. Cellular changes in diabetic and idiopathic gastroparesis. Gastroenterology. 2011;140(5):1575-85 e8.
pubmed: 21300066
doi: 10.1053/j.gastro.2011.01.046
Murphy MS, Brunetto AL, Pearson AD, Ghatei MA, Nelson R, Eastham EJ, et al. Gut hormones and gastrointestinal motility in children with cystic fibrosis. Dig Dis Sci. 1992;37(2):187–92.
pubmed: 1735334
doi: 10.1007/BF01308170
Cassilly D, Kantor S, Knight LC, Maurer AH, Fisher RS, Semler J, et al. Gastric emptying of a non-digestible solid: assessment with simultaneous SmartPill pH and pressure capsule, antroduodenal manometry, gastric emptying scintigraphy. Neurogastroenterol Motil. 2008;20(4):311–9.
pubmed: 18194154
doi: 10.1111/j.1365-2982.2007.01061.x
Kloetzer L, Chey WD, McCallum RW, Koch KL, Wo JM, Sitrin M, et al. Motility of the antroduodenum in healthy and gastroparetics characterized by wireless motility capsule. Neurogastroenterol Motil. 2010;22(5):527–33, e117.
pubmed: 20122128
Parkman HP, Hasler WL, Fisher RS, American GA. American Gastroenterological Association technical review on the diagnosis and treatment of gastroparesis. Gastroenterology. 2004;127(5):1592–622.
pubmed: 15521026
doi: 10.1053/j.gastro.2004.09.055
Carlson DA, Gyawali CP, Khan A, Yadlapati R, Chen J, Chokshi RV, et al. Classifying esophageal motility by FLIP panometry: a study of 722 subjects with manometry. Am J Gastroenterol. 2021;116(12):2357–66.
pubmed: 34668487
pmcid: 8825704
doi: 10.14309/ajg.0000000000001532
Ng C, Dellschaft NS, Hoad CL, Marciani L, Ban L, Prayle AP, et al. Postprandial changes in gastrointestinal function and transit in cystic fibrosis assessed by Magnetic Resonance Imaging. J Cyst Fibros. 2021;20(4):591–7.
pubmed: 32561324
doi: 10.1016/j.jcf.2020.06.004
FDA. Domperidone, FDA IND 2021 [Available from: https://www.fda.gov/drugs/investigational-new-drug-ind-application/how-request-domperidone-expanded-access-use . Accessed 2021.
Pitt HA, Mann LL, Berquist WE, Ament ME, Fonkalsrud EW, DenBesten L. Chronic intestinal pseudo-obstruction. Management with total parenteral nutrition and a venting enterostomy. Arch Surg. 1985;120(5):614–8.
pubmed: 3921005
doi: 10.1001/archsurg.1985.01390290090015
Rahnemai-Azar AA, Rahnemaiazar AA, Naghshizadian R, Kurtz A, Farkas DT. Percutaneous endoscopic gastrostomy: indications, technique, complications and management. World J Gastroenterol. 2014;20(24):7739–51.
pubmed: 24976711
pmcid: 4069302
doi: 10.3748/wjg.v20.i24.7739
Wysowski DK, Bacsanyi J. Cisapride and fatal arrhythmia. N Engl J Med. 1996;335(4):290–1.
pubmed: 8657260
doi: 10.1056/NEJM199607253350416
Tillman EM, Smetana KS, Bantu L, Buckley MG. Pharmacologic treatment for pediatric gastroparesis: a review of the literature. J Pediatr Pharmacol Ther. 2016;21(2):120–32.
pubmed: 27199619
pmcid: 4869769
Soykan I, Sarosiek I, McCallum RW. The effect of chronic oral domperidone therapy on gastrointestinal symptoms, gastric emptying, and quality of life in patients with gastroparesis. Am J Gastroenterol. 1997;92(6):976–80.
pubmed: 9177513
Malik Z, Kataria R, Modayil R, Ehrlich AC, Schey R, Parkman HP, et al. Gastric per oral endoscopic myotomy (G-POEM) for the treatment of refractory gastroparesis: early experience. Dig Dis Sci. 2018;63(9):2405–12.
pubmed: 29468376
doi: 10.1007/s10620-018-4976-9
Schatz RA, Zhang Q, Lodhia N, Shuster J, Toskes PP, Moshiree B. Predisposing factors for positive D-Xylose breath test for evaluation of small intestinal bacterial overgrowth: a retrospective study of 932 patients. World J Gastroenterol. 2015;21(15):4574–82.
