| Progressive neuromuscular disease in children with chronic cholestasis and vitamin E deficiency: diagnosis and treatment with alpha tocopherol. | |
| | |
MedLine Citation:
|
PMID: 7057316 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
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. |
| | |
Authors:
|
M A Guggenheim; S P Ringel; A Silverman; B E Grabert |
Related Documents
:
|
20640216 - Patients with symptomatic primary hyperparathyroidism: an anaesthetic challenge. 9015486 - Primary hyperparathyroidism in a paediatric hospital. 3827466 - Association of primary hyperparathyroidism with myotonic dystrophy in two patients. 3738426 - Cobalamin and unsaturated transcobalamin values in pernicious anaemia: relation to trea... 2527376 - Dehydroepiandrosterone-sulfate/cortisol ratio in panic disorder. 20236196 - Paederus dermatitis in egypt: a clinicopathological and ultrastructural study. |
Publication Detail:
|
Type: Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
|
Title: The Journal of pediatrics Volume: 100 ISSN: 0022-3476 ISO Abbreviation: J. Pediatr. Publication Date: 1982 Jan |
Date Detail:
|
Created Date: 1982-04-22 Completed Date: 1982-04-22 Revised Date: 2007-11-14 |
Medline Journal Info:
|
Nlm Unique ID: 0375410 Medline TA: J Pediatr Country: UNITED STATES |
Other Details:
|
Languages: eng Pagination: 51-8 Citation Subset: AIM; IM |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Adolescent Child Child, Preschool Cholestasis / complications* Humans Infant Injections, Intramuscular Muscles / pathology Neuromuscular Diseases / complications*, diagnosis, drug therapy Vitamin E / administration & dosage, therapeutic use* Vitamin E Deficiency / complications* |
| Grant Support | |
ID/Acronym/Agency:
|
RR-69/RR/NCRR NIH HHS |
| Chemical | |
Reg. No./Substance:
|
1406-18-4/Vitamin E |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Long-term consequences of Reye syndrome: a sibling-matched, controlled study of neurologic, cognitiv...
Next Document: Lactose malabsorption in recurrent abdominal pain of childhood.