Fructose, Galactose, Lactose Flashcards
Essential fructosuria enzyme? What are the symptoms? Why is asymptomatic and benign?
Enzyme: fructokinase
Symptoms: fructose in urine and blood
Because fructose does not get trapped in the cells
Fructose intolerance enzyme? What is the difference between essential fructosuria and fructose intolerance? What is inhibited because of the enzyme def.? Why is it inhibited?
Enzyme: Aldolase B
Fructose is phosphorylated in Aldolase B def.
Gluconeogenesis and Glycogenlysis
Phosphate is decreased
Symptoms of Fructose intolerance will come up when you ingest what kinds of food? What will show up in the urine and will the urine show glucose?
Honey, juice, fruit
reduced sugars
No
Symptoms of Fructose intolerance?
Hygoglycemia, jaundice, cirrhosis, vomiting
Galactokinase def. enzyme? If galactose is present in the diet, what will accumulate? Symptoms?
Galactokinase
Galactitol
Failure to track objects or develop social smile
Classic galactosemia enzyme? How does this enzyme def. cause damage to organs like the lens?
galactose-1-phosphate uridyltransferase
“Classic 11”
toxic substances accumulate in the lens
Symptoms of galactosemia? In neonates, this can lead to what kind of sepsis? What two sugars do you have to exclude for these patients?
Cataracts, hepatosplenomegaly, intellectual disability, failure to thrive.
E.coli
galactose and lactose
“Fructose is to Aldolase B as Galactose is to UridylTransferase (FAB GUT)”
Classic Galactosemia and Fructose intolerance can lead to a depletion of?
phosphate
In what organs do you only have aldose reductase?
Lens, Schwann cells, retina, kidney.
Lactase is what kind of enzyme? How does gastroenteritis and autoimmune disease cause lactose intolerance? What bug can cause gastroenteritis?
brush border
Loss of brush border
Rotavirus
What will stool show in a patient with lactose def. (2 things)? What about a mucosal biopsy?
decreased pH and and increased H+ breathe (increased acid)
Normal mucosa
