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
Hereditary coproporphyria (HCP) Autosomal dominant
Defect / target: Coproporphyrinogen oxidase - step 6 (mitochondrial)
Mechanism: Block in conversion of coproporphyrinogen III to protoporphyrinogen IX causes coproporphyrin to accumulate after ring closure while upstream ALA/PBG rise during derepressed ALAS1 states, giving both cutaneous and neurovisceral features.
↑ Accumulates: Coproporphyrinogen/coproporphyrin III; ALA and PBG during attacks· ↓ Deficient: Protoporphyrinogen IX and heme
Presentation: Acute neurovisceral attacks (abdominal pain, neuropathy, psychiatric symptoms) precipitated by barbiturates/ethanol/drugs PLUS photosensitive skin lesions
Labs: Elevated urinary ALA/PBG in attacks; elevated fecal/urinary coproporphyrin III
Treatment: Hemin, glucose, avoid precipitating drugs, sun protection
HCP and VP straddle both zones - the exception board-writers love
Learn the mechanism: Heme synthesis (porphyrias) →
FMK 08.4 Heme Metabolism · slide 11, 14, 30; handout