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intolerancia fruktózy

Record number	d005633
Date	06.06.2025
Type	M - MESH
Topical term	intolerancia fruktózy
Other term	Slovak (Pseudonym) neznášanlivosť fruktózy
See also	(Skutočné meno) fruktózabisfosfátaldoláza (Later heading) fruktózabisfosfátaldoláza
UDC	C16.320.565.202.251.271C18.452.648.202.251.271
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.

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Number of the records: 1