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.
Carbohydrate · FMK 08.1
Hereditary fructose intolerance (secondary hyperuricemia) Autosomal recessive
Defect / target: Aldolase B deficiency
Mechanism: Fructose-1-phosphate is trapped, sequestering phosphate and depleting ATP; the resulting AMP degradation drives secondary purine breakdown and uric acid overproduction.
↑ Accumulates: Fructose-1-phosphate, uric acid· ↓ Deficient: Intracellular phosphate and ATP
Presentation: Hypoglycemia, vomiting, hepatomegaly after fructose/sucrose ingestion; hyperuricemia/gout as a secondary purine-overproduction state (per lecture)
Labs: reducing sugar in urine, negative glucose oxidase, elevated LFTs, hypoglycemia
Treatment: Avoid fructose, sucrose, sorbitol
Listed with von Gierke as 'unrelated metabolic diseases with secondary purine overproduction'
Learn the mechanism: Fructose metabolism →Reasoning case →
FMK 08.1 Nucleic Acid Metabolism; FMK 03.4 Fructose, Galactose & Ethanol Metabolism · slide 08.1: 19; 03.4: 6, 8, 23, 24, 25, 26