Fructose intolerance

Question 1

Aldolase B deficiency

Answer 1

incidence 1:20000
mutation in ALDOB, chromosome 9
F-1-P builds up
fructose no longer used for energy

Question 2

Fructose intake

Answer 2

Free fructose
sucrose
sorbitol

(fruits, vegetables, honey)

Question 3

Effects

Answer 3

hypophosphatemia
hypoglycemia
F-1-P inhibits glycogen enzymes
hepatomegaly
hepatic steatosis
hyperurisemia
hypermagnesemia
cell necrosis (liver, kidney)

Question 4

Hypoglycemia

Answer 4

no aldolase B - glucokinase stays in cytoplasm - glycogenolysis/gluconeogenesis slows down - glucose release to blood slows down - hypoglycemia

Question 5

Hyperuricemia

Answer 5

ATP depletion, Pi depletion enhance- purine nucleotide - xanthine - uric acid - hyperuricemia

Question 6

Essential fructosuria

Answer 6

fructokinase deficiency
high fructose in blood - fructosemia
unmetabolized fructose to urin - fructosuria
asymptomatic
no treatment