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European Journal of Pediatrics

, Volume 150, Issue 12, pp 824–828 | Cite as

Small-intestinal abnormalities in cystic fibrosis patients

  • E. Eggermont
  • K. De Boeck
Review

Abstract

A survey is given of the pathophysiology of the main alterations in the small intestine of cystic fibrosis patients. Special attention is paid to the understanding of meconium ileus in the fetus and the newborn, the repercussion of duodenal acidity on the duodenal mucosa and the intraduodenal digestion, and primary and secondary biochemical alterations in the secretory-digestive-absorptive function of the small-intestinal mucosa. The meconium equivalent syndrome and its connection with the atypical course of intussusception and appendiceal perforation with silent pelvic abscess are also discussed.

Key words

Absorption Alkaline phosphatase Cystic fibrosis Digestion Disaccharidase 

Abbreviations

CF

cystic fibrosis

CFTR

cystic fibrosis transmembrane conductance regulator

mRNA

messenger ribonucleic acid

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References

  1. 1.
    Adrian TE, McKiernan J, Johnstone DI, Hiller EJ, Vyas H, Sarson DI, Bloom SR (1980) Hormonal abnormalities of the pancreas and gut in cystic fibrosis. Gastroenterology 79:460–465PubMedGoogle Scholar
  2. 2.
    Ahmed F, Ellis J, Murphy J, Wootton S, Jackson AA (1990) Excessive faecal loss of vitamin A (retinol) in cystic fibrosis. Arch Dis Child 65:589–593PubMedGoogle Scholar
  3. 3.
    Anderson MP, Welsh MJ (1990) c-AMP and calcium activate different apical chloride channels in airway and intestinal epithelia. Poster presentation at the 1990 North American Cystic Fibrosis Conference, Arlington, Virginia, 3–6 OctoberGoogle Scholar
  4. 4.
    Antonowicz I, Shwachman H (1979) Meconium in health and in disease. Adv Pediatr 26:275–310PubMedGoogle Scholar
  5. 5.
    Antonowicz I, Lebenthal E, Shwachman H (1976) Disaccharidase activities in pancreatic insufficiency and reappraisal of lactase activity in cystic fibrosis. Pediatr Res 10:351Google Scholar
  6. 6.
    Baxter R, Goldhill J, Hardcastle J, Hardcastle PT, Taylor CJ (1990) Enhanced intestinal glucose and alanine transport in cystic fibrosis. Gut 31:817–820PubMedGoogle Scholar
  7. 7.
    Bisset WM (1988) The development of motor control systems in the gastrointestinal tract of the preterm infant. In: Milla PJ (ed) Disorders of gastrointestinal motility in childhood. John Wiley and Sons, Chichester, pp 17–27Google Scholar
  8. 8.
    Brock DJH, Bedgood D, Hayward C (1984) Prenatal diagnosis of cystic fibrosis by assay of amniotic fluid microvillar enzymes. Hum Genet 65:248–251CrossRefPubMedGoogle Scholar
  9. 9.
    Claass AHW, Kleijer WJ, Van Diggelen OP, Van der Veer E, Sips HJ (1986) Prenatal detection of cystic fibrosis: comparative study of maltase and alkaline phosphatase activities in amniotic fluid. Prenat Diagn 6:419–427PubMedGoogle Scholar
  10. 10.
    Claus D, Pringot J, Francois B, De Meyer R (1973) The intestinal radiological abnormalities of cystic fibrosis. Ann Radiol 16:177–181Google Scholar
  11. 11.
    Cox K, Isenberg JN (1978) Hypersecretion of gastric acid in patients with pancreatic exocrine insufficiency due to cystic fibrosis. Gastroenterology 74:1022Google Scholar
  12. 12.
    Dalzell AM, Freestone NS, Billington D, Heaf DP (1990) Small intestinal permeability and orocaecal transit time in cystic fibrosis. Arch Dis Child 65:585–588PubMedGoogle Scholar
  13. 13.
    De Jonge HR (1989) The molecular basis of chloride channel dysregulation in cystic fibrosis. Acta Paediatr Scand [Suppl] 363:14–19Google Scholar
  14. 14.
