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Pankreasinsuffizienz bei Mukoviszidose und metabolische Komplikationen — ein diagnostisch vielschichtiges Problem

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Zusammenfassung

Die cystische Fibrose (CF) oder Mukoviszidose ist eine autosomal rezessiv vererbte Multiorganerkrankung. Sie ist die häufigste Ursache einer exokrinen Pankreasinsuffizienz im Kindes- und Jugendalter. Ca. 85–90% aller CF-Patienten sind pankreasinsuffizient und bedürfen einer oralen Substitution mit Pankreasenzymen [62]. Neben dem Pankreas sind alle exokrinen Drüsen und Organe betroffen, die durch Epithelzellen ausgekleidet sind. Im Gegensatz zur Lungenbeteiligung beginnt die Pankreaserkrankung meistens bereits in utero. So sind es im jungen Kindesalter meistens die Zeichen der Malassimilation mit ihren Folgen, die eine CF vermuten lassen und zur Diagnose führen.

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Literatur

  1. Abman, SH, Reardon MC, Accurso FJ, Hammond KB, Sokol RJ (1985) Hypoalbuminemia at diagnosis as a marker for severe respiratory course in infants with cystic fibrosis identified by newborn screening. Pediatr. 107: 933–935

    Article  CAS  Google Scholar 

  2. Benini L, Caliari S, Guidi GC et al. (1989) Near infrared spectroscopy for fecal measurement: comparison with conventional gravimetric and titrimeteric methods. Gut 30: 1344–1347

    Article  PubMed  CAS  Google Scholar 

  3. Benini L, Caliari S, Bonafante F et al. (1992) Near infrared reflectance measurement of nitrogen faecal losses. Gut. 33: 749–752

    Article  PubMed  CAS  Google Scholar 

  4. Berschneider HM, Knowles MR, Azizkhan RG et al. (1988) Altered intestinal chloride transport in cystic fibrosis. FASEB J 2: 2625–2629

    CAS  Google Scholar 

  5. Bines J, Jacobowitz Israel EE (1991) Hypoproteinemia, anemia, and failure to thrive in an infant. Gastroenterol 101: 848–856

    CAS  Google Scholar 

  6. Bonin A, Roy CC, Lasalle R, Weber A, Morin CL (1973) Fecal chymotrypsin: a reliable index of exocrine pancreatic function in children. J Pediatr 83: 594–600

    Article  PubMed  CAS  Google Scholar 

  7. Bronstein MN, Sokol RJ, Abman SH, Chatfield BA, Hammond KB, Hambidge KM, Stall CD, Accurso FJ (1992) Pancreatic insufficiency, growth, and nutrition in infants identified by newborn screening as having cystic fibrosis. J Pediatr 120: 533–540

    Article  PubMed  CAS  Google Scholar 

  8. Cleghorn G, Benjamin L, Corey M, Forstner G, Dati F, Durie P (1985) Age-related alterations of immunoreactive pancreatic lipase and cationic trypsinogen in young children with cystic fibrosis. J Pediatr 107: 377–381

    Article  PubMed  CAS  Google Scholar 

  9. Cleghorn G, Benjamin L, Corey M, Forstner G, Dati F, Durie P (1986) Serum immunore- active pancreatic lipase and cationic tripsinogen for the assessment of exocrine pancreatic function in older patients with cystic fibrosis. Pediatrics. 77: 301–306

    PubMed  CAS  Google Scholar 

  10. Corey M, Gaskin K, Durie P, Levison H, Forstner G (1984) Improved prognosis in CF patients with normal fat absorption. J Pediatr Gastroenterol Nutr 3 (suppl): 99–105

    Article  Google Scholar 

  11. Couper R; Durie PR (1991) Pancreatic function tests. In: Walker WA, Durie PR, Hamilton JR, Walker-Smith JA, Watkins JB. Pediatric Gastrointestinal Disease. Philadelphia, Toronto: Decker, B.C. pp. 1341 - 1353.