pubmed: 25914466
pmcid: 4402304
doi: 10.3748/wjg.v21.i15.4574
Rezaie A, Buresi M, Lembo A, Lin H, McCallum R, Rao S, et al. Hydrogen and methane-based breath testing in gastrointestinal disorders: the North American consensus. Am J Gastroenterol. 2017;112(5):775–84.
pubmed: 28323273
pmcid: 5418558
doi: 10.1038/ajg.2017.46
Tabori H, Arnold C, Jaudszus A, Mentzel HJ, Renz DM, Reinsch S, et al. Abdominal symptoms in cystic fibrosis and their relation to genotype, history, clinical and laboratory findings. PLoS ONE. 2017;12(5):e0174463.
pubmed: 28472055
pmcid: 5417419
doi: 10.1371/journal.pone.0174463
Furnari M, De Alessandri A, Cresta F, Haupt M, Bassi M, Calvi A, et al. The role of small intestinal bacterial overgrowth in cystic fibrosis: a randomized case-controlled clinical trial with rifaximin. J Gastroenterol. 2019;54(3):261–70.
pubmed: 30232597
doi: 10.1007/s00535-018-1509-4
Fridge JL, Conrad C, Gerson L, Castillo RO, Cox K. Risk factors for small bowel bacterial overgrowth in cystic fibrosis. J Pediatr Gastroenterol Nutr. 2007;44(2):212–8.
pubmed: 17255834
doi: 10.1097/MPG.0b013e31802c0ceb
Pimentel M, Saad RJ, Long MD, Rao SSC. ACG clinical guideline: small intestinal bacterial overgrowth. Am J Gastroenterol. 2020;115(2):165–78.
pubmed: 32023228
doi: 10.14309/ajg.0000000000000501
Rao SSC, Bhagatwala J. Small intestinal bacterial overgrowth: clinical features and therapeutic management. Clin Transl Gastroenterol. 2019;10(10):e00078.
pubmed: 31584459
pmcid: 6884350
doi: 10.14309/ctg.0000000000000078
Jacobs C, Coss Adame E, Attaluri A, Valestin J, Rao SS. Dysmotility and proton pump inhibitor use are independent risk factors for small intestinal bacterial and/or fungal overgrowth. Aliment Pharmacol Ther. 2013;37(11):1103–11.
pubmed: 23574267
pmcid: 3764612
doi: 10.1111/apt.12304
Burton SJ, Hachem C, Abraham JM. Luminal gastrointestinal manifestations of cystic fibrosis. Curr Gastroenterol Rep. 2021;23(3):4.
doi: 10.1007/s11894-021-00806-5
Moshiree B, Khan A, Jones ML, Singh S, Wahl C, Chuang E. S0507 SIBO diagnosis: clinical survey of practice patterns, unmet needs, and perception of a novel ingestible diagnostic capsule. Off J Am Coll Gastroenterol | ACG. 2020;115:S236–7.
doi: 10.14309/01.ajg.0000704076.84304.8a
Rao SS, Moshiree B, Lee N, Jones ML, Chuang E, Singh S. S1282 Evaluation of Smart Capsule Bacterial Detection System (SCBDS) assay and duodenal culture in subjects suspected of SIBO and undergoing upper endoscopy: interim analysis. Off J Am Coll Gastroenterol | ACG. 2020;115:S644.
doi: 10.14309/01.ajg.0000707176.78864.91
De Lisle RC, 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. 2007;293(3):G577–84.
pubmed: 17615175
doi: 10.1152/ajpgi.00195.2007
Shah SC, Day LW, Somsouk M, Sewell JL. Meta-analysis: antibiotic therapy for small intestinal bacterial overgrowth. Aliment Pharmacol Ther. 2013;38(8):925–34.
pubmed: 24004101
doi: 10.1111/apt.12479
Emmanuel AV, Shand AG, Kamm MA. Erythromycin for the treatment of chronic intestinal pseudo-obstruction: description of six cases with a positive response. Aliment Pharmacol Ther. 2004;19(6):687–94.
