Advertisement

Zystische Fibrose — Normalernährung oder Ernährungstherapie?

  • S. Koletzko
  • B. Koletzko
Conference paper

Zusammenfassung

Eine chronische Mangelernährung hielt man über Jahrzehnte für ein untrennbar mit der zystischen Fibrose (CF) verbundenes Symptom. Untergewicht, vermindertes Längenwachstum und eine verzögerte Pubertätsentwicklung der betroffenen Kinder und Jugendlichen sowie eine reduzierte Endgröße im Erwachsenenalter wurden als schicksalhafte Folge der Erkrankung angesehen, oder sie galten als natürliche Adaptation an eine auftretende pulmonale Insuffizienz [22, 46]. Obwohl bereits einige Untersuchungen einen Zusammenhang zwischen dem Vorliegen einer schweren Malnutrition und einem ungünstigen Verlauf von Lungenfunktion und Überlebenskurven von CF-Patienten aufzeigten [14, 29, 43], erbrachte erst die Veröffentlichung von Corey et al. [15] Hinweise für eine kausale Beziehung zwischen Ernährungsstatus und Langzeit¬prognose. Corey et al. verglichen die Überlebenskurven der in Toronto und Boston betreuten CF-Patienten. Sie zeigten, daß die in Toronto betreuten Patienten etwa ab dem 10. Lebensjahr deutlich bessere Überlebenskurven als die Bostoner Patienten aufwiesen und im Schnitt 9 Jahre älter wurden. Die Autoren konnten zeigen, daß keine wesentlichen Unterschiede zwischen den beiden CF-Zentren bezüglich Ambulanzgröße, Geschlechts- und Altersverteilung und ethnischem Hintergrund der Patienten sowie der Behand-lungsprinzipien pulmonaler Komplikationen vorlagen.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Literatur

