Abstract: FR-PO1014
Carnitine Deficiency as a Metabolic Contributor to Recurrent Hypoglycemia in an Adolescent on Maintenance Hemodialysis
Session Information
- Hemodialysis: Clinical Challenges, Patient-Centered Outcomes, and Quality of Life
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Dialysis
- 801 Dialysis: Hemodialysis and Frequent Dialysis
Authors
- Vohra, Vanya, Lucile Salter Packard Children's Hospital at Stanford, Palo Alto, California, United States
- Wong, Cynthia, Lucile Salter Packard Children's Hospital at Stanford, Palo Alto, California, United States
Introduction
Hemodialysis removes 60-80% of plasma carnitine per session leading to carnitine deficiency which is often overlooked in pediatric patients. We present a case of recurrent severe hypoglycemia in a 16-year-old male on maintenance hemodialysis with concurrent carnitine deficiency.
Case Description
A 16-year-old male with VACTERL association, esophageal atresia s/p repair, and end-stage kidney disease on thrice-weekly hemodialysis presented with recurrent severe hypoglycemia. During routine hemodialysis, he developed shakiness, weakness, and collapse with altered responsiveness. Point-of-care glucose was 22 mg/dL and improved after gastrostomy-administered sugar water and intravenous dextrose. Four days later, continuous glucose monitoring alerted for recurrent hypoglycemia with fingerstick glucose of 45 mg/dL while he remained asymptomatic and was admitted for further evaluation. A supervised 24-hour fast demonstrated preserved fasting glucose homeostasis with glucose >60 mg/dL. However, after resumption of bolus gastrostomy feeds and high-carbohydrate snacks, he developed hypoglycemia to 47 mg/dL with elevated insulin levels, consistent with postprandial reactive hyperinsulinism. Metabolic evaluation revealed severe secondary carnitine deficiency with free carnitine 8.6 µmol/L and elevated acylcarnitine/free carnitine ratio. Review of prior records demonstrated persistent carnitine deficiency despite oral supplementation, suggesting inadequate replacement in the setting of chronic dialytic losses. Interventions included reduction of simple sugars, incorporation of complex carbohydrates and cornstarch supplementation, and escalation of L-carnitine replacement.
Discussion
This case illustrates the complex interplay between hemodialysis, carnitine deficiency, and hypoglycemia. While primary hypoglycemia mechanism was post-prandial hyperinsulinism from rapid carbohydrate absorption, concurrent carnitine deficiency likely amplified metabolic vulnerability. L-carnitine enables long-chain fatty acid transport into mitochondria for beta-oxidation, generating ketones as alternative cerebral fuel and supporting hepatic gluconeogenesis. Carnitine deficiency impairs these pathways, potentially exacerbating hypoglycemia severity and limiting counter-regulatory responses. The elevated acylcarnitine/free ratio indicates blocked mitochondrial oxidation with acyl-CoA accumulation.