Genetics of Pyruvate Carboxylase Deficiency Medication

Updated: Mar 15, 2019
  • Author: Richard E Frye, MD, PhD; Chief Editor: Luis O Rohena, MD, MS, FAAP, FACMG  more...
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Medication

Medication Summary

Sodium dichloroacetate is a compound that is believed to activate the PDC by inhibiting the inactivating kinase, resulting in decreased lactate production and promotion of pyruvate oxidation.

Acute decompensation during illness in patients with PCD requires management of the acidosis with hydration and intravenous bicarbonate.

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Enzyme activator

Class Summary

Dichloroacetate (DCA) sodium is the only drug found to activate the enzyme complex.

Sodium dichloroacetate

Designated as an orphan drug in the United States. Used to treat lactic acidosis. This is a compound that is believed to activate the PDC by inhibiting the inactivating kinase, resulting in decreased lactate production and promotion of pyruvate oxidation.

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Alkalinizing agents

Class Summary

Sodium bicarbonate is used as a gastric, systemic, and urinary alkalinizer and has been used in the treatment of acidosis resulting from metabolic and respiratory causes, including diabetic coma, diarrhea, kidney disturbances, and shock. Sodium bicarbonate also increases renal clearance of acidic drugs.

Sodium bicarbonate

Bicarbonate can be used to correct the acidosis in chronic and acute settings.

Citrate solutions (Bicitra, Polycitra)

Several solutions containing citrate with sodium or potassium or both are available as alkalinizing agents. With normal hepatic function, 1 mEq of citrate is converted to 1 mEq of bicarbonate.

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