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J R Soc Med. 1989; 82(Suppl 16): 11–20.
PMCID: PMC1291914

A rational approach to the nutritional care of patients with cystic fibrosis.

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Selected References

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  • Kraemer R, Rüdeberg A, Hadorn B, Rossi E. Relative underweight in cystic fibrosis and its prognostic value. Acta Paediatr Scand. 1978 Jan;67(1):33–37. [PubMed]
  • Roy CC, Darling P, Weber AM. A rational approach to meeting macro- and micronutrient needs in cystic fibrosis. J Pediatr Gastroenterol Nutr. 1984;3 (Suppl 1):S154–S162. [PubMed]
  • Gurwitz D, Corey M, Francis PW, Crozier D, Levison H. Perspectives in cystic fibrosis. Pediatr Clin North Am. 1979 Aug;26(3):603–615. [PubMed]
  • SHWACHMAN H. Therapy of cystic fibrosis of the pancreas. Pediatrics. 1960 Jan;25:155–163. [PubMed]
  • Allan JD, Mason A, Moss AD. Nutritional supplementation in treatment of cystic fibrosis of the pancreas. Am J Dis Child. 1973 Jul;126(1):22–26. [PubMed]
  • Gracey M, Burke V, Anderson CM. Assessment of medium-chain triglyceride feeding in infants with cystic fibrosis. Arch Dis Child. 1969 Jun;44(235):401–403. [PMC free article] [PubMed]
  • Crozier DN. Cystic fibrosis: a not-so-fatal disease. Pediatr Clin North Am. 1974 Nov;21(4):935–950. [PubMed]
  • Pencharz PB. Energy intakes and low-fat diets in children with cystic fibrosis. J Pediatr Gastroenterol Nutr. 1983;2(3):400–402. [PubMed]
  • Gaskin KJ, Durie PR, Lee L, Hill R, Forstner GG. Colipase and lipase secretion in childhood-onset pancreatic insufficiency. Delineation of patients with steatorrhea secondary to relative colipase deficiency. Gastroenterology. 1984 Jan;86(1):1–7. [PubMed]
  • Gaskin KJ, Durie PR, Corey M, Wei P, Forstner GG. Evidence for a primary defect of pancreatic HCO3-secretion in cystic fibrosis. Pediatr Res. 1982 Jul;16(7):554–557. [PubMed]
  • Zentler-Munro PL, Fine DR, Batten JC, Northfield TC. Effect of cimetidine on enzyme inactivation, bile acid precipitation, and lipid solubilisation in pancreatic steatorrhoea due to cystic fibrosis. Gut. 1985 Sep;26(9):892–901. [PMC free article] [PubMed]
  • Belli DC, Levy E, Darling P, Leroy C, Lepage G, Giguère R, Roy CC. Taurine improves the absorption of a fat meal in patients with cystic fibrosis. Pediatrics. 1987 Oct;80(4):517–523. [PubMed]
  • Daniels L, Davidson GP, Martin AJ. Comparison of the macronutrient intake of healthy controls and children with cystic fibrosis on low fat or nonrestricted fat diets. J Pediatr Gastroenterol Nutr. 1987 May-Jun;6(3):381–386. [PubMed]
  • Scott RB, O'Loughlin EV, Gall DG. Gastroesophageal reflux in patients with cystic fibrosis. J Pediatr. 1985 Feb;106(2):223–227. [PubMed]
  • Feigelson J, Girault F, Pecau Y. Gastro-oesophageal reflux and esophagitis in cystic fibrosis. Acta Paediatr Scand. 1987 Nov;76(6):989–990. [PubMed]
  • Rosenstein BJ, Langbaum TS. Incidence of distal intestinal obstruction syndrome in cystic fibrosis. J Pediatr Gastroenterol Nutr. 1983 May;2(2):299–301. [PubMed]
  • Pencharz P, Hill R, Archibald E, Levy L, Newth C. Energy needs and nutritional rehabilitation in undernourished adolescents and young adult patients with cystic fibrosis. J Pediatr Gastroenterol Nutr. 1984;3 (Suppl 1):S147–S153. [PubMed]
  • Vaisman N, Pencharz PB, Corey M, Canny GJ, Hahn E. Energy expenditure of patients with cystic fibrosis. J Pediatr. 1987 Oct;111(4):496–500. [PubMed]
  • Buchdahl RM, Cox M, Fulleylove C, Marchant JL, Tomkins AM, Brueton MJ, Warner JO. Increased resting energy expenditure in cystic fibrosis. J Appl Physiol (1985) 1988 May;64(5):1810–1816. [PubMed]
