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. 1986 Jun;27(6):714–718. doi: 10.1136/gut.27.6.714

Effect of abnormal liver function on vitamin E status and supplementation in adults with cystic fibrosis.

R J Stead, D P Muller, S Matthews, M E Hodson, J C Batten
PMCID: PMC1433330  PMID: 3721295

Abstract

Patients with cystic fibrosis tend to have reduced serum concentrations of vitamin E and are therefore at risk of developing the neurological complications associated with vitamin E deficiency. Improved survival in cystic fibrosis has resulted in an increasing number of older patients who may develop hepatobiliary complications which may further impair the absorption of vitamin E. In this study the vitamin E status and results of supplementation with oral vitamin E were compared in adult patients with and without evidence of liver involvement as assessed by routine liver function tests. The serum vitamin E concentrations were reduced below normal in 24 of 25 patients. The mean serum vitamin E concentration was significantly lower (p less than 0.05) in those patients with abnormal liver function. When vitamin E status was assessed as the serum vitamin E/cholesterol ratio, however, there was no significant difference between those patients with normal and abnormal liver function. After supplementation with oral vitamin E, either 10 mg/kg/day for one month or 200 mg/day (equivalent to 3.4 to 4.4 mg/kg/day) for up to three months, there was no significant difference in the vitamin E status between the two groups. The results of this study indicate that in general, patients with cystic fibrosis and abnormal liver function do not require increased supplements of vitamin E compared with those with normal liver function.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Alvarez F., Landrieu P., Laget P., Lemonnier F., Odièvre M., Alagille D. Nervous and ocular disorders in children with cholestasis and vitamin A and E deficiencies. Hepatology. 1983 May-Jun;3(3):410–414. doi: 10.1002/hep.1840030321. [DOI] [PubMed] [Google Scholar]
  2. Bennett M. J., Medwadowski B. F. Vitamin A, vitamin E, and lipids in serum of children with cystic fibrosis or congenital heart defects compared with normal children. Am J Clin Nutr. 1967 May;20(5):415–421. doi: 10.1093/ajcn/20.5.415. [DOI] [PubMed] [Google Scholar]
  3. Bye A. M., Muller D. P., Wilson J., Wright V. M., Mearns M. B. Symptomatic vitamin E deficiency in cystic fibrosis. Arch Dis Child. 1985 Feb;60(2):162–164. doi: 10.1136/adc.60.2.162-a. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Elias E., Muller D. P., 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. doi: 10.1016/s0140-6736(81)91342-8. [DOI] [PubMed] [Google Scholar]
  5. Farrell P. M., Bieri J. G., Fratantoni J. F., Wood R. E., di Sant'Agnese P. A. 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. doi: 10.1172/JCI108760. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Farrell P. M., Levine S. L., Murphy M. D., Adams A. J. Plasma tocopherol levels and tocopherol-lipid relationships in a normal population of children as compared to healthy adults. Am J Clin Nutr. 1978 Oct;31(10):1720–1726. doi: 10.1093/ajcn/31.10.1720. [DOI] [PubMed] [Google Scholar]
  7. Guggenheim M. A., Jackson V., Lilly J., Silverman A. Vitamin E deficiency and neurologic disease in children with cholestasis: A prospective study. J Pediatr. 1983 Apr;102(4):577–579. doi: 10.1016/s0022-3476(83)80189-9. [DOI] [PubMed] [Google Scholar]
  8. Guggenheim M. A., Ringel S. P., Silverman A., Grabert B. E. Progressive neuromuscular disease in children with chronic cholestasis and vitamin E deficiency: diagnosis and treatment with alpha tocopherol. J Pediatr. 1982 Jan;100(1):51–58. doi: 10.1016/s0022-3476(82)80234-5. [DOI] [PubMed] [Google Scholar]
  9. Harding A. E., Muller D. P., Thomas P. K., Willison H. J. Spinocerebellar degeneration secondary to chronic intestinal malabsorption: a vitamin E deficiency syndrome. Ann Neurol. 1982 Nov;12(5):419–424. doi: 10.1002/ana.410120503. [DOI] [PubMed] [Google Scholar]
