ONKI3 | Medical Subject Headings

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Fructose Intolerance

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Hierarchy:	Medical Subject Headings (MeSH) ∟ Congenital, Hereditary, and Neonatal Diseases and Abnormalities ∟ Genetic Diseases, Inborn ∟ Metabolism, Inborn Errors ∟ Carbohydrate Metabolism, Inborn Errors ∟ Fructose Metabolism, Inborn Errors ∟ Fructose Intolerance 1 coordinate concept ∟ Fructose-1,6-Diphosphatase Deficiency Medical Subject Headings (MeSH) ∟ Nutritional and Metabolic Diseases ∟ Metabolic Diseases ∟ Metabolism, Inborn Errors ∟ Carbohydrate Metabolism, Inborn Errors ∟ Fructose Metabolism, Inborn Errors ∟ Fructose Intolerance 1 coordinate concept ∟ Fructose-1,6-Diphosphatase Deficiency X
Broader terms:	Medical Subject Headings (MeSH) ∟ Congenital, Hereditary, and Neonatal Diseases and Abnormalities ∟ Genetic Diseases, Inborn ∟ Metabolism, Inborn Errors ∟ Carbohydrate Metabolism, Inborn Errors ∟ Fructose Metabolism, Inborn Errors ∟ Fructose Intolerance Medical Subject Headings (MeSH) ∟ Nutritional and Metabolic Diseases ∟ Metabolic Diseases ∟ Metabolism, Inborn Errors ∟ Carbohydrate Metabolism, Inborn Errors ∟ Fructose Metabolism, Inborn Errors ∟ Fructose Intolerance Fructose Metabolism, Inborn Errors > Carbohydrate Metabolism, Inborn Errors > Metabolism, Inborn Errors > Genetic Diseases, Inborn > Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Medical Subject Headings (MeSH) Fructose Metabolism, Inborn Errors > Carbohydrate Metabolism, Inborn Errors > Metabolism, Inborn Errors > Metabolic Diseases > Nutritional and Metabolic Diseases > Medical Subject Headings (MeSH) X
Annotation:	an inborn error of fructose metab; do not use /congen & do not coord with INFANT, NEWBORN, DISEASES X
historyNote*:	91(75)(64-66); was see under FRUCTOSE METABOLISM, INBORN ERRORS 1989-90; was see under CARBOHYDRATE METABOLISM, INBORN ERRORS 1964-88 (Prov 1964-66) X
onlineNote*:	use CARBOHYDRATE METABOLISM, INBORN ERRORS to search FRUCTOSE INTOLERANCE 1966-74 (as Prov 1966) X
publicMeSHNote*:	91; was see under FRUCTOSE METABOLISM, INBORN ERRORS 1989-90; was see under CARBOHYDRATE METABOLISM, INBORN ERRORS 1967-88 X
Scope note:	An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (EC 2.1.2.13) activity, resulting in accumulation of fructose-1-phosphate. The accumulated fructose-1-phosphate inhibits glycogenolysis and gluconeogenesis, causing severe hypoglycemia following ingestion of fructose. Prolonged fructose ingestion in infants leads ultimately to hepatic failure and death. Patients develop a strong distaste for sweet food, and avoid a chronic course of the disease by remaining on a fructose- and sucrose-free diet. X
activeMeSHYear*:	2007X
dateCreated*:	1999-01-01X
dateEstablished*:	1991-01-01X
dateRevised*:	2006-07-05X
recordAuthorizer*:	sjnX
recordMaintainer*:	lktX
recordOriginator*:	NLMX
Related term:	Fructose-Bisphosphate Aldolase X
Type:	Concept X
Coordinate terms:	Fructose-1,6-Diphosphatase Deficiency X
URI:	X
Labels and equivalent concepts:	Fruktoosi-intoleranssi (fi) Fruktoosinsietokyvyttömyys (fi, replaced) Fruktosintolerans (sv) Intolerantia fructosi (la-FI, replaced) X
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