FFA: free fatty acid; MCADD: medium-chain acyl-CoA dehydrogenase deficiency; VLCADD: very long-chain acyl-CoA dehydrogenase deficiency; GSD: glycogen storage disease; F-1,6-Pase: fructose-1,6-bisphosphatase; PC: pyruvate carboxylase.
* A glucagon stimulation test supports the diagnosis of an insulin-mediated disorder. In a hypoglycemic patient, a rise in blood glucose of ≥30 mg/dL in response to glucagon is inappropriate, indicates excessive glycogen reserves, and provides indirect evidence of an insulin-mediated disorder. For a protocol for a diagnostic fast and glucagon stimulation test, refer to UpToDate content on hypoglycemia in children.
¶ Some ketotic hypoglycemic disorders may not have detectable acidemia, especially idiopathic ketotic hypoglycemia, growth hormone deficiency, and cortisol deficiency.Do you want to add Medilib to your home screen?