  10. Harries J. T., Muller D. P. Absorption of different doses of fat soluble and water miscible preparations of vitamin E in children with cystic fibrosis. Arch Dis Child. 1971 Jun;46(247):341–344. doi: 10.1136/adc.46.247.341. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Harries J. T., Muller D. P. Absorption of vitamin E in children with biliary obstruction. Gut. 1971 Jul;12(7):579–584. doi: 10.1136/gut.12.7.579. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Horwitt M. K., Harvey C. C., Dahm C. H., Jr, Searcy M. T. Relationship between tocopherol and serum lipid levels for determination of nutritional adequacy. Ann N Y Acad Sci. 1972 Dec 18;203:223–236. doi: 10.1111/j.1749-6632.1972.tb27878.x. [DOI] [PubMed] [Google Scholar]
  13. Howard L., Ovesen L., Satya-Murti S., Chu R. Reversible neurological symptoms caused by vitamin E deficiency in a patient with short bowel syndrome. Am J Clin Nutr. 1982 Dec;36(6):1243–1249. doi: 10.1093/ajcn/36.6.1243. [DOI] [PubMed] [Google Scholar]
  14. Muller D. P., Harries J. T., Lloyd J. K. The relative importance of the factors involved in the absorption of vitamin E in children. Gut. 1974 Dec;15(12):966–971. doi: 10.1136/gut.15.12.966. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Muller D. P., Lloyd J. K., Bird A. C. Long-term management of abetalipoproteinaemia. Possible role for vitamin E. Arch Dis Child. 1977 Mar;52(3):209–214. doi: 10.1136/adc.52.3.209. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Muller D. P., Lloyd J. K. Effect of large oral doses of vitamin E on the neurological sequelae of patients with abetalipoproteinemia. Ann N Y Acad Sci. 1982;393:133–144. doi: 10.1111/j.1749-6632.1982.tb31239.x. [DOI] [PubMed] [Google Scholar]
  17. Muller D. P., Lloyd J. K., Wolff O. H. Vitamin E and neurological function. Lancet. 1983 Jan 29;1(8318):225–228. doi: 10.1016/s0140-6736(83)92598-9. [DOI] [PubMed] [Google Scholar]
  18. Park R. W., Grand R. J. Gastrointestinal manifestations of cystic fibrosis: a review. Gastroenterology. 1981 Dec;81(6):1143–1161. [PubMed] [Google Scholar]
  19. Rosenblum J. L., Keating J. P., Prensky A. L., Nelson J. S. A progressive neurologic syndrome in children with chronic liver disease. N Engl J Med. 1981 Feb 26;304(9):503–508. doi: 10.1056/NEJM198102263040902. [DOI] [PubMed] [Google Scholar]
  20. Schaefer E. J., Levy R. I. Pathogenesis and management of lipoprotein disorders. N Engl J Med. 1985 May 16;312(20):1300–1310. doi: 10.1056/NEJM198505163122007. [DOI] [PubMed] [Google Scholar]
  21. Shwachman H., Kowalski M., Khaw K. T. Cystic fibrosis: a new outlook. 70 patients above 25 years of age. Medicine (Baltimore) 1977 Mar;56(2):129–149. [PubMed] [Google Scholar]
  22. Sokol R. J., Bove K. E., Heubi J. E., Iannaccone S. T. Vitamin E deficiency during chronic childhood cholestasis: presence of sural nerve lesion prior to 2 1/2 years of age. J Pediatr. 1983 Aug;103(2):197–204. doi: 10.1016/s0022-3476(83)80344-8. [DOI] [PubMed] [Google Scholar]
  23. Sokol R. J., Heubi J. E., Iannaccone S. T., Bove K. E., Balistreri W. F. Vitamin E deficiency with normal serum vitamin E concentrations in children with chronic cholestasis. N Engl J Med. 1984 May 10;310(19):1209–1212. doi: 10.1056/NEJM198405103101901. [DOI] [PubMed] [Google Scholar]
  24. Sokol R. J., Heubi J. E., Iannaccone S., Bove K. E., Balistreri W. F. Mechanism causing vitamin E deficiency during chronic childhood cholestasis. Gastroenterology. 1983 Nov;85(5):1172–1182. [PubMed] [Google Scholar]
  25. Underwood B. A., Denning C. R. 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. doi: 10.1203/00006450-197201000-00004. [DOI] [PubMed] [Google Scholar]
  26. Watkins J. B., Tercyak A. M., Szczepanik P., Klein P. D. Bile salt kinetics in cystic fibrosis: influence of pancreatic enzyme replacement. Gastroenterology. 1977 Nov;73(5):1023–1028. [PubMed] [Google Scholar]
  27. Weber A. M., Roy C. C., Morin C. L., Lasalle R. Malabsorption of bile acids in children with cystic fibrosis. N Engl J Med. 1973 Nov 8;289(19):1001–1005. doi: 10.1056/NEJM197311082891903. [DOI] [PubMed] [Google Scholar]
  28. Willison H. J., Muller D. P., Matthews S., Jones S., Kriss A., Stead R. J., Hodson M. E., Harding A. E. A study of the relationship between neurological function and serum vitamin E concentrations in patients with cystic fibrosis. J Neurol Neurosurg Psychiatry. 1985 Nov;48(11):1097–1102. doi: 10.1136/jnnp.48.11.1097. [DOI] [PMC free article] [PubMed] [Google Scholar]

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