Function
- In thiamine pyrophosphate (TPP), a cofactor for several dehydrogenase enzyme reactions (Be APT):
- Branched-chain ketoacid dehydrogenase
- α-Ketoglutarate dehydrogenase (TCA cycle)
- Pyruvate dehydrogenase (links glycolysis to TCA cycle)
- Transketolase (HMP shunt)
Deficiency
- Impaired glucose breakdown → ATP depletion, worsened by glucose infusion; highly aerobic tissues (e.g., brain, heart) are affected first. In patients with chronic alcohol overuse or malnutrition, give thiamine before dextrose to reduce the risk of precipitating Wernicke encephalopathy.
- Diagnosis is made by increased RBC transketolase activity following vitamin B₁ administration.
Syndromes caused by deficiency
Disorder | Characteristics |
Wernicke encephalopathy | Acute, reversible, life-threatening neurologic condition. Symptoms: Confusion, Ophthalmoplegia/Nystagmus, Ataxia (CorONA beer). |
Korsakoff syndrome | Amnestic disorder due to chronic alcohol overuse; presents with confabulation, personality changes, memory loss (permanent). |
Wernicke-Korsakoff syndrome | Damage to medial dorsal nucleus of thalamus, mammillary bodies. Presentation is combination of Wernicke encephalopathy and Korsakoff syndrome. |
Dry beriberi | Polyneuropathy, symmetric muscle wasting. Spell beriberi as Ber1Ber1 to remember vitamin B₁. |
Wet beriberi | High-output cardiac failure (due to systemic vasodilation). Spell beriberi as Ber1Ber1 to remember vitamin B₁. |