Propionic acidaemia (PPA) is a disorder of amino acid and odd-chain fatty acid metabolism. Hypoglycemia is a commonly described finding rather than hyperglycemia during metabolic decompansation of PPA. We describe 9 months old boy with PPA, who was admitted with metabolic decompansation, persistent hyperglycemia and transient insulin resistance. Hyperglycemia did not respond to high insulin infusion. Plasma glucose only improved when glucose infusion rate (GIR) reached 7 mg/kg/min. The literature review showed high mortality rate in patients with organic acidaemias, with severe insulin-resistant hyperglycaemia. An important challenge is the balance between enough GIR to achieve targeted insulin level beyond which risks of hyperglycemia start to outweigh potential anabolic benefits of additional insulin secretion. Given the few case reports published, timely clinical attention should be made to achieve adequate caloric delivery through alternative sources other than high GIR to permit for better glycemic control, especially when insulin-resistant hyperglycemia is present.

Propionic acidaemia (PPA) is a disorder of amino acid and odd-chain fatty acid metabolism. Hypoglycemia is a commonly described finding rather than hyperglycemia during metabolic decompansation of PPA. We describe 9 months old boy with PPA, who was admitted with metabolic decompansation, persistent ...