  1. 1.
    Abemathy RS (1976) Bulging fontanelle as presenting sign in cystic fibrosis: vitamin A metabolism and effect on cerebrospinal fluid pressures. Am J Dis Child 130: 1360–1362Google Scholar
  2. 2.
    Abman SH, Accurso FJ, Bowman CM (1986) Persistent mobidity and mortality of protein calorie malnutrition in young infants with CF. J Pediatr Gastroenterol Nutr 5: 393–396PubMedCrossRefGoogle Scholar
  3. 3.
    Beckerman RC, Taussig LM (1979) Hypoelectrolytemia and metabolic alkalosis in infants with cystic fibrosis. Pediatrics 63: 580–583PubMedGoogle Scholar
  4. 4.
    Bell L, Linton WL, Corey M, Durie P, Forstner G (1981) Nutrient intakes of ad¬olescents with cystic fibrosis. J Can Diet Ass 42: 62–71Google Scholar
  5. 5.
    Belli DC, Levy E, Darling P, Leroy C, Lepage G, Giguere R, Roy CC (1987) Taurine improves the absorption of a fat meal in patients with cystic fibrosis. Pediatrics 80: 517–523PubMedGoogle Scholar
  6. 6.
    Bines HE, Jacobowitz E (1991) Hypoproteinemia, anemia and failure to thrive in an infant. Gastroenterology 101: 848–856PubMedGoogle Scholar
  7. 7.
    Boland MP, MacDonald NE, Stoski DS, Soucy P, Patrick J (1986) Chromic jejunostomy feeding with a non-elemental formula in undernourished patients with cystic fibrosis. Lancet i: 232–234Google Scholar
  8. 8.
    Brady MS, Rickard K, Yu P, Eigen H (1991) Effectiveness and safety of small vs. large doses of enteric coated pancreatic enzymes in reducing steatorrhea in children with cystic fibrosis. Pediatr Pulmonol 10: 79, 85Google Scholar
  9. 9.
    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: 533–540PubMedCrossRefGoogle Scholar
  10. 10.
    Buchdahl RM, Cox M, Fullelylove C, Marchant JL, Tomkins AM, Brueton MJ, Warner JO (1988) Increased energy expenditure in cyxtic fibrosis. J Appl Physiol 64: 1810–1816PubMedCrossRefGoogle Scholar
  11. 11.
    Buchdahl RM, Fulleylove C, Marchant JL, Warner JO, Brueton MJ (1989) Energy and nutrient tintakes in cystic fibrosis. Arch Dis Child 64: 373–378PubMedCrossRefGoogle Scholar
  12. 12.
    Bye AME, Muller DPR, Wilson J, Wrigth VM, Mearns MB (1985) Symptomatic vitamin E deficiency in cystic fibrosis. Arch Dis Child 60: 162–164Google Scholar
  13. 13.
    Chase HP, Long MA, Lavin MH (1979) Cystic fibrosis and malnutrition. J Pediatr 95: 337–347PubMedCrossRefGoogle Scholar
  14. 14.
    Corey M, Gaskin K, Durie P, Levison H, Forstner G (1984) Improved prognosis in CF patients with normal fat absorption. J Pediatr Gastroenterol Nutr [Suppl] 1: S99–S105CrossRefGoogle Scholar
  15. 15.
    Corey M, McLaughlin FJ, Williams M, Levison H (1988) A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol 41: 583–591PubMedCrossRefGoogle Scholar
  16. 16.
    Cornelissen EAM, van Lieburg AF, Motohara K, van Oostrom CG (1992) Vitamin K status in cystic fibrosis. Acta Paediatr 81: 658–661PubMedCrossRefGoogle Scholar
  17. 17.
    Dalzell AM, Shepherd RW, Dean B, Cleghorn GJ, Holt TL, Francis PJ (1992) Nutritional rehabilitation in cystic fibrosis: a 5 year follow-up study. J Pediatr Gastroenterol Nutr 15: 141–145PubMedCrossRefGoogle Scholar
  18. 18.
    Darling PB, Lepage G, Leroy C, Masson P, Roy CC (1985) Effect of taurine supplements on fat absorption in cystic fibrosis. Pediatr Res 19: 578–582PubMedCrossRefGoogle Scholar
  19. 19.
    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: 45–49PubMedCrossRefGoogle Scholar
  20. 20.
    Dolan TF, Rowe DS, Gibson LE (1970) Edema and hypoproteinemia in infants with cystic fibrosis. Clin Pediatr 9: 295–297CrossRefGoogle Scholar
  21. 21.
    Durie PR, Bell L, Linton W, Corey ML, Forstner GG (1980) Effect of cimetidine and sodium bicarbonate on pancratic replacement therapy in cystic fibrosis. Gut 21: 778–786PubMedCrossRefGoogle Scholar
  22. 22.
    Durie PR, Pencharz PB (1989) A rational approach to the nutritional care of patients with cystic fibrosis. J R Soc Med [Suppl] 16: 11–20Google Scholar
  23. 23.
