Fatty acids

Question 2

Findings of Beta - oxidation disorder :

Answer 2

Elevated liver enzymes, Hyperammonemia, hypoglycemia and hypoketosis;

Question 3

Carnitine is required to shuttle fatty acids into the mitochondria.

Answer 3

TRUE

Question 4

In a carnitine deficiency, serum carnitine is low (absence of carnitine) and acylcarnitine levels are also low.

Answer 4

True

Question 5

In a beta-oxidation enzymatic defect, ******* accumulates.

Answer 5

Acylcarnitines.

Question 6

In this disorder, impaired membrane transport prevents carnitine uptake by cells. Carnitine is also lost in the urine leading to low serum carnitine levels. Babies with this condition develop hypoketotic hypoglycemia, liver failure, and hyperammonemia (secondary to liver failure). Symptoms worsen during fasting when fatty acid metabolism is required for fuel.

Answer 6

Primary carnitine deficiency

Question 7

RBCs and *** dont use Fatty acids as a source of energy.

Answer 7

Brain

RBCs: Glycolysis only
Brain Glucose and Ketones only