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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
1
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pubmed:dateCreated |
1982-4-22
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pubmed:abstractText |
We have studied four children (ages 6 to 17 years) with chronic cholestasis who developed a slowly progressive neuromuscular disease characterized by ataxia, dysmetria, areflexia, loss of vibratory sensation, and a variable ophthalmoplegia. Serum vitamin E concentration were low in all patients prior to treatment. Muscle histochemical studies in all four patients showed autofluorescent basophilic esterase and acid phosphatase-positive cytoplasmic inclusions and occasional necrotic fibers. These distinctive muscle changes are similar to those described in vitamin E-deficient animals. Intramuscular injections of alpha tocopherol were required in three patients to achieve normal serum vitamin E values. High-dose oral supplementation was effective in one patient. After normalization of serum vitamin E concentrations for six to 14 months, the neurologic disease has improved in all four patients.
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pubmed:grant | |
pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
AIM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Jan
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pubmed:issn |
0022-3476
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
100
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
51-8
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pubmed:dateRevised |
2007-11-14
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pubmed:meshHeading |
pubmed-meshheading:7057316-Adolescent,
pubmed-meshheading:7057316-Child,
pubmed-meshheading:7057316-Child, Preschool,
pubmed-meshheading:7057316-Cholestasis,
pubmed-meshheading:7057316-Humans,
pubmed-meshheading:7057316-Infant,
pubmed-meshheading:7057316-Injections, Intramuscular,
pubmed-meshheading:7057316-Muscles,
pubmed-meshheading:7057316-Neuromuscular Diseases,
pubmed-meshheading:7057316-Vitamin E,
pubmed-meshheading:7057316-Vitamin E Deficiency
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pubmed:year |
1982
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pubmed:articleTitle |
Progressive neuromuscular disease in children with chronic cholestasis and vitamin E deficiency: diagnosis and treatment with alpha tocopherol.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
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