    Elias E, Muller DPR, Scott J (1981) Association of spinocerebellar disorders with cystic fibrosis or chronic childhood cholestasis and very low serum vitamin E. Lancet 2: 1319–1321CrossRefGoogle Scholar
  24. 24.
    Farrell PM, Hubbard VS (1983) Nutrition in cystic fibrosis: vitamins, fatty acids and minerals. In: Lloyd-Still JD (ed) Textbook of cystic fibrosis. Wright, Boston, pp 263–292Google Scholar
  25. 25.
    Feigal RJ, Shapiro BL (1979) Mitochondrial calcium uptake and oxygen consumption in cystic fibrosis. Nature 278: 276–277PubMedCrossRefGoogle Scholar
  26. 26.
    Feigelson J, Girault F, Pecau Y (1987) Gastro-oesophageal reflux and esophagitis in cystic fibrosis. Acta Paediatr Scand 76: 989–990PubMedCrossRefGoogle Scholar
  27. 27.
    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. Imperial Press, Toronto, pp 137–149Google Scholar
  28. 28.
    Frisancho A (1981) New norms of upper limb fat and muscle areas for assessment of nutritional status. Am J Clin Nutr 34: 2540–2545PubMedGoogle Scholar
  29. 29.
    Gaskin K, Gurwitz D, Durie P, Corey M, Levison H, Forstner G (1982) Im-proved respiratory prognosis in patients with cystic fibrosis with normal fat absorption. J Pediatr 100: 857–862PubMedCrossRefGoogle Scholar
  30. 30.
    Gibbens DT, Gilsanz V, Boechat MI, Dufer D, Carlson ME, Wang CI (1988) Osteoporosis in cystic fibrosis. J Pediatr 113: 295–300PubMedCrossRefGoogle Scholar
  31. 31.
    Gibson RA, Teubner JK, Haines K, Cooper DM, Davidson GP (1986) Relationships between pulmonary function and plasma fatty acid levels in cystic fibrosis patients. J Pediatr Gastroenterol Nutr 5: 408–415PubMedCrossRefGoogle Scholar
  32. 32.
    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: 52–58PubMedCrossRefGoogle Scholar
  33. 33.
    Hanly JG, McKenna MJ, Quigley C, Freaney R, Muldowney FP, Fitzgerald MX (1985) Hypovitaminosis D and response to supplementation in older patients with cystic fibrosis. J Med 56: 377–385Google Scholar
  34. 34.
    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: 321–326PubMedCrossRefGoogle Scholar
  35. 35.
    Kane RE, Hobbs PJ, Black PG (1990) Comparison of low, medium and high carbohydrate formulas for nighttime enteral feedings in cystic fibrosis patients. J Parenteral Enteral Nutr 14: 47–52CrossRefGoogle Scholar
  36. 36.
    Kerem E, Corey M, Kerem B, Rommens J, Markiewicz D, Levison H, Tsui L, Durie P (1990) The releation between genotype and phenotype in cystic fibrosis - analysis of the most common mutation (delta F 508). N Engl J Med 323: 1517–1522PubMedCrossRefGoogle Scholar
  37. 37.
    Kindstedt-Arfwidson K, Strandvik B (1988) Food intake in patients with cystic fibrosis on an ordinary diet. Scand J Gastroenterol [Suppl 143]: 160–162Google Scholar
  38. 38.
    Koletzko B (1986) Essentielle Fettsäuren: Bedeutung für Medizin und Ernährung. Akt Endokrinol Stoffwechsel 7: 18–27Google Scholar
  39. 39.
    Koletzko B, Rühl-Bagheri I, Thiel I, Steinkamp G (1992) Effects of a formula Supplement rich in linoleic acid on the essential fatty acid status of cystic fibrosis patients. Clin Nutr 11 [Suppl]: 39–40CrossRefGoogle Scholar
  40. 40.
    Koletzko S, Koletzko B, Reinhardt D (1993) Aktuelle Aspekte der Ernährungstherapie bei cystischer Fibrose. Monatsschr Kinderheilkd (im Druck)Google Scholar
  41. 41.
    Koletzko S, Stringer DA, Cleghorn GJ, Durie PR (1989) Intestinal lavage treatment of distal intestinal obstruction syndrome in cystic fibrosis. Pediatrics 83: 727–733PubMedGoogle Scholar
  42. 42.
    Koletzko S, Corey M, Ellis L, Spino M, Stringer DA, Durie PR (1990) Effects of Cisapride in patients with cystic fibrosis and distal intestinal obstruction syndrome. J Pediatr 117: 815–822PubMedCrossRefGoogle Scholar
  43. 43.
    Kraemer R, Rüdeberg A, Hadorn B, Rossi E (1978) Relative underweight in cystic fibrosis and its prognostic value. Acta Paediatr Scand 67: 33–37PubMedCrossRefGoogle Scholar
  44. 44.
    Kühnelt P, Mundlos S, Adler G (1991) Einfluß der Pelletgröße eines Pankreasenzympräparates auf die duodenale lipolytische Aktivität. Z Gastroenterol 29: 417–421PubMedGoogle Scholar