  • Feigal RJ, Shapiro BL. Mitochondrial calcium uptake and oxygen consumption in cystic fibrosis. Nature. 1979 Mar 15;278(5701):276–277. [PubMed]
  • Stutts MJ, Knowles MR, Gatzy JT, Boucher RC. Oxygen consumption and ouabain binding sites in cystic fibrosis nasal epithelium. Pediatr Res. 1986 Dec;20(12):1316–1320. [PubMed]
  • Shepherd RW, Holt TL, Vasques-Velasquez L, Coward WA, Prentice A, Lucas A. Increased energy expenditure in young children with cystic fibrosis. Lancet. 1988 Jun 11;1(8598):1300–1303. [PubMed]
  • Thet LA, Alvarez H. Effect of hyperventilation and starvation on rat lung mechanics and surfactant. Am Rev Respir Dis. 1982 Aug;126(2):286–290. [PubMed]
  • Chase HP, Long MA, Lavin MH. Cystic fibrosis and malnutrition. J Pediatr. 1979 Sep;95(3):337–347. [PubMed]
  • Congden PJ, Bruce G, Rothburn MM, Clarke PC, Littlewood JM, Kelleher J, Losowsky MS. Vitamin status in treated patients with cystic fibrosis. Arch Dis Child. 1981 Sep;56(9):708–714. [PMC free article] [PubMed]
  • Deren JJ, Arora B, Toskes PP, Hansell J, Sibinga MS. Malabsorption of crystalline vitamin B 12 in cystic fibrosis. N Engl J Med. 1973 May 3;288(18):949–950. [PubMed]
  • Abernathy RS. Bulging fontanelle as presenting sign in cystic fibrosis. Vitamin A metabolism and effect on cerebrospinal fluid pressure. Am J Dis Child. 1976 Dec;130(12):1360–1362. [PubMed]
  • Underwood BA, Denning CR. Blood and liver concentrations of vitamins A and E in children with cystic fibrosis of the pancreas. Pediatr Res. 1972 Jan;6(1):26–31. [PubMed]
  • Rasmussen M, Michalsen H, Lie SO, Nilsson A, Petersen LB, Norum KR. Intestinal retinol esterification and serum retinol in children with cystic fibrosis. J Pediatr Gastroenterol Nutr. 1986 May-Jun;5(3):397–403. [PubMed]
  • Jacob RA, Sandstead HH, Solomons NW, Rieger C, Rothberg R. Zinc status and vitamin A transport in cystic fibrosis. Am J Clin Nutr. 1978 Apr;31(4):638–644. [PubMed]
  • Friedman HZ, Langman CB, Favus MJ. Vitamin D metabolism and osteomalacia in cystic fibrosis. Gastroenterology. 1985 Mar;88(3):808–813. [PubMed]
  • Gibbens DT, Gilsanz V, Boechat MI, Dufer D, Carlson ME, Wang CI. Osteoporosis in cystic fibrosis. J Pediatr. 1988 Aug;113(2):295–300. [PubMed]
  • Gallo-Torres HE. Obligatory role of bile for the intestinal absorption of vitamin E. Lipids. 1970 Apr;5(4):379–384. [PubMed]
  • Farrell PM, Bieri JG, Fratantoni JF, Wood RE, di Sant'Agnese PA. The occurrence and effects of human vitamin E deficiency. A study in patients with cystic fibrosis. J Clin Invest. 1977 Jul;60(1):233–241. [PMC free article] [PubMed]
  • Elias E, Muller DP, Scott J. Association of spinocerebellar disorders with cystic fibrosis or chronic childhood cholestasis and very low serum vitamin E. Lancet. 1981 Dec 12;2(8259):1319–1321. [PubMed]
  • Sitrin MD, Lieberman F, Jensen WE, Noronha A, Milburn C, Addington W. Vitamin E deficiency and neurologic disease in adults with cystic fibrosis. Ann Intern Med. 1987 Jul;107(1):51–54. [PubMed]
  • SUNG JH. NEUROAXONAL DYSTROPHY IN MUCOVISCIDOSIS. J Neuropathol Exp Neurol. 1964 Oct;23:567–583. [PubMed]
  • Walters TR, Koch HF. Hemorrhagic diathesis and cystic fibrosis in infancy. Am J Dis Child. 1972 Nov;124(5):641–642. [PubMed]
  • Komp DM, Selden RF., Jr Coagulation abnormalities in cystic fibrosis. Chest. 1970 Nov;58(5):501–503. [PubMed]
  • Solomons NW, Wagonfeld JB, Rieger C, Jacob RA, Bolt M, Horst JV, Rothberg R, Sandstead H. Some biochemical indices of nutrition in treated cystic fibrosis patients. Am J Clin Nutr. 1981 Apr;34(4):462–474. [PubMed]
  • Castillo R, Landon C, Eckhardt K, Morris V, Levander O, Lewiston N. Selenium and vitamin E status in cystic fibrosis. J Pediatr. 1981 Oct;99(4):583–585. [PubMed]
