Skip to main content
SpringerLink
Log in

Treatment of Gastrointestinal Manifestations in Cystic Fibrosis

  • Chapter
Pancreatic Disease

  • 85 Accesses

Abstract

Cystic fibrosis (CF) is a disease still to be discovered by clinical adult gastroenterologists, particularly in European countries, where most adult patients are currently treated in pediatric centers. Gastrointestinal manifestations of CF are very much age related: meconium ileus occurring in the newborn, consequences of exocrine pancreatic insufficiency becoming obvious during the infant period, CF liver disease appearing at school age, diabetes mellitus and adenocarcinoma being observed in adulthood (Park and Grand 1981; Shalon and Adelson 1996) (Table 1).

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  • Balistreri WF (1997) Bile acid therapy in pediatric hepatobiliary disease: the role of ursodeoxycholic acid. J Pediatr Gastroenterol Nutr 24:573–589

    Article  PubMed  CAS  Google Scholar 

  • Bittner P, Sailer T, Posselt H-G, Bender SW, Bertele-Harms RM, Arleth S, Magdorf K, Wolf A, Krawinkel M, Ott H (1990) Behandlung der Mukoviszidose mit Ursodeoxycholsäure: Ergebnisse einer kontrollierten Doppelblindstudie. Internationales Mukoviszidose-Symposium, Titisee (Germany), October 19–21, 1990, pp 1–2

    Google Scholar 

  • Bodmer MW, Angal S, Yarranton GT, Harris TJR, Lyons A, King DJ, Pieroni G, Riviere C, Verger R, Lowe PA (1987) Molecular cloning of a human gastric lipase and expression of the enzyme in yeast. Biochim Biophys Acta 909:237–244

    PubMed  CAS  Google Scholar 

  • Borowitz DS, Grand RJ, Durie PR, Consensus Committee (1995) Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy. J Pediatr 127:681–684

    Article  PubMed  CAS  Google Scholar 

  • Brady MS, Rickard K, Yu P-L, Eigen H (1991) Effectiveness and safety of small vs. large doses of enteric coated pancreatic enzymes in reducing steatorrhea in children with cystic fibrosis: a prospective randomized study. Pediatr Pulmonol 10:79–85

    Article  PubMed  CAS  Google Scholar 

  • Colombo C, Setchell KDR, Podda M, Crosignani A, Roda A, Curcio L, Ronchi M, Giunta A (1990) Effects of ursodeoxycholic acid therapy for liver disease associated with cystic fibrosis. J Pediatr 117:482–489

    Article  PubMed  CAS  Google Scholar 

  • Colombo C, Apostolo MG, Ferrari M, Seia M, Genoni S, Giunta A, Sereni LP (1994) Analysis of risk factors for the development of liver disease associated with cystic fibrosis. J Pediatr 124:393–399

    Article  PubMed  CAS  Google Scholar 

  • Cotting J, Lentze MJ, Reichen J (1990) Effects of ursodeoxycholic acid treatment on nutrition and liver function in patients with cystic fibrosis and long-standing cholestasis. Gut 31:918–921

    Article  PubMed  CAS  Google Scholar 

  • Couper RTL, Corey M, Moore DJ, Fisher LJ, Forstner GG, Durie PR (1992) Decline of exocrine pancreatic function in cystic fibrosis patients with pancreatic sufficiency. Pediatr Res 32:179–182

    Article  PubMed  CAS  Google Scholar 

  • Crisci KL, Greenberg SB, Wolfson BJ, Geller E, Vinocur CD (1997) Contrast enema findings of fibrosing colonopathy. Pediatr Radiol 27:315–316

    Article  PubMed  CAS  Google Scholar 

  • Delaney SJ, Wainwright BJ (1996) New pharmaceutical approaches to the treatment of cystic fibrosis. Nat Med 2:392–393

    Article  PubMed  CAS  Google Scholar 

  • Dodge JA, Macpherson C (1996) Colonic strictures in cystic fibrosis. J R Soc Med 88[Suppl 25]:3–8

    Google Scholar 

  • Feigelson J, Anagnostopoulos C, Poquet M, Pecau Y, Munck A, Navarro J (1993) Liver cirrhosis in cystic fibrosis — therapeutic implications and long-term follow-up. Arch Dis Child 68:653–657

    Article  PubMed  CAS  Google Scholar 

  • Figarella C (ed) (1996) Advances in cystic fibrosis: gastrointestinal and nutritional aspects. Eur J Gastroenterol Hepatol 8:729

    Google Scholar 

  • FitzSimmons SC, Burkhart GA, Borowitz D, Grand RJ, Hammerstrom T, Durie PR, Lloyd-Still JD, Lowenfels AB (1997) High-dose pancreatic-enzyme supplements and fibrosing colonopathy in children with cystic fibrosis. N Engl J Med 336:1283–1289

    Article  PubMed  CAS  Google Scholar 

  • Galabert C, Montet JC, Lengrand D, Lecuire A, Sotta C, Figarella C, Chazalette JP (1992) Effects of ursodeoxycholic acid on liver function in patients with cystic fibrosis and chronic cholestasis. J Pediatr 121:138–141

