Vitamin A and Vitamin E Replacement in Abetalipoproteinemia
Authors: JOHN G. BIERI
Journal: Annals of Internal Medicine (1984)

Brief Reports1 February 1984Vitamin A and Vitamin E Replacement in AbetalipoproteinemiaJOHN G. BIERI, Ph.D., JEFFREY M. HOEG, M.D., ERNST J. SCHAEFER, M.D., LOREN A. ZECH, M.D., H. BRYAN BREWER Jr, M.D.JOHN G. BIERI, Ph.D.Search for more papers by this author, JEFFREY M. HOEG, M.D.Search for more papers by this author, ERNST J. SCHAEFER, M.D.Search for more papers by this author, LOREN A. ZECH, M.D.Search for more papers by this author, H. BRYAN BREWER Jr, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-100-2-238 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptBecause patients with abetalipoproteinemia are unable to secrete apolipoprotein B, they cannot normally transport lipids in their circulation and thus have depressed plasma concentrations of various lipophilic substances, including triglycerides, cholesterol, and fat-soluble vitamins (1, 2). Administration of vitamin A and water-soluble preparations of vitamin E has halted or slowed the progressive neurologic and retinal deterioration seen in patients with this disease (3-6). These beneficial effects occurred despite the inability of plasma vitamin E to return to normal levels (5). By evaluating plasma and hepatic levels of vitamins A and E in a patient with abetalipoproteinemia after she received replacement...References1. HERBERTASSMANGOTTOFREDRICKSON PGAD. Familial lipoprotein deficiency: abetalipoproteinemia, hypobetalipoproteinemia, and Tangier disease. In: STANBURY JB, WYNGAARDEN JB, FREDRICKSON DS, GOLDSTEIN JL, BROWN MS, eds. The Metabolic Basis of Inherited Disease. 5th ed. New York: McGraw-Hill Book Company; 1983:589-621. Google Scholar2. MULLERHARRIESLLOYD DJJ. Vitamin E therapy in abetalipoproteinaemia. Arch Dis Child. 1970;45:715. CrossrefMedlineGoogle Scholar3. AZIZIZAIDMANESHCHARSZEINBERG EJJA. Abetalipoproteinemia treated with parenteral and oral vitamins A and E, and with medium chain triglycerides. Arch Paediatr Scand. 1978;67:796-801. MedlineGoogle Scholar4. MILLERDAVISILLINGWORTHBRADLEY RCDW. The neuropathy of abetalipoproteinemia. Neurology (NY). 1980;30:1286-91. CrossrefMedlineGoogle Scholar5. MULLERLLOYD DJ. Effect of large oral doses of vitamin E on the neurological sequelae of patients with abetalipoproteinemia. Ann NY Acad Sci. 1982;393:133-44. CrossrefMedlineGoogle Scholar6. BISHARAMERINCOOPERAZIZIDELPREDECKELBAUM SSMEGR. Combined viatmin A and E therapy prevents retinal electrophysiological deterioration in abetalipoproteinaemia. Br J Ophthalmol. 1982;66:767-70. CrossrefMedlineGoogle Scholar7. BIERIPOUKKA JR. Red cell content of vitamin E and fatty acids in normal subjects and patients with abnormal lipid metabolism. Int Z Vitaminforsch. 1970;40:344-50. MedlineGoogle Scholar8. UNDERWOODSIEGELDOLINSKIWEISELL BHMR. Liver stores of alpha-tocopherol in a normal population dying suddenly and rapidly from unnatural causes in New York City. Am J Clin Nutr. 1970;23:1314-21. CrossrefMedlineGoogle Scholar9. SAUBERLICHHODGESWALLACE HRD. Vitamin A metabolism and requirements in the human studied with the use of labeled retinol. Vit Horm. 1974;32:251-75. CrossrefMedlineGoogle Scholar10. KAYDENCHOWBJORNSON HCL. Spectrophotometric method for determination of tocopherol in red blood cells. J Lipid Res. 1973;14:533-40. CrossrefMedlineGoogle Scholar11. KAYDENHATAMTRABER HLM. The measurement of nanograms of tocopherol from needle aspiration biopsies of adipose tissue: normal and abetalipoproteinemic subjects. J Lipid Res. 1983;24:652-6. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: National Institutes of Health Bethesda, Maryland PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited ByDevelopmental and Inherited Liver DiseasePrimary immunodeficiency associated with defects in CD1 and CD1-restricted T cellsGenetic and metabolic liver diseasePrimary deficiency of microsomal triglyceride transfer protein in human abetalipoproteinemia is associated with loss of CD1 functionMolecular Neurobiology of Retinal DegenerationThe Role of the Microsomal Triglygeride Transfer Protein in AbetalipoproteinemiaManagement of Hereditary Retinal DegenerationsLipid studies in retinitis pigmentosaAngioid streaks associated with abetalipoproteinemiaAbsorption, lipoprotein transport, and regulation of plasma concentrations of vitamin E in humans.Liver fibrosis in a patient with familial homozygous hypobetalipoproteinaemia: possible role of vitamin supplementation.Metabolic pigmentary retinopathies: Diagnosis and therapeutic attemptsPostprandial plasma vitamin A metabolism in humans: A reassessment of the use of plasma retinyl esters as markers for intestinally derived chylomicrons and their remnantsVitamin E and fatty acids in the grey seal (Halichoerus grypus)Increased urinary mevalonic acid excretion in patients with abetalipoproteinemia and homozygous hypobetalipoproteinemiaAbetalipoproteinaemia in adults: Role of vitamin therapyGenetic Defects in Vitamin Utilization. Part I: General Aspects and Fat-Solumbe VitaminsUltrastructural changes in the organ of corti and in the ganglion spiral cochleae after vitamin A deficiencyThe Quebec Cooperative Study of Friedreich's Ataxia: 1974-1984 — 10 Years of ResearchAcanthocytes and Disorders of Lipoprotein Metabolism 1 February 1984Volume 100, Issue 2Page: 238-239KeywordsAdipose tissueApolipoproteinsAttentionBlood plasmaCholesterolLipidsVitamin AVitamin E Issue Published: 1 February 1984 PDF DownloadLoading ...