    De Jonge HR, Tilly BC, Van Gageldonk PGM, Veeze HJ, Sinaasappel M, Hansen M, Bijman J, Keulemans J, Hoogeveen AT (1990) Expression of the CF defect in intestinal epithelium. Pediatr Pulmonol [Suppl] 5:217Google Scholar
  15. 15.
    Drumm ML, Pope HA, Cliff WH, Rommens JM, Marvin SA, Tsui LC, Collins FS, Frizzell RA, Wilson JM (1990) Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer. Cell 62:1227–1233CrossRefPubMedGoogle Scholar
  16. 16.
    Durie PR (1988) Gastrointestinal motility disorders in cystic fibrosis. In: Milla PJ (ed) Disorders of gastrointestinal motility in childhood. John Wiley and Sons, Chichester, pp 91–99Google Scholar
  17. 17.
    Durie PR (1990) Small intestinal motility in cystic fibrosis. Pediatr Pulmonol [Suppl] 5:101–102Google Scholar
  18. 18.
    Eggermont E (1966) Enzymic activities in meconium from human foetuses and newborn. Biol Neonat 10:266–280PubMedGoogle Scholar
  19. 19.
    Eggermont E (1985) The role of the small intestine in cystic fibrosis patients. Acta Paediatr Scand [Suppl] 317:16–21Google Scholar
  20. 20.
    Eggermont E (1988) Pancreatic function testing in meconium disease in cystic fibrosis. J Pediatr Gastroenterol Nutr 7:435Google Scholar
  21. 21.
    Eggermont E, Rutgeerts L (1974) Enterokinase activity in the duodenal content of CF-patients. Abstracts Fifth Annual Meeting of the European Working Group for Cystic Fibrosis, Verona, Italy, 22–23 April, European Working Group for Cystic Fibrosis, VeronaGoogle Scholar
  22. 22.
    Flick JA, Perman JA (1990) Increased intestinal permeability in cystic fibrosis correlates with the degree of pancreatic exocrine dysfunction. Pediatr Pulmonol [Suppl] 5:97–98Google Scholar
  23. 23.
    Frase LL, Strickland AD, Kachel GW, Krejs GJ (1985) Enhanced glucose absorption in the jejunum of patients with cystic fibrosis. Gastroenterology 88:478–484PubMedGoogle Scholar
  24. 24.
    Freye HB, Kurtz SM, Spock A, Capp MP (1964) Light and electron microscopic examination of the small bowel of children with cystic fibrosis. J Pediatr 64:575–579PubMedGoogle Scholar
  25. 25.
    Gilbert J, Kelleher J, Littlewood JM, Evans DF (1988) Ileal pH in cystic fibrosis. Scand J Gastroenterol 23 [Suppl] 143:132–134Google Scholar
  26. 26.
    Gill JR, Bartter FC (1978) Evidence for a prostaglandin-independent defect in chloride reabsorption in the loop of Henle as a proximal cause of Bartter syndrome. Am J Med 65:766–772CrossRefPubMedGoogle Scholar
  27. 27.
    Gosden CM, Gosden JR (1984) Fetal abnormalities in cystic fibrosis suggest a deficiency in proteolysis of cholecystokinin. Lancet II:541–546CrossRefGoogle Scholar
  28. 28.
    Hadorn B, Johansen PG, Anderson CM (1968) Pancreozymin secretion test of exocrine pancreatic function in cystic fibrosis and the significance of the result for the pathogenesis of the disease. Can Med Ass J 98:377–385PubMedGoogle Scholar
  29. 29.
    Hardcastle J, Hardcastle PT, Taylor CJ (1990) Ion transport abnormalities in the intestine in cystic fibrosis. Pediatr Pulmonol [Suppl] 5:95–97Google Scholar
  30. 30.
    Hoogeveen AT, Keulemans J, Willemsen R, Bijman J, Scholte BJ, Galgaard H, Edixhoven MJ, De Jonge HR (1990) Immunological characterization of 155 kDa and 195 kDa proteins as candidates for the cystic fibrosis gene product. Pediatr Pulmonol [Suppl] 5:190Google Scholar
  31. 31.
    Kelleher J, Littlewood JM, Evans DF, Pye G (1990) Small bowel pH profiles in cystic fibrosis. Pediatr Pulmonol [Suppl] 5:270Google Scholar
  32. 32.
    Launiala K, Perheentupa J, Pasternack A, Hallman N (1968) Familial chloride diarrhoea — chloride malabsorption. Mod Probl Paediatr 11:137–149Google Scholar
  33. 33.
