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Fructosuria: Fructosuria,, disturbance of fructose metabolism resulting from a hereditary disorder or intolerance. Normally, fructose is first metabolized in the body. Full text. Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (K), or click on a page. A collection of disease information resources and questions answered by our Genetic and Rare Diseases Information Specialists for Fructosuria.

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Normally, fructose is first metabolized in the body to fructosephosphate by a specific organic catalyst or enzyme called fructokinase. Contact our editors with your feedback. From Wikipedia, the free encyclopedia. The diagnosis of hereditary fructose intolerance. Urine samples from the brother and 2 sisters fductosuria intermittent fructosuria.

At the bottom of the article, feel free to list any sources that support your changes, so that we can frucrosuria understand their context. By using this site, you agree to the Terms of Use and Privacy Policy. Using 31 P magnetic resonance spectroscopy to measure changes in liver metabolite concentrations in adults with fructosuria, Boesiger et al.


Laron counted 50 published cases, of which 18 were in Jews.

CC ]. The fructose tolerance test was normal and fructosuria persisted after fructose was entirely excluded from the diet, but had decreased frutosuria when the patient was seen 2 years later.

D ICD – Essential fructosuria is a fructosuris, asymptomatic defect of intermediary metabolism characterized by the intermittent appearance of fructose in the urine summary by Bonthron et al. Disorders of carbohydrate metabolism. The enzyme deficiency was demonstrated in liver by Schapira et al.

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Retrieved from ” https: The patient’s parents were first cousins. This page was last edited on 24 Aprilat Congenital alactasia Sucrose intolerance.

Keep Exploring Britannica Evolution. The Editors of Encyclopaedia Britannica. Diagnosis and Treatment 5th ed.

Fructosuria | disease |

In fructosuria this particular enzyme is defective, and the concentration of fructose increases in the blood and urine. Views Read Edit View history.


Sign up for our Demystified newsletter and get this free guide. Fructokinase deficiency Ketohexokinase deficiency Prevalence: Unfortunately, our editorial approach may not be able to accommodate all contributions.

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Essential fructosuria was first described independently by Czapek and Zimmer in a man who also suffered from diabetes mellitus. Khachadurian described nonalimentary fructosuria in an month-old Arab boy who frucosuria from sickle-cell thalassemia. Learn More in these related Britannica articles: Internet URLs are the best.

Disorders of fructose metabolism.

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Levulose im Harn eines Diabetikers. Disaccharide catabolism Congenital alactasia Sucrose intolerance. Inborn error of carbohydrate metabolism: Thank You for Your Contribution!