  45. 45.
    Levy LD, Durie PR, Pencharz PB, Corey ML (1985) Effects of long-term nutritional rehabilitation in cystic fibrosis. J Pediatr 107: 225–230PubMedCrossRefGoogle Scholar
  46. 46.
    Littlewood JM, MacDonald A (1987) Rationale of modern dietary recommendations in cystic fibrosis. J R Soc Med [Suppl] 15: 16–24Google Scholar
  47. 47.
    Lloyd-Still JD, Johnson SB, Holman RT (1981) Essential fatty acid status in cystic fibrosis and the effects of safflower oil supplementation. Am J Clin Nutr 34: 1–7PubMedGoogle Scholar
  48. 48.
    MacDonald A, Kelleher J, Littlewood JM (1988) A normal fat diet for cystic fibrosis: is a dietitian still needed? Scanc J Gastroenterol 23 [Suppl]: 157–159CrossRefGoogle Scholar
  49. 49.
    Marcoulis G, Parmentier Y, Nicolas JP, Jimenez M, Gerard P (1980) Cobalamine malabsorption due to nondegradation of R proteins in the human intestine. J Clin Invest 66: 430–440CrossRefGoogle Scholar
  50. 50.
    Marcus MS, Sondel SA, Farrell PM, Laxova A, Carey PM, Langhough R, Mischler EH (1991) Nutritional status in infants with cystic fibrosis associated with early diagnosis and intervention. Am J Clin Nutr 54: 578–585PubMedGoogle Scholar
  51. 51.
    McKenna MC, Hubbard VS, Bieri JG (1985) Linoleic acid absorption from lipid supplements in patients with cystic fibrosis with pancreatic insufficiency and in control subjects. J Pediatr Gastroenterol Nutr 4: 45–51PubMedCrossRefGoogle Scholar
  52. 52.
    Meyer JH, Elashoff J, Porter-Fink V, Dressman J, Amidon GL (1988) Human postprandial gastric emptying of 1–3 millimeter spheres. Gastroenterology 94: 1315–1325PubMedGoogle Scholar
  53. 53.
    Moore MC, Greene HL, Donald WD, Dunn GD (1986) Enteral-tube feeding as adjunct therapy in malnourished patients with cystic fibrosis: a clinical study and literature review. Am J Clin Nutr 44: 33–41PubMedGoogle Scholar
  54. 54.
    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 cystiv fibrosis. J Pediatr Gastroenterol Nutr 1: 355–359PubMedCrossRefGoogle Scholar
  55. 55.
    Nussbaum E, Boat TF, Wood RE, Doershuk CF (1979) Cystic fibrosis with acute hypoelectrolemia and metabolic alkalosis in infancy. AM J Dis Child 133: 965–966PubMedGoogle Scholar
  56. 56.
    O’Loughlin E, Forbes D, Parsons H, Scott B, Cooper D, Gall G (1986) Nutritional rehabilitation of malnourished patients with cystic fibrosis. Am J Clin Nutr 43: 732–737PubMedGoogle Scholar
  57. 57.
    O’Rawe A, Mcintosh I, Dodge JA, Brock DJH, Redmond AOB, Ward R, Mac Pherson AJS (1992) Increased energy expenditure in cystic fibrosis is associated with specific mutation. Clin Sei 82: 71–76Google Scholar
  58. 58.
    Parsons HG, Beaudy P, Dumas A, Pencharz PB (1983) Energy needs and growth in children with cystic fibrosis. J Pediatr Gastroenterol Nutr 2: 44–49PubMedCrossRefGoogle Scholar
  59. 59.
    Pelekanos JT, Holt TL, Ward LC, Cleghorn GJ, Shepherd RW (1990) Protein turnover in malnourished patients with cystic fibrosis: effects of elemental and nonelemental nutritional supplements. J Pediatr Gastroenterol Nutr 10: 339–343PubMedCrossRefGoogle Scholar
  60. 60.
    Ramsey BW, Farrell PM, Pencharz P, and the Consensus Committee (1992) Nutritional assessment and management in cystic fibrosis: a consensus report. Am J Clin Nutr 55: 108–116Google Scholar
  61. 61.
    Rayner RJ, Tyrell JC, Hiller EJ, Marenah C, Neugebauer MA, Vernon SA, Brimlow G (1989) Night blindness and conjunctival xerosis caused by vitamin A deficiency in patients with cystic fibrosis. Arch Dis Child 64: 1151–1156PubMedCrossRefGoogle Scholar
  62. 62.
    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: 271–274PubMedCrossRefGoogle Scholar
  63. 63.
    Reisman J, Petrou C, Corey M, Stringer D, Durie P, Levison H (1989) Hypoalbuminemia at initial examination in patients with cystic fibrosis. J Pediatr 115: 755–758PubMedCrossRefGoogle Scholar
  64. 64.
    Reiter EO, Brugman SM, Pike JW, Pitt M, Dokoh S, Haussler MR, Gerstle RS, Taussig LM (1985) Vitamin D metabolites in adolescents and young adults with cystic fibrosis: effects of sun and season. J Pediatr 106: 21–26PubMedCrossRefGoogle Scholar
