Glucose and leucine kinetics in idiopathic ketotic hypoglycaemia.

Arch Dis Child

Biochemical Genetics and National Neonatal Screening Laboratories, University Children's Hospital Vienna, Vienna, Austria.

Published: June 2006

AI Article Synopsis

  • The study aimed to understand how glucose and leucine metabolism is affected in children with ketotic hypoglycemia (KH) and its underlying causes.
  • Compared to a control group, children with KH exhibited lower insulin levels, higher ketone body levels, and a higher basal metabolic rate, indicating a distinct metabolic profile.
  • The findings suggest that KH results from an inability to maintain glucose production in the liver, along with increased energy demands and decreased leucine oxidation rates.

Article Abstract

Aims: To investigate glucose and leucine kinetics in association with metabolic and endocrine investigations in children with ketotic hypoglycaemia (KH) in order to elucidate the underlying pathophysiology.

Methods: Prospective interventional study using stable isotope tracer in nine children (mean age 4.23 years, range 0.9-9.8 years; seven males) with KH and 11 controls (mean age 4.57 years, range 0.16-12.3 years; four males).

Results: Plasma insulin levels were significantly lower in KH compared to subjects in the non-KH group. Plasma ketone body levels were significantly higher in KH than in non-KH. Basal metabolic rate was significantly higher in subjects with KH (45.48+/-7.41 v 31.81+/-6.72 kcal/kg/day) but the respiratory quotients were similar in both groups (KH v non-KH, 0.84+/-0.05 v 0.8+/-0.04. Leucine oxidation rates were significantly lower in children with KH (12.25+/-6.25 v 31.96+/-8.59 micromol/kg/h). Hepatic glucose production rates were also significantly lower in KH (3.84+/-0.46 v 6.6+/-0.59 mg/kg/min).

Conclusions: KH is caused by a failure to sustain hepatic glucose production rather than by increased glucose oxidation rates. Energy demand is significantly increased, whereas leucine oxidation is reduced.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2082774PMC
http://dx.doi.org/10.1136/adc.2005.089425DOI Listing

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