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.
Heme · FMK 08.4
Variegate porphyria (VP) Autosomal dominant
Defect / target: Protoporphyrinogen oxidase - step 7 (mitochondrial)
Mechanism: Failure to oxidize protoporphyrinogen IX to protoporphyrin IX causes protoporphyrinogen and coproporphyrinogen to accumulate after ring closure, with ALA/PBG accumulation during induced attacks, so both photosensitivity and acute attacks occur.
↑ Accumulates: Protoporphyrinogen IX and coproporphyrinogen; ALA and PBG during attacks· ↓ Deficient: Protoporphyrin IX and heme
Presentation: Acute neurovisceral attacks precipitated by drugs (barbiturates, ethanol) plus cutaneous blistering photosensitivity
Labs: Elevated urinary ALA/PBG in attacks; elevated fecal protoporphyrin and coproporphyrin; characteristic plasma fluorescence peak
Treatment: Hemin, glucose, avoid precipitating drugs, sun protection
HCP and VP straddle both zones (acute + cutaneous)
Learn the mechanism: Heme synthesis (porphyrias) →
FMK 08.4 Heme Metabolism · slide 11, 14, 30; handout