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Essential fructosuria, caused by a deficiency of the enzyme hepatic fructokinase, is a clinically benign condition characterized by the incomplete metabolism of fructose in the liver, leading to its excretion in urine. Fructokinase (sometimes called ketohexokinase) is the first enzyme involved in the degradation of fructose to fructose-1-phosphate in the liver.

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rdf:type
rdfs:label
  • Fructosurie (de)
  • Essential fructosuria (en)
  • Fruttosuria (it)
  • Fructosurie (nl)
  • Эссенциальная фруктозурия (ru)
rdfs:comment
  • Als Fructosurie oder Fruktosurie bezeichnet man die vermehrte Ausscheidung von Fruchtzucker (Fructose) über den Harn durch die Niere. Ursächlich ist eine Anhäufung von Fructose im Blut, die Fructosämie, weil nicht genügend Fructose im Körper abgebaut wird. (de)
  • La fruttosuria è la presenza di fruttosio nelle urine. Quantità trascurabili di fruttosio possono essere normalmente presenti nelle urine anche nel soggetto sano. La presenza in quantità più alte assume invece rilevanza clinica e può essere sintomo di diabete mellito grave. La fruttosuria, definita fruttosuria essenziale, è una condizione clinica benigna e rara caratterizzata dal mancato o ridotto metabolismo del fruttosio a livello epatico. (it)
  • Fructosurie is een toestand waarbij zich fructose in de urine bevindt. Dit is het gevolg van een tekort aan het . Dit is een erfelijke aandoening. Het is niet ernstig of gevaarlijk. Het moet niet worden verward met Fructose-intolerantie, wat wel een ernstige aandoening is. (nl)
  • Эссенциальная фруктозурия — редкий, рецессивно наследуемый дефект углеводного обмена. Заболевание связано с недостаточным синтезом фруктокиназы в печени и других тканях. Встречаемость заболевания оценивается на уровне около 1 случая на 130 тысяч человек. Другая тяжёлая форма фруктозурии обусловлена недостаточным образованием и снижением синтеза . Исследуется с 1930-х годов. (ru)
  • Essential fructosuria, caused by a deficiency of the enzyme hepatic fructokinase, is a clinically benign condition characterized by the incomplete metabolism of fructose in the liver, leading to its excretion in urine. Fructokinase (sometimes called ketohexokinase) is the first enzyme involved in the degradation of fructose to fructose-1-phosphate in the liver. (en)
foaf:name
  • Essential fructosuria (en)
name
  • Essential fructosuria (en)
foaf:depiction
  • http://commons.wikimedia.org/wiki/Special:FilePath/Fructose.svg
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thumbnail
caption
  • Fructose (en)
field
synonyms
  • Ketohexokinase deficiency (en)
has abstract
  • Als Fructosurie oder Fruktosurie bezeichnet man die vermehrte Ausscheidung von Fruchtzucker (Fructose) über den Harn durch die Niere. Ursächlich ist eine Anhäufung von Fructose im Blut, die Fructosämie, weil nicht genügend Fructose im Körper abgebaut wird. (de)
  • Essential fructosuria, caused by a deficiency of the enzyme hepatic fructokinase, is a clinically benign condition characterized by the incomplete metabolism of fructose in the liver, leading to its excretion in urine. Fructokinase (sometimes called ketohexokinase) is the first enzyme involved in the degradation of fructose to fructose-1-phosphate in the liver. This defective degradation does not cause any clinical symptoms, fructose is either excreted unchanged in the urine or metabolized to fructose-6-phosphate by alternate pathways in the body, most commonly by hexokinase in adipose tissue and muscle. (en)
  • La fruttosuria è la presenza di fruttosio nelle urine. Quantità trascurabili di fruttosio possono essere normalmente presenti nelle urine anche nel soggetto sano. La presenza in quantità più alte assume invece rilevanza clinica e può essere sintomo di diabete mellito grave. La fruttosuria, definita fruttosuria essenziale, è una condizione clinica benigna e rara caratterizzata dal mancato o ridotto metabolismo del fruttosio a livello epatico. (it)
  • Fructosurie is een toestand waarbij zich fructose in de urine bevindt. Dit is het gevolg van een tekort aan het . Dit is een erfelijke aandoening. Het is niet ernstig of gevaarlijk. Het moet niet worden verward met Fructose-intolerantie, wat wel een ernstige aandoening is. (nl)
  • Эссенциальная фруктозурия — редкий, рецессивно наследуемый дефект углеводного обмена. Заболевание связано с недостаточным синтезом фруктокиназы в печени и других тканях. Встречаемость заболевания оценивается на уровне около 1 случая на 130 тысяч человек. Другая тяжёлая форма фруктозурии обусловлена недостаточным образованием и снижением синтеза . Исследуется с 1930-х годов. (ru)
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ICD9
  • 271.2
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page length (characters) of wiki page
DiseasesDB
  • 5001
ICD10
  • E74.1
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