    Google Scholar 

  12. 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 

  13. Crossley JR, Smith PA, Edgar PW, Gluckman PD, Elliott RB (1981) Neonatal screening for cystic fibrosis, using immunoreactive trypsin assay in dried blood spots. Clin Chim Acta 113: 111–121

    Article  PubMed  CAS  Google Scholar 

  14. Culler FL, Mckean LP, Buchanan CN, Caplan DB, Meacham LR (1994) Short Communication: Glipizide treatment of patients with cystic fibrosis and impaired glucose tolerance. J Pediatr Gastroenterol. Nutr. 18: 375–378

    Article  PubMed  CAS  Google Scholar 

  15. Cumming JGR, Forsyth JS, Boyd EJS, Frost GJ, Cuschieri A (1986) Diagnosis of exocrine insufficiency in cystic fibrosis by use of fluorescein dilaurate test. Arch. Dis. Child. 61: 573–575

    Article  PubMed  CAS  Google Scholar 

  16. Delchier JC, Vidon N, Girardin MFS-M, Soule JC, Moulin C, Huchet B, Zylberberg P (1991) Fate of orally ingested enzymes in pancreatic insufficiency: comparison of two pancreatic enzyme preparations. Aliment Pharmacol Therap 5: 365–378

    Article  CAS  Google Scholar 

  17. Dewit O, Prentice A, Coward A, Weaver LT (1992) Starch digestion in young children with cystic fibrosis measured using a 13C breath test. Pediatr. Res. 32: 45–49

    Article  PubMed  CAS  Google Scholar 

  18. Durie PR, Bell L, Linton W, Corey ML, Forstner GG (1980) Effect of cimetidine and sodium bicarbonate on pancreatic replacement therapy in cystic fibrosis. Gut 21: 778 - 786

    Article  PubMed  CAS  Google Scholar 

  19. Durie PR, Forstner GG, Gaskin KJ, Moore DJ, Cleghorn GJ, Wong SS, Corey ML (1986) Age-related alternations of immunoreactive pancreatic cationic tripsinogen in sera from cystic fibrosis patients with and without pancreatic insufficiency. Pediatr Res 20: 209–213

    Article  PubMed  CAS  Google Scholar 

  20. Durie PR, Forstner CG (1989) Pathophysiology of the exocrine pancreas in cystic fibrosis. J. Roy. Soc. Med. 16: 2–10

    Google Scholar 

  21. Durie PR, Goldberg DM (1986) Biochemical tests of pancreatic function in infancy and childhood. Adv. Clin. Enzymol. 4: 77–92

    Google Scholar 

  22. Forget MT, Van Den Neucker A, Strik J, Van Kreel B, Kuijten R (1994) The acid steatocrit: a much improved method. J Pediatr Gastroenterol Nutr 19: 229–303

    Google Scholar 

  23. Forstner G, Gall G, Corey M, Durie P, Hill R, Gaskin K (1980) Digestion and absorption of nutrients in cystic fibrosis. In: Sturgess JM (ed) Perspectives in cystic fibrosis. Toronto: Imperial Press, pp. 137–149.

    Google Scholar 

  24. Forstner G, Durie PR (1991) Cystic fibrosis. In: Walker WA, Durie PR, Hamilton JR, Walker-Smith J A, Watkins JB. Pediatric gastrointestinal disease. Philadelphia, Toronto: Dekker, BC pp 1179–1197

    Google Scholar 

  25. Frizzell RA (1987) Cystic fibrosis: A disease of ion channels? Trends Neurosci. 10: 190–193

    Article  CAS  Google Scholar 

  26. Gaskin K, Gurwitz D, Durie P, Corey M, Levison H, Forstner G (1982) Improved respiratory prognosis in CF patients with normal fat absorption. J Pediatr 100: 857–862

    Article  PubMed  CAS  Google Scholar 

  27. Gaskin K, Waters D, Dorney S, Gruca M, O’halloran M, Wilcken B (1991) Assessment of pancreatic function in screened infants with cystic fibrosis. Pediatr. Pulmonol Suppl 7: 69–71

    Article  PubMed  CAS  Google Scholar 

  28. Gaskin KJ, Durie P, Hill RE, Lee LM, Forstner GG (1982) Colipase and maximally activated pancreatic lipase in normal subjects and patients with steatorhea. J Clin Invest 69: 427–434

    Article  PubMed  CAS  Google Scholar 

  29. Gaskin KJ, Durie P, Lee L, Forstner GG (1984) Colipase and lipase secretion in childhood onset of pancreatic insufficiency: delineation of patients with steatorrhea with relative colipase deficiency. Gastroenterol 86: 1–7