  • Green CG, Doershuk CF, Stern RC. Symptomatic hypomagnesemia in cystic fibrosis. J Pediatr. 1985 Sep;107(3):425–428. [PubMed]
  • Godson C, Ryan MP, Brady HR, Bourke S, FitzGerald MX. Acute hypomagnesaemia complicating the treatment of meconium ileus equivalent in cystic fibrosis. Scand J Gastroenterol Suppl. 1988;143:148–150. [PubMed]
  • Ater JL, Herbst JJ, Landaw SA, O'Brien RT. Relative anemia and iron deficiency in cystic fibrosis. Pediatrics. 1983 May;71(5):810–814. [PubMed]
  • Heinrich HC, Bender-Götze C, Gabbe EE, Bartels H, Oppitz KH. Absorption of inorganic iron- (59Fe2+) in relation to iron stores in pancreatic exocrine insufficiency due to cystic fibrosis. Klin Wochenschr. 1977 Jun 15;55(12):587–593. [PubMed]
  • Farrell PM, Mischler EH, Engle MJ, Brown DJ, Lau SM. Fatty acid abnormalities in cystic fibrosis. Pediatr Res. 1985 Jan;19(1):104–109. [PubMed]
  • Rogiers V, Dab I, Crokaert R, Vis HL. Long chain non-esterified fatty acid pattern in plasma of cystic fibrosis patients and their parents. Pediatr Res. 1980 Sep;14(9):1088–1091. [PubMed]
  • Houvet D, Clerc M. Considerations relatives a un cas d'anencephalie avec difference foeto-maternelle du phenotype Pi. Clin Chim Acta. 1980 Mar 14;102(1):105–110. [PubMed]
  • Cleghorn GJ, Stringer DA, Forstner GG, Durie PR. Treatment of distal intestinal obstruction syndrome in cystic fibrosis with a balanced intestinal lavage solution. Lancet. 1986 Jan 4;1(8471):8–11. [PubMed]
  • Weizman Z, Forstner GG, Gaskin KJ, Kopelman H, Wong S, Durie PR. Bentiromide test for assessing pancreatic dysfunction using analysis of para-aminobenzoic acid in plasma and urine. Studies in cystic fibrosis and Shwachman's syndrome. Gastroenterology. 1985 Sep;89(3):596–604. [PubMed]
  • Durie PR, Forstner GG, Gaskin KJ, Moore DJ, Cleghorn GJ, Wong SS, Corey ML. Age-related alterations of immunoreactive pancreatic cationic trypsinogen in sera from cystic fibrosis patients with and without pancreatic insufficiency. Pediatr Res. 1986 Mar;20(3):209–213. [PubMed]
  • Shepherd R, Cooksley WG, Cooke WD. Improved growth and clinical, nutritional, and respiratory changes in response to nutritional therapy in cystic fibrosis. J Pediatr. 1980 Sep;97(3):351–357. [PubMed]
  • Mansell AL, Andersen JC, Muttart CR, Ores CN, Loeff DS, Levy JS, Heird WC. Short-term pulmonary effects of total parenteral nutrition in children with cystic fibrosis. J Pediatr. 1984 May;104(5):700–705. [PubMed]
  • Bertrand JM, Morin CL, Lasalle R, Patrick J, Coates AL. Short-term clinical, nutritional, and functional effects of continuous elemental enteral alimentation in children with cystic fibrosis. J Pediatr. 1984 Jan;104(1):41–46. [PubMed]
  • Levy LD, Durie PR, Pencharz PB, Corey ML. Effects of long-term nutritional rehabilitation on body composition and clinical status in malnourished children and adolescents with cystic fibrosis. J Pediatr. 1985 Aug;107(2):225–230. [PubMed]
  • Boland MP, Stoski DS, MacDonald NE, Soucy P, Patrick J. Chronic jejunostomy feeding with a non-elemental formula in undernourished patients with cystic fibrosis. Lancet. 1986 Feb 1;1(8475):232–234. [PubMed]
  • Shepherd RW, Holt TL, Thomas BJ, Kay L, Isles A, Francis PJ, Ward LC. Nutritional rehabilitation in cystic fibrosis: controlled studies of effects on nutritional growth retardation, body protein turnover, and course of pulmonary disease. J Pediatr. 1986 Nov;109(5):788–794. [PubMed]
  • Levy L, Durie P, Pencharz P, Corey M. Prognostic factors associated with patient survival during nutritional rehabilitation in malnourished children and adolescents with cystic fibrosis. J Pediatr Gastroenterol Nutr. 1986 Jan;5(1):97–102. [PubMed]

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