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Disorders of Fructose Metabolism


Steinmann, B; Santer, R (2012). Disorders of Fructose Metabolism. In: Saudubray, G; van den Berghe, G; Walter, J H. Inborn Metabolic Diseases - Diagnosis and Treatment. Berlin: Springer, 157-165.

Abstract

Three inborn errors are known in the pathway of fructose metabolism depicted in Fig. 9.1. Essential fructosuria is a harmless anomaly characterised by the appearance of fructose in the urine after the intake of fructose-containing food. In hereditary fructose intolerance (HFI), fructose may provoke prompt gastrointestinal discomfort and hypoglycaemia upon ingestion, symptoms that may vary from patient to patient and depend on the ingested dose. Fructose may cause liver and kidney failure when taken persistently, and its intake becomes life threatening when it is given intravenously. Fructose-1,6-bisphosphatase (FBPase) deficiency is also usually considered an inborn error of fructose metabolism although, strictly speaking, it is a defect of gluconeogenesis.

Abstract

Three inborn errors are known in the pathway of fructose metabolism depicted in Fig. 9.1. Essential fructosuria is a harmless anomaly characterised by the appearance of fructose in the urine after the intake of fructose-containing food. In hereditary fructose intolerance (HFI), fructose may provoke prompt gastrointestinal discomfort and hypoglycaemia upon ingestion, symptoms that may vary from patient to patient and depend on the ingested dose. Fructose may cause liver and kidney failure when taken persistently, and its intake becomes life threatening when it is given intravenously. Fructose-1,6-bisphosphatase (FBPase) deficiency is also usually considered an inborn error of fructose metabolism although, strictly speaking, it is a defect of gluconeogenesis.

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Item Type:Book Section, not refereed, further contribution
Communities & Collections:04 Faculty of Medicine > University Children's Hospital Zurich > Medical Clinic
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:2012
Deposited On:20 Feb 2013 13:43
Last Modified:05 Apr 2016 16:31
Publisher:Springer
Publisher DOI:https://doi.org/10.1007/978-3-642-15720-2_9
Related URLs:http://opac.nebis.ch/F/?local_base=NEBIS&CON_LNG=GER&func=find-b&find_code=SYS&request=006871102

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