  65. 65.
    Shepherd RW, Holt TL, Thomas BJ, Kay L, Isles A, Francis PJ, Ward LC (1986) Nutritional rehabilitation in cystic fibrosis: controlled studies of effects on nutritional growth retardation, body protein turnover and the course of pulmonary disease. J Pediatr 109: 788–794PubMedCrossRefGoogle Scholar
  66. 66.
    Shepherd RW, Vasques-Velasquez L, Prentice A, Holt TL, Coward WA, Lucas A (1988) Increased energy eypenditure in young children with cystic fibrosis. Lancet 1: 1300–1303PubMedCrossRefGoogle Scholar
  67. 67.
    Shmerling DH, Forrer JCW, Prader A (1970) Fecal fat and nitrogen in healthy children and in children with malabsorption or maldigestion. Pediatrics 5: 690–695Google Scholar
  68. 68.
    Sokol RJ, Reardon MC, Accurso FJ, Stall C, Narkewicz M, Abman SH, Hammond BK (1989) Fat-soluble-vitamin status during the first year of life in infants with cystic fibrosis identified by screening of newborns. Am J Clin Nutr 50: 1064–1071PubMedGoogle Scholar
  69. 69.
    Steinkamp G, Rühl I, Müller MJ, von der Hardt H (1989) Increased resting energy expenditure is related to the severity of lung disease in cystic fibrosis. Pediatr Pulmonol [Suppl] 4: 146 (Abstract)Google Scholar
  70. 70.
    Steinkamp G, Rühl I, von der Hardt H (1990) Long-term effects of nocturnal gastrostomy feedings on nutritional status and lung function in CF. Pediatric Pulmonol [Supl] 5: 267 (Abstact)Google Scholar
  71. 71.
    Strandvik B, Brönnegard M, Gilljam H, Carlstedt-Duke J (1988) Relation between defective regulation of arachidonic acid release and symptoms in cystic fibrosis. Scand J Gastroenterol 23 [Suppl 143]: 1–4CrossRefGoogle Scholar
  72. 72.
    Stutts MJ, Knowles MR, Gatzy JT, Boucher RC (1986) Oxygen consumption and ouabain binding sites in cystic fibrosis nasal epithelium. Pediatr Res 20: 1316–1320PubMedCrossRefGoogle Scholar
  73. 73.
    Thompson GN, Robb TA, Davidson GP (1987) Taurine supplementation, fat absorption, and growth in cystic fibrosis. J Pediatr 111: 501–506PubMedCrossRefGoogle Scholar
  74. 74.
    Thompson GN (1988) Excessive taurine loss predisposes to taurine deficiency in cystic fibrosis. J Pediatr Gastroenterol Nutr 7: 214–219PubMedCrossRefGoogle Scholar
  75. 75.
    Tomezsko JL, Stallings V, Scanlin TF (1992) Dietary intake of healthy children with cystic fibrosis compared with normal control children. Pediatrics 90: 547–553PubMedGoogle Scholar
  76. 76.
    Vaisman N, Pencharz PB, Corey M, Canny GJ, Hahn E (1987 a) Energy expenditure of patients with cystic fibrosis. J Pediatr 111: 496–500Google Scholar
  77. 77.
    Vaisman N, Levy LD, Pencharz PB, Tan YK, Soldin SJ, Canny GJ, Hahn E (1987 b) Effect of salbutamol on resting energy expenditure in patients with cystic fibrosis. J Pediatr 111: 137–139Google Scholar
  78. 78.
    Vaisman N, Clarke R, Rossi M, Goldberg E, Zello GA, Pencharz PB (1992) Protein turnover and resting energy expenditure in patients with undernutrition and chronic lung disease. Am J Clin Nutr 55: 63–69PubMedGoogle Scholar
  79. 79.
    Vernon SA, Neugebauer MAZ, Brimlow G, Tyrell JC, Hiller EJ (1989) Conjunctival xerosis in cystic fibrosis. J R Soc Med 82: 46–47PubMedGoogle Scholar
  80. 80.
    Walters TR, Koch HF (1972) Hemorrhagic diathesis and cystic fibrosis in infancy. Am J Dis Child 124: 641–643PubMedGoogle Scholar
  81. 81.
    Waters DL, Dorney SFA, Gaskin KJ, Gruca MA, O’Halloran M, Wilcken B (1990) Pancreatic function in infants indentified as having cystic fibrosis in a neonatal screening program. N Engl J Med 322: 303–308PubMedCrossRefGoogle Scholar
  82. 82.
    Weber AM, Roy CC, Morin CL, Lasalle R (1973) Malabsorption of bile acids in children with cystic fibrosis. N Engl J Med 289: 1001–1005PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1993

Authors and Affiliations

  • S. Koletzko
  • B. Koletzko

There are no affiliations available

Personalised recommendations