TY - JOUR
T1 - Intramuscular Vitamin E Repletion in Children With Chronic Cholestasis
AU - Perlmutter, David H.
AU - Gross, Paul
AU - Jones, H. Roy
AU - Fulton, Anne
AU - Grand, Richard J.
PY - 1987/2
Y1 - 1987/2
N2 - Progressive spinocerebellar degeneration was identified in six children with chronic cholestatic liver disease and attributed to severe vitamin E deficiency. In addition to areflexia, ataxia, dysmetria, and diminished vibratory and position sense, three patients had pigmentary retinopathy. Abnormalities were present on electromyography, nerve conduction studies, and electroretinography. Because the vitamin E deficiency was not corrected by oral administration of massive doses of vitamin E, vitamin E was administered by the intramuscular route. With doses of 50 to 100 mg of vitamin E every three to seven days, over a 32-month interval vitamin E deficiency and abnormal red blood cell peroxide hemolysis were corrected. Other than discomfort and occasional edema at the site of injection, there were no side effects of parenteral vitamin E therapy. In several other studies intramuscular vitamin E therapy has produced significant neurologic improvement in patients with similar characteristics. In this study clinical progression of spinocerebellar degeneration was arrested but improvement could not be demonstrated despite adequate vitamin E replacement.
AB - Progressive spinocerebellar degeneration was identified in six children with chronic cholestatic liver disease and attributed to severe vitamin E deficiency. In addition to areflexia, ataxia, dysmetria, and diminished vibratory and position sense, three patients had pigmentary retinopathy. Abnormalities were present on electromyography, nerve conduction studies, and electroretinography. Because the vitamin E deficiency was not corrected by oral administration of massive doses of vitamin E, vitamin E was administered by the intramuscular route. With doses of 50 to 100 mg of vitamin E every three to seven days, over a 32-month interval vitamin E deficiency and abnormal red blood cell peroxide hemolysis were corrected. Other than discomfort and occasional edema at the site of injection, there were no side effects of parenteral vitamin E therapy. In several other studies intramuscular vitamin E therapy has produced significant neurologic improvement in patients with similar characteristics. In this study clinical progression of spinocerebellar degeneration was arrested but improvement could not be demonstrated despite adequate vitamin E replacement.
UR - http://www.scopus.com/inward/record.url?scp=0023106341&partnerID=8YFLogxK
U2 - 10.1001/archpedi.1987.04460020060027
DO - 10.1001/archpedi.1987.04460020060027
M3 - Article
C2 - 3468801
AN - SCOPUS:0023106341
SN - 0002-922X
VL - 141
SP - 170
EP - 174
JO - American Journal of Diseases of Children
JF - American Journal of Diseases of Children
IS - 2
ER -