Categorization of hypoglycemic disorders based on biochemical profile

Schematic representation of the biochemical profile obtained on the critical sample obtained during an episode of hypoglycemia. The biochemical profile helps to identify the pathophysiologic category of the underlying hypoglycemic disorder.

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.

Original figure modified for this publication. From: Thornton PS, Stanley CA, De Leon DD, et al. Recommendations from the Pediatric Endocrine Society for evaluation and management of persistent hypoglycemia in neonates, infants, and children. J Pediatr 2015; 167:238. Illustration used with the permission of Elsevier Inc. All rights reserved.

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Categorization of hypoglycemic disorders based on biochemical profile