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Glucose and leucine kinetics in idiopathic ketotic hypoglycaemia

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 μmol/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.

  • BMI, body mass index
  • BMR, basal metabolic rate
  • GC-MS, gas chromatography-mass spectrometry
  • KH, ketotic hypoglycaemia
  • KIC, ketoisocaproic acid
  • NEFA, non-esterified fatty acids
  • NHS, National Health Service
  • RQ, respiratory quotient
  • MAT, mitochondrial acetoacetyl CoA thiolase
  • MSUD, maple syrup urine disease
  • SCOT, succinyl CoA oxoacid transferase
  • SD, standard deviation
  • tracer
  • isotope
  • ketone body
  • mass spectrometry
  • energy expenditure

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