    Article  PubMed  CAS  Google Scholar 

  • Grand RJ (1996) Gastrointestinal manifestations of cystic fibrosis. Gastrointestinal Dis Today 5:8–13

    Google Scholar 

  • Gregory PC (1996) Gastrointestinal pH, motility/transit and permeability in cystic fibrosis. J Pediatr Gastroenterol Nutr 23:513–523

    Article  PubMed  CAS  Google Scholar 

  • Grubman SA, Fang SL, Mulberg AE, Perrone RD, Rogers LC, Lee DW, Armentano D, Murray SL, Dorkin HL, Cheng SH, Smith AE, Jefferson DM (1995) Correction of the cystic fibrosis defect by gene complementation in human intrahepatic biliary epithelial cell lines. Gastroenterology 108:584–592

    Article  PubMed  CAS  Google Scholar 

  • Guarner L, Rodríguez R, Guarner F, Malagelada J-R (1993) Fate of oral enzymes in pancreatic insufficiency. Gut 34:708–712

    Article  PubMed  CAS  Google Scholar 

  • Haber HP, Benda N, Fitzke G, Lang A, Langenberg M, Riethmüller J, Stern M (1997) Colonic wall thickness measured by ultrasound: striking differences in patients with cystic fibrosis versus healthy controls. Gut 40:406–411

    PubMed  CAS  Google Scholar 

  • Klima LD, Kowdley KV, Lewis SL, Wood DE, Aitken ML (1997) Successful lung transplantation in spite of cystic fibrosis-associated liver disease: a case series. J Heart Lung Transplant 16:934–938

    PubMed  CAS  Google Scholar 

  • Koletzko S (1997) Pankreasbeteiligung bei Cystischer Fibrose. Z Gastroenterol [Suppl 1]:143–159

    Google Scholar 

  • Lancellotti L, Cabrini G, Zanolla L, Mastella G (1996) High-versus low-lipase acid-resistant enzyme preparations in cystic fibrosis: a crossover randomized clinical trial. J Pediatr Gastroenterol Nutr 22:73–78

    Article  PubMed  CAS  Google Scholar 

  • Lepage G, Paradis K, Lacaille F, Senechal L, Ronco N, Champagne J, Lenaerts C, Roy CC, Rasquin-Weber A (1997) Ursodeoxycholic acid improves the hepatic metabolism of essential fatty acids and retinol in children with cystic fibrosis. J Pediatr 130:52–58

    Article  PubMed  CAS  Google Scholar 

  • Lindblad A, Glaumann H, Strandvik B (1998) A two-year prospective study of the effect of ursodeoxycholic acid on urinary bile acid excretion and liver morphology in cystic fibrosis-associated liver disease. Hepatology 27:166–174

    Article  PubMed  CAS  Google Scholar 

  • Littlewood JM, Hind CRK (eds) (1996) Fibrosing colonopathy in children with cystic fibrosis. Postgrad Med J 72[Suppl 2]:S2–S64

    Google Scholar 

  • Löser C, Möllgaard A, Fölsch UR (1996) Faecal elastase 1: a novel, highly sensitive, and specific tubeless pancreatic function test. Gut 39:580–586

    Article  PubMed  Google Scholar 

  • Lowdon J, Goodchild MC, Ryley HC, Doull IJM (1998) Maintenance of growth in cystic fibrosis despite reduction in pancreatic enzyme supplementation. Arch Dis Child 78:377–378

    Article  PubMed  CAS  Google Scholar 

  • Mack DR, Traystman MD, Colombo JL, Sammut PH, Kaufman SS, Vanderhoof JA, Antonson DL, Markin RS, Shaw BW, Langnas AN (1995) Clinical denouement and mutation analysis of patients with cystic fibrosis undergoing liver transplantation for biliary cirrhosis. J Pediatr 127:881–887

    Article  PubMed  CAS  Google Scholar 

  • Mac Sweeney EJ, Oades PJ, Buchdahl R, Rosenthal M, Bush A (1995) Relation of thickening of colon wall to pancreatic-enzyme treatment in cystic fibrosis. Lancet 345:752–756

    Article  PubMed  CAS  Google Scholar 

  • Maeda H, Danel C, Crystal RG (1994) Adenovirus-mediated transfer of human lipase complementary DNA to the gallbladder. Gastroenterology 106:1638–1644

    PubMed  CAS  Google Scholar 

  • O’Brien S, Fitzgerald MX, Hegarty JE (1992) A controlled trial of ursodeoxycholic acid treatment in cystic fibrosis-related liver disease. Eur J Gastroenterol Hepatol 4:857–863

    Google Scholar 

  • O’Hare MMT, McMaster C, Dodge JA (1995) Stated versus actual lipase activity in pancreatic enzyme supplements: implications for clinical use. J Pediatr Gastroenterol Nutr 21:59–63

    Article  PubMed  Google Scholar 

  • Park RW, Grand, RJ (1981) Gastrointestinal manifestations of cystic fibrosis: a review. Gastroenterology 81:1143–1161

