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Titolo:
USE OF DESFERRIOXAMINE IN THE TREATMENT OF ACERULOPLASMINEMIA
Autore:
MIYAJIMA H; TAKAHASHI Y; KAMATA T; SHIMIZU H; SAKAI N; GITLIN JD;
Indirizzi:
WASHINGTON UNIV,SCH MED,ST LOUIS CHILDRENS HOSP,DEPT PEDIAT,1 CHILDRENS PL ST LOUIS MO 63110 WASHINGTON UNIV,SCH MED,EDWARD MALLINCKRODT DEPT PEDIAT ST LOUIS MO 63110 HAMAMATSU UNIV,SCH MED,DEPT MED 1 HAMAMATSU SHIZUOKA 43131 JAPAN
Titolo Testata:
Annals of neurology
fascicolo: 3, volume: 41, anno: 1997,
pagine: 404 - 407
SICI:
0364-5134(1997)41:3<404:UODITT>2.0.ZU;2-C
Fonte:
ISI
Lingua:
ENG
Soggetto:
HEREDITARY CERULOPLASMIN DEFICIENCY; BLEPHAROSPASM; HEMOSIDEROSIS; METABOLISM;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
15
Recensione:
Indirizzi per estratti:
Citazione:
H. Miyajima et al., "USE OF DESFERRIOXAMINE IN THE TREATMENT OF ACERULOPLASMINEMIA", Annals of neurology, 41(3), 1997, pp. 404-407

Abstract

Aceruloplasminemia is a newly recognized autosomal recessive disorderof iron metabolism resulting in neurodegeneration of the retina and basal ganglia. We report here on the treatment of a patient who developed progressive extrapyramidal symptoms that included blepharospasm, grimacing, and rigidity associated with increased iron deposition in thebrain and visceral organs. Treatment for 10 months with the iron chelator desferrioxamine decreased brain iron stores, prevented progression of the neurological symptoms, and reduced plasma lipid peroxidation. These data suggest that early treatment with this chelator may be useful in such patients to diminish central nervous system iron accumulation and to prevent or ameliorate neurological symptoms associated withneurodegeneration.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 02/06/20 alle ore 01:50:55