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HEPATOLENTICULAR DISEASE [2 records]
Record 1 - internal organization data 2019-02-13
Record 1, English
Record 1, Subject field(s)
- Human Diseases
- Genetics
Record 1, Main entry term, English
- Wilson disease
1, record 1, English, Wilson%20disease
correct
Record 1, Abbreviations, English
Record 1, Synonyms, English
- Wilson’s disease 2, record 1, English, Wilson%26rsquo%3Bs%20disease
correct
- hepatolenticular degeneration 3, record 1, English, hepatolenticular%20degeneration
correct
- hepato-lenticular degeneration 4, record 1, English, hepato%2Dlenticular%20degeneration
correct
- hepatolenticular disease 5, record 1, English, hepatolenticular%20disease
correct
- hepato-lenticular disease 6, record 1, English, hepato%2Dlenticular%20disease
correct
Record 1, Textual support, English
Record number: 1, Textual support number: 1 DEF
... a rare genetic disorder characterized by excess copper stored in various body tissues, particularly the liver, brain, and corneas of the eyes [that] is progressive and, if left untreated, it may cause liver (hepatic) disease, central nervous system dysfunction, and death. 7, record 1, English, - Wilson%20disease
Record 1, French
Record 1, Domaine(s)
- Maladies humaines
- Génétique
Record 1, Main entry term, French
- maladie de Wilson
1, record 1, French, maladie%20de%20Wilson
correct, feminine noun
Record 1, Abbreviations, French
Record 1, Synonyms, French
- dégénérescence hépatolenticulaire 2, record 1, French, d%C3%A9g%C3%A9n%C3%A9rescence%20h%C3%A9patolenticulaire
correct, feminine noun
Record 1, Textual support, French
Record number: 1, Textual support number: 1 DEF
[...] affection héréditaire, autosomale récessive de l'excrétion hépatique du cuivre, amenant à une accumulation toxique de cuivre dans le foie ainsi que le cerveau, la cornée et d'autres organes. 3, record 1, French, - maladie%20de%20Wilson
Record 1, Key term(s)
- dégénérescence hépato-lenticulaire
Record 1, Spanish
Record 1, Textual support, Spanish
Record 2 - internal organization data 2006-05-24
Record 2, English
Record 2, Subject field(s)
- Medication
Record 2, Main entry term, English
- penicillamine
1, record 2, English, penicillamine
correct
Record 2, Abbreviations, English
Record 2, Synonyms, English
- Cuprimine 2, record 2, English, Cuprimine
trademark
- Depen 2, record 2, English, Depen
trademark
Record 2, Textual support, English
Record number: 2, Textual support number: 1 OBS
A degradation product of penicillin; a chelating agent used in the treatment of lead poisoning, hepatolenticular degeneration, and cystinuria, and in the removal of excess copper in Wilson disease. 3, record 2, English, - penicillamine
Record 2, French
Record 2, Domaine(s)
- Médicaments
Record 2, Main entry term, French
- pénicillamine
1, record 2, French, p%C3%A9nicillamine
correct, feminine noun
Record 2, Abbreviations, French
Record 2, Synonyms, French
- Cuprimine 2, record 2, French, Cuprimine
trademark
- Depen 2, record 2, French, Depen
trademark
Record 2, Textual support, French
Record number: 2, Textual support number: 1 CONT
La maladie de Wilson est une maladie héréditaire rare qui provoque une surcharge en cuivre, responsable de troubles neurologiques et hépatiques qui apparaissent dans l'enfance ou dans l'adolescence. Le traitement de référence est la pénicillamine, un chélateur du cuivre, généralement capable de faire régresser en quelques mois les troubles cliniques lorsqu'ils ne sont pas trop sévères et de résorber la surcharge en cuivre. 3, record 2, French, - p%C3%A9nicillamine
Record 2, Spanish
Record 2, Campo(s) temático(s)
- Medicamentos
Record 2, Main entry term, Spanish
- penicilamina
1, record 2, Spanish, penicilamina
feminine noun
Record 2, Abbreviations, Spanish
Record 2, Synonyms, Spanish
Record 2, Textual support, Spanish
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