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DeCS
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Descriptor English:
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Fructose Intolerance
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Descriptor Spanish:
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Intolerancia a la Fructosa
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Descriptor Portuguese:
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Intolerância à Frutose
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Tree Number:
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C16.320.565.202.251.271
C18.452.648.202.251.271
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Definition English:
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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. |
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Indexing Annotation English:
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an inborn error of fructose metab; do not use /congen & do not coord with INFANT, NEWBORN, DISEASES
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See Related English:
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Fructose-Bisphosphate Aldolase
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History Note English:
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1991(1975); use FRUCTOSE METABOLISM, INBORN ERRORS 1989-1990; use CARBOHYDRATE METABOLISM, INBORN ERRORS 1964-1988
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Allowable Qualifiers English:
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Record Number:
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24002
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Unique Identifier:
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D005633
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Occurrence in VHL:
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Similar:
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DeCS CID-10 SciELO LILACS LIS
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