    CAS  Google Scholar 

  30. Geffner ME, Lippe BM, Maclaren NK, Riley WJ (1988) Role of autoimmunity in insulinopenia and carbohydrate derangements associated with cystic fibrosis. J Pediatr 112: 419–421

    Article  PubMed  CAS  Google Scholar 

  31. Greer R, Shepherd R, Cleghorn G, Bowling FG, Holt T (1991) Evaluation of growth and changes in body composition following neonatal diagnosis of cystic fibrosis. J Pediatr Gastroenterol Nutr 13: 52–58

    Article  PubMed  CAS  Google Scholar 

  32. Gross V, Schoelmerich J, Denzel K, Gerok W (1989) Case report: Relapsing pancreatitis as initial manifestation of cystic fibrosis in a young man without pulmonary disease. Int J Pancreatol 4: 221–228

    PubMed  CAS  Google Scholar 

  33. Guarner L, Rodriguez R, Malagelada J (1993) Fate of oral enzymes in pancreatic insufficiency. Gut 34: 708–712

    Article  PubMed  CAS  Google Scholar 

  34. Hadorn B, Johansen PG, Anderson CM (1968) Pancreozymin secretin test of exocrine pancreatic function in cystic fibrosis and the significance of the result for the pathogenesis of the disease. Canad Med Ass J 98: 377–385

    CAS  Google Scholar 

  35. Heijerman HG, Lamers CB, Bakker W (1991) Omeprazole enhances the efficacy of pancreatin (pancrease) in cystic fibrosis. Ann Intern Med 114: 200–201

    PubMed  CAS  Google Scholar 

  36. Iacono G, Carroccio A, Cavataio F, Montalto G, Mancuso C, Balsamo V, Notarbartolo A (1990) Steatocrit test: normal range and physiological variations in infants. J Pediatr Gastroenterol. Nutr. 11: 53–57

    Article  PubMed  CAS  Google Scholar 

  37. Imrie J, Fagan D, Sturgess J (1979) Quantitative evaluation of the development of the exocrine pancreas in cystic fibrosis and control subjects. Am J Pathol 95: 697–708

    PubMed  CAS  Google Scholar 

  38. Jeejeebhoy KN, Ahmed S, Kozak G (1970) Determination of fecal fats containing both medium and long chain triglycerides and fatty acids. Clin Biochem 3: 157–163

    PubMed  CAS  Google Scholar 

  39. Kane RE, Black P (1989) Glucose intolerance with low-, medium-, and high-carbohydrate formulas during nighttime enteral feedings in cystic fibrosis patients. J Pediatr Gastroenterol Nutr 8: 321–326

    Article  PubMed  CAS  Google Scholar 

  40. Kerem B, Rommens JM, Buchanan JA et al. (1989) Identification of the cystic fibrosis gene: Genetic analysis. Science 245: 1073–1079

    Article  PubMed  CAS  Google Scholar 

  41. Kerem E, Corey M, Kerem B-S, Rommens J, Markiewicz D, Levison H, Tsui L-C, Durie P (1990) The relation between genotype and phenotype in cystic fibrosis - analysis of the most common mutation (Delta F5os). N Eng J Med 323: 1517–1522

    Article  CAS  Google Scholar 

  42. Khouri MR, Huang G, Shiau YF (1986) Sudan stain of fecal fat: new insight into an old test. Gastroenterol 96: 421–427

    Google Scholar 

  43. Knowles MR, Gatzy JAT, Boucher RC (1983) Relative ion permeability of normal and cystic fibrosis epithelium. J Clin Invest 71: 1410–1417

    Article  PubMed  CAS  Google Scholar 

  44. Knowlton RG, Cohen-Haguenauer O, Nguyen VC, et al. (1985) A polymorphic DNA marker linked to cystic fibrosis is located in chromosome 7. Nature 318: 380–381

    Article  PubMed  CAS  Google Scholar 

  45. Koletzko S, Stringer D, Cleghorn GJ, Durie PR (1989) Lavage treatment of distal intestinal obstuction syndrome in children with cystic fibrosis. Pediatrics 83: 727–733

    PubMed  CAS  Google Scholar 

  46. Koletzko S, Corey M, Ellis L, Spino M, Stringer DA, Durie RP (1990) Effects of cisapride in patients with cystic fibrosis and distal intestinal obstuction syndrome. J Pediatr 117: 815–822

