Transient Insulin Resistance in Propionic Acidaemia: Knowing is half the battle

Propionic acidaemia (PPA) is a disorder of amino acid and odd-chain fatty acid metabolism. Hypoglycaemia is a more commonly described finding rather than hyperglycaemia during metabolic decompensation of PPA. There is a high mortality rate in patients with organic acidaemias having severe insulin-resistant hyperglycaemia. We report a nine-month-old boy with PPA who was admitted to tertiary care hospital in Muscat, Oman, in 2018 with metabolic decompensation, persistent hyperglycaemia and transient insulin resistance. Hyperglycaemia did not respond to high insulin infusion. Plasma glucose only improved when glucose infusion rate (GIR) reached 7 mg/kg/min. The patient has full recovery and was discharged, with follow up plan. It is important to balance the GIR to achieve the targeted insulin level, beyond which the risks of hyperglycaemia start to outweigh the potential anabolic benefits of additional insulin secretion. Timely clinical attention should be given to achieve adequate caloric delivery through alternative sources other than high GIR to permit better glycaemic control, especially when insulin-resistant hyperglycaemia is present.