    Lebenthal E, Hatch TF, Lee PC (1981) Development of disaccharidases in premature, small for gestational age and full term infants. In: Lebenthal E (ed) Gastroenterology and nutrition in infancy. Raven Press, New York, pp 413–422Google Scholar
  34. 34.
    Leclercq-Foucart J, Forget P, Sodroyez-Goffaux F, Zappitelli A (1986) Intestinal permeability to [51Cr] EDTA in children with cystic fibrosis. J Pediatr Gastroenterol Nutr 5:384–387PubMedGoogle Scholar
  35. 35.
    Leclercq-Foucart J, Forget PP, Van Cutsem JL (1987) Lactulose-rhamnose intestinal permeability in children with cystic fibrosis. J Pediatr Gastroenterol Nutr 6:66–70PubMedGoogle Scholar
  36. 36.
    Lloyd-Still JD (1990) Cystic fibrosis (CF), Crohn disease (CD), biliary abnormalities and cancer. Pediatr Pulmonol [Suppl] 5:270Google Scholar
  37. 37.
    Mantle M, Stewart G (1989) Intestinal mucins from normal subjects and patients with cystic fibrosis. Biochem J 259:243–253PubMedGoogle Scholar
  38. 38.
    Martens M, De Boeck K, Van der Steen K, Smet M, Eggermont E (1991) A right lower quadrant mass in cystic fibrosis: a diagnostic challenge. Eur J Pediatr (in press)Google Scholar
  39. 39.
    Matseshe JW, Go ULW, Dimagno EP (1977) Meconium ileus equivalent complicating cystic fibrosis in postneonatal children and young adults. Gastroenterology 72:732–736PubMedGoogle Scholar
  40. 40.
    Montrose-Rafizadeh C, Guggino WB, Montrose MH (1990) Expression of CFTR is modulated by cell differentiation in a cloned human intestinal cell line. Pediatr Pulmonol [Suppl] 5:192Google Scholar
  41. 41.
    Muller F, Aubry MC, Gasser B, Duchatel F, Boue J, Boue A (1985) Prenatal diagnosis of cystic fibrosis. II Meconium ileus in affected fetures. Prenat Diagn 5:109–117PubMedGoogle Scholar
  42. 42.
    Park RW, Grand RJ (1981) Gastrointestinal manifestations of cystic fibrosis — a review. Gastroenterology 81:1143–1161PubMedGoogle Scholar
  43. 43.
    Pecau Y, Feigelson J (1975) Une anomalie coprologique dans la mucoviscidose. Arch Fr Pediatr 32:733–740PubMedGoogle Scholar
  44. 44.
    Ringe D, Petsko GA (1990) Cystic fibrosis, a transport problem? Nature 346:312–313CrossRefPubMedGoogle Scholar
  45. 45.
    Riordan JR, Rommens JM, Kerem B, Alon N, Rozmahel R, Grzelczak Z, Zielenski J, Lok S, Plavsic N, Chou JL, Drumm ML, Iannuzzi MC, Collins FS, Tsui LC (1989) Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245:1066–1073PubMedGoogle Scholar
  46. 46.
    Shwachman H, Antonowicz I (1981) Studies on meconium. In: Lebenthal E (ed) Gastroenterology and nutrition in infancy. Raven Press, New York, pp 83–93Google Scholar
  47. 47.
    Sood R, Auerbach W, Shannon W, Buchwald M (1990) Regulation of expression of CFTR in vitro differentiation of human intestinal epithelial cells. Pediatr Pulmonol [Suppl] 5:194Google Scholar
  48. 48.
    Van Diggelen OP, Janse HC, Kleijer WJ (1983) Disaccharidases in amniotic fluid as possible prenatal marker for cystic fibrosis. Lancet I:817CrossRefGoogle Scholar
  49. 49.
    Wesley A, Forstner JF, Qureshi R, Mantle M, Forstner GG (1983) Human intestinal mucin in cystic fibrosis. Pediatr Res 17:65–69PubMedGoogle Scholar
  50. 50.
    Wulkan RW, Sinaasappel M, Bouquet J, Lindemans J (1990) Alpha-1-antitrypsin clearance in children with cystic fibrosis. Pediatr Pulmonol [Suppl] 5:272Google Scholar
  51. 51.
    Youngberg CA, Berardi RR, Howatt WF, Hyneck ML, Amidon GL, Meyer JH, Dressman JB (1987) Comparison of gastrointestinal pH in cystic fibrosis and healthy subjects. Dig Dis Sci 32:472–480CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag 1991

Authors and Affiliations

  • E. Eggermont
    • 1
  • K. De Boeck
    • 1
  1. 1.Department of PaediatricsUniversity Hospital GasthuisbergLeuvenBelgium

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