Learn / FMK
Every FMK condition
299 diseases, deficiencies, toxicities and drug mechanisms harvested from every FMK deck — the ones sprinkled through a single bullet as much as the ones with their own slide. Pick what you're given and what you have to name, or let it cycle; distractors come from the same category so you have to discriminate (hypoketotic vs. ketotic hypoglycemia, which GSD, which urea-cycle enzyme…). Every answer shows the whole card with the lecture and slide.
Lipid · FMK 05.6
VLCAD deficiency Autosomal recessive
Defect / target: Very-long-chain acyl-CoA dehydrogenase
Mechanism: Very-long-chain fatty acids cannot begin mitochondrial β-oxidation, causing MCAD-like fasting hypoketotic hypoglycemia plus energy failure in heart and muscle.
↑ Accumulates: very-long-chain acylcarnitines (C14–C18; textbook)· ↓ Deficient: ketones, glucose, ATP in heart/muscle
Presentation: MCAD-like hypoketotic hypoglycemia plus cardiomyopathy and rhabdomyolysis
Labs: acylcarnitine profile on newborn screen
Unifying pattern of mitochondrial FAO disorders: fasting HYPOKETOTIC hypoglycemia
Learn the mechanism: Lipolysis, carnitine shuttle & β-oxidation →
FMK 05.6 Fatty Acid Metabolism II · slide 15, 18