USMLE (Fach) / Biochemistry - Nutrition (Lektion)
In thiamine pyrophosphate (TPP), a cofactor for several dehydrogenase enzyme reactions:- Pyruvate dehydrogenase (links glycolysis to TCA cycle)- α-ketoglutarate dehydrogenase (TCA cycle)- Transketolase (HMP shunt)- Branched-chain ketoacid dehydrogenase
Deficiency- Impaired glucose breakdown → ATP depletion worsened by glucose infusion; highly aerobic tissues (eg, heart, brain) are affected first.- In alcoholic or malnourished patients, give thiamine before dextrose to ↓ risk of precipitating Wernicke encephalopathy.- Diagnosis made by ↑ in RBC transketolase activity following vitamin B1 administration.
Wernicke-Korsakoff syndrome:- Triad: confusion + vision changes (ophthalmoplegia, nystagmus) + ataxia- Confabulation- Personality change- Memory loss (permanent)Damage to medial dorsal nucleus of the thalamus, mammillary bodies
Wet beribery – high-output cardiac failure (dilated cardiomyopathy), edemaDry beriberi – polyneuropathy, symmetrical muscle wasting
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