pubmed: 15023171
doi: 10.1111/j.1365-2036.2004.01900.x
Tomomasa T, Kuroume T, Arai H, Wakabayashi K, Itoh Z. Erythromycin induces migrating motorcomplex in human gastrointestinal tract. Dig Dis Sci. 1986;31(2):157–61.
pubmed: 3943442
doi: 10.1007/BF01300701
Reddymasu SC, Soykan I, McCallum RW. Domperidone: review of pharmacology and clinical applications in gastroenterology. Am J Gastroenterol. 2007;102(9):2036–45.
pubmed: 17488253
doi: 10.1111/j.1572-0241.2007.01255.x
Gonzalez Z, McCallum R. Small bowel dysmotility, pseudoobstruction, and functional correlation with histopathology: lessons learned. Curr Gastroenterol Rep. 2020;22(3):14.
pubmed: 32078071
doi: 10.1007/s11894-020-0748-8
Lipton AB, Knauer CM. Pseudo-obstruction of the bowel. Therapeutic trial of metoclopramide. Am J Dig Dis. 1977;22(3):263-5
Emmanuel AV, Kamm MA, Roy AJ, Kerstens R, Vandeplassche L. Randomised clinical trial: the efficacy of prucalopride in patients with chronic intestinal pseudo-obstruction–a double-blind, placebo-controlled, cross-over, multiple n = 1 study. Aliment Pharmacol Ther. 2012;35(1):48–55.
pubmed: 22061077
doi: 10.1111/j.1365-2036.2011.04907.x
Soudah HC, Hasler WL, Owyang C. Effect of octreotide on intestinal motility and bacterial overgrowth in scleroderma. N Engl J Med. 1991;325(21):1461–7.
pubmed: 1944424
doi: 10.1056/NEJM199111213252102
Verne GN, Eaker EY, Hardy E, Sninsky CA. Effect of octreotide and erythromycin on idiopathic and scleroderma-associated intestinal pseudoobstruction. Dig Dis Sci. 1995;40(9):1892–901.
pubmed: 7555439
doi: 10.1007/BF02208652
Edmunds MC, Chen JD, Soykan I, Lin Z, McCallum RW. Effect of octreotide on gastric and small bowel motility in patients with gastroparesis. Aliment Pharmacol Ther. 1998;12(2):167–74.
pubmed: 9692691
doi: 10.1046/j.1365-2036.1998.00289.x
Parthasarathy G, Ravi K, Camilleri M, Andrews C, Szarka LA, Low PA, Zinsmeister AR, Bharucha AE. Effect of neostigmine on gastroduodenal motility in patients with suspected gastrointestinal motility disorders. Neurogastroenterol Motil. 2015;27(12):1736–46.
pubmed: 26387781
pmcid: 4659742
doi: 10.1111/nmo.12669
O’Dea CJ, Brookes JH, Wattchow DA. The efficacy of treatment of patients with severe constipation or recurrent pseudo-obstruction with pyridostigmine. Colorectal Dis. 2010;12(6):540–8.
pubmed: 19508545
doi: 10.1111/j.1463-1318.2009.01838.x
Dukowicz AC, Lacy BE, Levine GM. Small intestinal bacterial overgrowth: a comprehensive review. Gastroenterol Hepatol (N Y). 2007;3(2):112–22.
pubmed: 21960820
Rubinstein S, Moss R, Lewiston N. Constipation and meconium ileus equivalent in patients with cystic fibrosis. Pediatrics. 1986;78(3):473–9.
pubmed: 3748682
doi: 10.1542/peds.78.3.473
Green J, Gilchrist FJ, Carroll W. Interventions for preventing distal intestinal obstruction syndrome (DIOS) in cystic fibrosis. Cochrane Database Syst Rev. 2018;6(6):CD012619.
pubmed: 29894558
Ferec C, Cutting GR. Assessing the disease-liability of mutations in CFTR. Cold Spring Harb Perspect Med. 2012;2(12):a009480.
pubmed: 23209179
pmcid: 3543074
doi: 10.1101/cshperspect.a009480
Patel D, Mathews S, van Unen V, Chan JE, Al-Hammadi N, Borowitz D, et al. Impaired distal colonic pH in adults with cystic fibrosis. J Cyst Fibros. 2023;22(2):290–5.
