• J Coll Physicians Surg Pak · Jan 2023

    Case Reports

    Anaesthetic Management of a Child with Propionic Acidemia.

    • Sheema Siraj and Fauzia Khan.
    • Department of Anaesthesiology, Patel Hospital, Karachi, Pakistan.
    • J Coll Physicians Surg Pak. 2023 Jan 1; 33 (1): 232-3.

    AbstractPropionic acidemia (PA) is an inherited autosomal recessive disorder of metabolism caused by a deficiency of propionyl CoA carboxylase, an enzyme that catalyses the conversion of propionyl CoA (PCA) to methylmalonyl CoA, inside the mitochondria, leading to inadequate metabolism of propionyl CoA causing hyperammonemia and metabolic acidosis. Children with PA require dextrose infusion to avoid protein catabolism. This child presented with severe metabolic decompensation and required urgent venous cutdown as there was a failure in establishing a peripheral intravenous line. Key Words: Propionic acidemia, Propionyl CoA, Emergency, Mitochondrial disorder, Organic acidemias.

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