Fructose intolerance Flashcards
1
Q
Aldolase B deficiency
A
incidence 1:20000
mutation in ALDOB, chromosome 9
F-1-P builds up
fructose no longer used for energy
2
Q
Fructose intake
A
Free fructose
sucrose
sorbitol
(fruits, vegetables, honey)
3
Q
Effects
A
hypophosphatemia hypoglycemia F-1-P inhibits glycogen enzymes hepatomegaly hepatic steatosis hyperurisemia hypermagnesemia cell necrosis (liver, kidney)
4
Q
Hypoglycemia
A
no aldolase B - glucokinase stays in cytoplasm - glycogenolysis/gluconeogenesis slows down - glucose release to blood slows down - hypoglycemia
5
Q
Hyperuricemia
A
ATP depletion, Pi depletion enhance- purine nucleotide - xanthine - uric acid - hyperuricemia
6
Q
Essential fructosuria
A
fructokinase deficiency high fructose in blood - fructosemia unmetabolized fructose to urin - fructosuria asymptomatic no treatment