pubmed: 36572613
doi: 10.1016/j.jcf.2022.12.011
de Sillos MD, Chiba SM, Soares ACF, Gomes CET, de Morais MB. Colonic transit time and fecal impaction in children and adolescents with cystic fibrosis-associated constipation. J Pediatr Gastroenterol Nutr. 2021;73(3):319–24.
pubmed: 34128499
doi: 10.1097/MPG.0000000000003202
Hedsund C, Gregersen T, Joensson IM, Olesen HV, Krogh K. Gastrointestinal transit times and motility in patients with cystic fibrosis. Scand J Gastroenterol. 2012;47(8–9):920–6.
pubmed: 22746323
doi: 10.3109/00365521.2012.699548
Hen J Jr, Dolan TF Jr, Touloukian RJ. Meconium plug syndrome associated with cystic fibrosis and Hirschsprung’s disease. Pediatrics. 1980;66(3):466–8.
pubmed: 7437119
Esposito C, Sammarco G, De Fazio C, Alicchio F, Settimi A. Another case of cystic fibrosis complicated by meconium ileus associated with Hirschsprung’s disease: a rare and important association. Pediatr Surg Int. 2008;24(9):1069–71.
pubmed: 18696083
doi: 10.1007/s00383-008-2199-4
Stern RC, Izant RJ Jr, Boat TF, Wood RE, Matthews LW, Doershuk CF. Treatment and prognosis of rectal prolapse in cystic fibrosis. Gastroenterology. 1982;82(4):707–10.
pubmed: 7060889
doi: 10.1016/0016-5085(82)90315-8
Kulczycki LL, Shwachman H. Studies in cystic fibrosis of the pancreas; occurrence of rectal prolapse. N Engl J Med. 1958;259(9):409–12.
pubmed: 13578072
doi: 10.1056/NEJM195808282590901
El-Chammas KI, Rumman N, Goh VL, Quintero D, Goday PS. Rectal prolapse and cystic fibrosis. J Pediatr Gastroenterol Nutr. 2015;60(1):110–2.
pubmed: 25162364
doi: 10.1097/MPG.0000000000000546
Green J, Carroll W, Gilchrist FJ. Interventions for treating distal intestinal obstruction syndrome (DIOS) in cystic fibrosis. Cochrane Database Syst Rev. 2018;8(8):CD012798.
pubmed: 30075058
Colombo C, Ellemunter H, Houwen R, Munck A, Taylor C, Wilschanski M, et al. Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients. J Cyst Fibros. 2011;10(Suppl 2):S24–8.
pubmed: 21658638
doi: 10.1016/S1569-1993(11)60005-2
Taylor CJ, Aswani N. The pancreas in cystic fibrosis. Paediatr Respir Rev. 2002;3(1):77–81.
pubmed: 12065186
McHugh DR, Cotton CU, Moss FJ, Vitko M, Valerio DM, Kelley TJ, et al. Linaclotide improves gastrointestinal transit in cystic fibrosis mice by inhibiting sodium/hydrogen exchanger 3. Am J Physiol Gastrointest Liver Physiol. 2018;315(5):G868–78.
pubmed: 30118317
pmcid: 9925117
doi: 10.1152/ajpgi.00261.2017
O’Brien CE, Anderson PJ, Stowe CD. Lubiprostone for constipation in adults with cystic fibrosis: a pilot study. Ann Pharmacother. 2011;45(9):1061–6.
pubmed: 21852592
doi: 10.1345/aph.1Q219
Benninga MA, Hussain SZ, Sood MR, Nurko S, Hyman P, Clifford RA, et al. Lubiprostone for pediatric functional constipation: randomized, controlled, double-blind study with long-term extension. Clin Gastroenterol Hepatol. 2022;20(3):602-10 e5.
pubmed: 33838349
doi: 10.1016/j.cgh.2021.04.005
Rosenbaum DP, Yan A, Jacobs JW. Pharmacodynamics, safety, and tolerability of the NHE3 inhibitor tenapanor: two trials in healthy volunteers. Clin Drug Investig. 2018;38(4):341–51.
pubmed: 29363027
pmcid: 5856883
doi: 10.1007/s40261-017-0614-0
Rao SSC, Quigley EMM, Chey WD, Sharma A, Lembo AJ. Randomized Placebo-controlled phase 3 trial of vibrating capsule for chronic constipation. Gastroenterology. 2023;164(7):1202-10 e6.
pubmed: 36822371
doi: 10.1053/j.gastro.2023.02.013