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Cystinuries Et Lithiase Cystinique by Hedi Ktata-S

Cystinuries Et Lithiase Cystinique

by Hedi Ktata-S
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Book cover type: Paperback
  • ISBN13: 9783841730558
  • Binding: Paperback
  • Subject: N/A
  • Publisher: Omniscriptum
  • Publisher Imprint: Omniscriptum
  • Publication Date:
  • Pages: 132
  • Original Price: GBP 47.0
  • Language: French
  • Edition: N/A
  • Item Weight: 205 grams
  • BISAC Subject(s): General

La lithiase cystinique est l'expression clinique unique de la cystinurie, maladie héréditaire peu fréquente de transmission autosomique récessive et à pénétrance variable et qui est définie par une anomalie du transport rénal et intestinal de la cystine et des acides aminés dibasiques: ornithine, lysine, arginine. Le défaut de réabsorption tubulaire proximale entraîne une excrétion urinaire anormalement élevée de ces acides aminés. La cystine étant le moins soluble de tous les acides aminés, il en résulte la formation répétée de calculs de cystine. Un traitement médical bien suivi est capable de stopper ou du moins de réduire sensiblement la formation de calculs chez les patients cystinuriques. Le maintien d'une diurèse élevée apparaît comme une composante fondamentale du traitement. Des contrôles cliniques, radiologiques et biologiques réguliers sont indispensables pour contrôler les effets et la tolérance du traitement. La disparition des cristaux de cystine est un des meilleurs critères d'efficacité du traitement. Une information du patient est indispensable pour obtenir son adhésion à l'observance d'un traitement qui doit ètre poursuivi à vie.

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