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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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 (aged 1 month to 17.0 years) with chronic forms of intrahepatic neonatal cholestasis and 47 children (aged 4 months to 8.0 years) 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
StatePublished - 1985

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Vitamin E Deficiency
Cholestasis
Nervous System Diseases
Vitamin E
Nervous System
Nervous System Malformations
Ataxia
Extrahepatic Cholestasis
Serum
Intrahepatic Cholestasis
Biliary Atresia
Ophthalmoplegia
Peripheral Nervous System Diseases
Neurologic Manifestations
Lipids

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Sokol, R. J., Guggenheim, M. A., Heubi, J. E., Iannaccone, S. T., Butler-Simon, N., Jackson, V., ... 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.

Frequency and clinical progression of the vitamin E deficiency neurologic disorder in children with prolonged neonatal cholestasis. / 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.

In: American Journal of Diseases of Children, Vol. 139, No. 12, 1985, p. 1211-1215.

Research output: Contribution to journalArticle

Sokol, RJ, Guggenheim, MA, Heubi, JE, Iannaccone, ST, Butler-Simon, N, Jackson, V, Miller, C, Cheney, M, Balistreri, WF & 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, vol. 139, no. 12, pp. 1211-1215.
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. / Frequency and clinical progression of the vitamin E deficiency neurologic disorder in children with prolonged neonatal cholestasis. In: American Journal of Diseases of Children. 1985 ; Vol. 139, No. 12. pp. 1211-1215.
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