    Article  PubMed  CAS  Google Scholar 

  47. Koletzko S, Koletzko B, Reinhardt D (1994) Aktuelle Aspekte der Ernährungstherapie bei zystischer Fibrose. Monatsschr. Kinderheilkd. 142: 432–445

    Google Scholar 

  48. Koletzko S, Haisch M, Seeboth I, Braden B, Hengeis K, Koletzko B, Hering P (1995) Isotope-selective non-dispersive infrared spectrometry for detection of Helicobacter pylori infection with 13C-urea breath test. Lancet. 345: 961–962

    Article  PubMed  CAS  Google Scholar 

  49. Koletzko S. Koletzko B (1993) Zystische Fibrose: Normalernährung oder Ernährungstherapie. In: Koletzko B (ed), Ernährung chronisch kranker Kinder und Jugendlicher. Heidelberg: Springer Verlag, pp. 167 - 190

    Chapter  Google Scholar 

  50. Kopelman H, Durie P, Gaskin K, Weizman Z, Forstner G (1985) Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis. N Eng J Med 312: 329–334

    Article  CAS  Google Scholar 

  51. Kopelman H, Corey M, Gaskin K, Durie P, Weizman Z, Forstner G (1988) Impaired Chloride Secretion, as well as bicarbonate secretion, underlies the fluid secretory defect in the cystic fibrosis pancreas. Gastroenterol 95: 349–355

    CAS  Google Scholar 

  52. Kopelman H, Forstner G, Durie P, Corey M (1989) Origins of chloride and bicarbonate secretory defects in the cystic fibrosis pancreas, as suggested by pancreatic function studies on control and CF subjects with preserved pancreatic function. Clin Invest Med 12: 207–211

    PubMed  CAS  Google Scholar 

  53. Kristidis P, Bozon D, Corey M, Markiewicz D, Rommens J, Tsui L-C, Durie P (1992) Genetic determination of exocrine pancreatic function in cystic fibrosis. N Eng J Med 323: 1517–1522

    Google Scholar 

  54. Lanng S, Thorsteinsson B, Lund-Andersen C, Nerup J, Schiotz PO, Koch C (1994) Diabetes mellitus in Danish cystic fibrosis patients: prevalence and late diabetic complications. Acta Paediatr 83: 72–77

    Article  PubMed  CAS  Google Scholar 

  55. Lembcke B, Braden B, Stein J (1994) Diagnostik der Steatorrhoe. Z Gastroenterol 32: 256–261

    PubMed  CAS  Google Scholar 

  56. Mainwright BJ, Scambler PJ, Schmidtke J et al. (1985) Localization of cystic fibrosis locus to human chromosome 7 cen-q22. Nature 318: 384–385

    Article  Google Scholar 

  57. Marcus MS, Sondel SA, Farell PM, Laxova A, Carey PM, Langhough R, Mischler EH (1991) Nutritional status of infants with cystic fibrosis associated with early diagnosis and inter-vention. Am J Clin Nutr 54: 578–585

    PubMed  CAS  Google Scholar 

  58. Meyer JH, Elashoff J, Porterfink V, Dressman J, Amidon GL (1988) Human postprandial gastric emptying of 1-3-millimeter spheres. Gastroenterol 94: 1315–1325

    CAS  Google Scholar 

  59. Mundlos S, Kiihnelt P, Adler G (1990). Monitoring enzyme replacement treatment in exocrine pancreatic insufficiency using the cholesteryl octanoate breath test. Gut 31: 1324–1328

    Article  PubMed  CAS  Google Scholar 

  60. Neis PF (1981) Zeub. Uber die Anwendbarkeit von Fluoresceindilaurat zur exokrinen Pankreasfunktionspriifung bei Kindern. Monatsschr. Kinderkeilkd. 129: 347–348

    CAS  Google Scholar 

  61. Nielsen OH, Larsen BF (1982) The incidence of anemia, hypoproteinemia, and edema in infants as presenting symptoms of cystic fibrosis: a retrospective survey of the frequency of this symptom complex in 130 patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 1: 355–359

    Article  PubMed  CAS  Google Scholar 

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

    CAS  Google Scholar 

  63. Quinton PM (1983) Chloride impermeability in cystic fibrosis. Nature. 301: 421–422

    Article  PubMed  CAS  Google Scholar 

  64. Reardon MC, Hammond KB, Accurso FJ, Fisher CD, Mccabe ERB, Cotton EK, Bowman CM (1984) Nutritional deficits exist before 2 months of age in some infants with cystic fibrosis identified by screening test. J Pediatr 105: 271–274