    PubMed  CAS  Google Scholar 

  • Pawel BR, de Chadarevian JP, Franco ME (1997) The pathology of fibrosing colonopathy of cystic fibrosis: a study of 12 cases and review of the literature. Hum Pathol 28:395–399

    Article  PubMed  CAS  Google Scholar 

  • Pickhardt PJ, Yagan N, Siegel MJ, Balfe DM, Rothbaum RJ (1998) Cystic fibrosis: CT findings of colonic disease. Radiology 206:725–730

    PubMed  CAS  Google Scholar 

  • Pohl M, Krackhardt B, Posselt HG, Lembcke B (1997) Ultrasound studies of the intestinal wall in patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 25:317–320

    Article  PubMed  CAS  Google Scholar 

  • Raimondo M, DiMagno EP (1994) Lipolytic activity of bacterial lipase survives better than that of porcine lipase in human gastric and duodenal content. Gastroenterology 107:231–235

    PubMed  CAS  Google Scholar 

  • Ramsey BW, Farrell PM, Pencharz P, Consensus Committee (1992) Nutritional assessment and management in cystic fibrosis: a consensus report. Am J Clin Nutr 55:108–116

    PubMed  CAS  Google Scholar 

  • Regele S, Henker J, Münch R, Barbier Y, Stern M (1996) Indirect parameters of pancreatic function in cystic fibrosis (CF) during a controlled double-blind trial of pancreatic supplementation. J Pediatr Gastroenterol Nutr 22:68–72

    Article  PubMed  CAS  Google Scholar 

  • Reichard KW, Vinocur CD, Franco M, Crisci KL, Flick JA, Billmire DF, Schidlow DV, Weintraub WH (1997) Fibrosing colonopathy in children with cystic fibrosis. J Pediatr Surg 32:237–241

    Article  PubMed  CAS  Google Scholar 

  • Scott-Jupp R, Lama M, Tanner MS (1991) Prevalence of liver disease in cystic fibrosis. Arch Dis Child 66:698–701

    Article  PubMed  CAS  Google Scholar 

  • Shalon LB, Adelson JW (1996) Cystic fibrosis: gastrointestinal complications and gene therapy. Pediatr Clin North Am 43:157–196

    Article  PubMed  CAS  Google Scholar 

  • Shwachman H (1975) Gastrointestinal manifestations of cystic fibrosis. Pediatr Clin North Am 22:787–805

    PubMed  CAS  Google Scholar 

  • Sinaasappel M (1995) Present and future treatment modalities for gastrointestinal diseases in cystic fibrosis. Neth J Med 46:275–279

    Article  PubMed  CAS  Google Scholar 

  • Smyth RL, Ashby D, O’Hea U, Burrows E, Lewis P, van Velzen D, Dodge JA (1995) Fibrosing colonopathy in cystic fibrosis: results of a case-control study. Lancet 346:1247–1251

    Article  PubMed  CAS  Google Scholar 

  • Soldan W, Henker J, Sprössig C (1997) Sensitivity and specificity of quantitative determination of pancreatic elastase 1 in feces of children. J Pediatr Gastroenterol Nutr 24:53–55

    Article  PubMed  CAS  Google Scholar 

  • Stevens JC, Maguiness KM, Hollingsworth J, Heilman DK, Chong SK (1998) Pancreatic enzyme supplementation in cystic fibrosis patients before and after fibrosing colonopathy. J Pediatr Gastroenterol Nutr 26:80–84

    Article  PubMed  CAS  Google Scholar 

  • Strong TV, Boehm K, Collins FS (1994) Localization of cystic fibrosis transmembrane conductance regulator mRNA in the human gastrointestinal tract by in situ hybridization. J Clin Invest 93:347–354

    Article  PubMed  CAS  Google Scholar 

  • Suzuki A, Mizumoto A, Sarr MG, DiMagno P (1997) Bacterial lipase and high-fat diets in canine exocrine pancreatic insufficiency: a new therapy of steatorrhea? Gastroenterology 112:2048–2055

    Article  PubMed  CAS  Google Scholar 

  • Van de Meeberg PC, Houwen RHJ, Sinaasappel M, Heijerman HGM, Bijleveld CMA, van Berge Henegouwen GP (1997) Low-dose versus high-dose ursodeoxycholic acid in cystic fibrosis-related cholestatic liver disease. Scand J Gastroenterol 32:369–373

    Article  PubMed  Google Scholar 

  • Zentler-Munro PL, Assoufi BA, Balasubramanian K, Cornell S, Benoliel D, Northfield TC, Hodson ME (1992) Therapeutic potential and clinical efficacy of acid-resistant fungal lipase in the treatment of pancreatic steatorrhoea due to cystic fibrosis. Pancreas 7:311–319

    Article  PubMed  CAS  Google Scholar 

Download references

Authors
  1. M. Stern
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

Copyright information

© 1999 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Stern, M. (1999). Treatment of Gastrointestinal Manifestations in Cystic Fibrosis. In: Pancreatic Disease. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-60068-5_18

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-60068-5_18

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-65357-8

  • Online ISBN: 978-3-642-60068-5

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation