Frequency and Clinical Progression of the Vitamin E Deficiency Neurologic Disorder in Children With Prolonged Neonatal Cholestasis

R. J. Sokol, M. A. Guggenheim, J. E. Heubi, S. T. Iannaccone, N. Butler-Simon, V. Jackson, C. Miller, M. Cheney, W. F. Balistreri, A. Silverman

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47 Scopus citations

Abstract

To determine the frequency of biochemical vitamin E deficiency and of the clinical signs of the vitamin E deficiency neurologic syndrome in children with prolonged neonatal cholestatic disorders, we studied 46 children with chronic forms of intrahepatic neonatal cholestasis and 47 children with extrahepatic biliary atresia. Based on serum vitamin E concentrations and the ratios of serum vitamin E concentration to total serum lipid concentration, 64% of the intrahepatic and 77% of the extrahepatic cholestasis groups were vitamin E deficient. Prior to age 1 year, neurologic function was normal in all children. Between ages 1 and 3 years, neurologic abnormalities were present in approximately 50% of the vitamin E–deficient children; after age 3 years, neurologic abnormalities were present in all vitamin E–deficient children. Areflexia was the first abnormality to develop between ages 1 and 4 years; truncal and limb ataxia, peripheral neuropathy, and ophthalmoplegia developed between ages 3 and 6 years. Neurologic dysfunction progressed to a disabling combination of findings by ages 8 to 10 years in the majority of vitamin E–deficient children. Neurologic function was normal in the vitamin E–sufficient children. We conclude that vitamin E status should be evaluated in infants in whom cholestasis is diagnosed, and effective therapy should be initiated to prevent or treat vitamin E deficiency at an early age.

Original languageEnglish (US)
Pages (from-to)1211-1215
Number of pages5
JournalAmerican Journal of Diseases of Children
Volume139
Issue number12
DOIs
StatePublished - Dec 1985

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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    Sokol, R. J., Guggenheim, M. A., Heubi, J. E., Iannaccone, S. T., Butler-Simon, N., Jackson, V., Miller, C., Cheney, M., Balistreri, W. F., & Silverman, A. (1985). Frequency and Clinical Progression of the Vitamin E Deficiency Neurologic Disorder in Children With Prolonged Neonatal Cholestasis. American Journal of Diseases of Children, 139(12), 1211-1215. https://doi.org/10.1001/archpedi.1985.02140140045024