    Article  PubMed  CAS  Google Scholar 

  65. Robinson P, Sly PD (1990) Placebo-controlled trial of misoprostol in cystic fibrosis. J Pediatr Gastroenterol. Nutr. 11: 37–40

    Article  PubMed  CAS  Google Scholar 

  66. Robinson PJ, Smith AL, Sly PD (1990) Duodenal pH in cystic fibrosis and its relationship to fat malabsorption. Dig. Dis. Sci. 35: 1299–1304

    Article  PubMed  CAS  Google Scholar 

  67. Shmerling DH, Forrer JCW, Prader A (1970) Fecal fat and nitrogen in healthy children and in children with malabsorption or maldigestion. Pediatrics. 5: 690–695

    Google Scholar 

  68. Shwachman, H (1975) Gastrointestinal manifestations of cystic fibrosis. Pediatr. Clin N Am 22: 787–805

    CAS  Google Scholar 

  69. Shwachman H, Lebenthal E, Khaw K-T (1975) Recurrent acute pancreatitis in patients with cystic fibrosis with normal pancreatic enzymes. Pediatrics 55: 86–95

    PubMed  CAS  Google Scholar 

  70. Skopnik H, Kentrup H, Kusenbach G, Pfaeffle R, Kock R (1993) Glukosehomdostase bei zystischer Fibrose: Oraler Glukosetoleranztest im Vergleich zu einer Formulabelastung. Monatsschr Kinderheilkd 141: 142–147

    Google Scholar 

  71. Sokol RJ, Reardon MC, Accurso FJ, Stall C, Narkewicz M, Abman SH, Hammond KB (1989) Fat-soluble-vitamin status during the first year of life in infants with cystic fibrosis identi¬fied by screening of newborns. Am J Clin Nutr 50: 1064–1071

    PubMed  CAS  Google Scholar 

  72. Sturgess JM (1984) Structural and developmental abnormalities of the exocrine pancreas in cystic fibrosis. Proceedings of the 5th Professional Conference of the Candian Cystic Fibrosis Foundation. J Pediatr Gastroenterol Nutr 3: 55–66

    Article  Google Scholar 

  73. Thompson GN (1988) Excessive taurine loss predisposes to taurine deficiency in cystic fibrosis. J Pediatr Gastroenterol Nutr 7: 214–219

    Article  PubMed  CAS  Google Scholar 

  74. Vantrappen GR, Rutgeerts PJ, Ghoos YF, Hiele MI (1989) Mixed triglyceride breath test: a noninvasive test of pancreatic lipase activity in the duodenum. Gastroenterol 96: 1126–1134

    CAS  Google Scholar 

  75. Waters, D. L., S. F. A. Dorney, K. J. Gaskin, M. A. Gruca, M. O’halloran, B. Wilcken. Pancreatic function in infants identified as having cystic fibrosis in a neonatal screening program. N Engl J Med. 322 (1990) 303–308

    Article  PubMed  CAS  Google Scholar 

  76. Weber AM, Roy CC, Morin CL, Lasalle R (1973) Malabsorption of bile acids in children with cystic fibrosis. N Engl J Med 289: 1001–1005

    Article  PubMed  CAS  Google Scholar 

  77. Welsh, M. J., A. E. Smith. Molucular mechanisms of CFTR chloride in cystic fibrosis. Cell. 73 (1993) 1251–1254

    Article  PubMed  CAS  Google Scholar 

  78. Wilcken B, (1987) An evaluation of screening for cystic fibrosis in genetics and epithelial cell dysfunction In: Riordan JR, Buchwald M (eds). Cystic fibrosis. New York: Alan R. Liss, pp. 201

    Google Scholar 

  79. Zipf WC (1990) Cystic Fibrosis Foundation. Consensus conference on CF-related diabetes mellitus. pp.1 1. Concepts in Care.

    Google Scholar 

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Koletzko, S. (1996). Pankreasinsuffizienz bei Mukoviszidose und metabolische Komplikationen — ein diagnostisch vielschichtiges Problem. In: Kist, M., Caspary, W.F., Lentze, M.J. (eds) Ökosystem Darm VII. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-80327-7_26

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