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Glucose and leucine kinetics in idiopathic ketotic hypoglycaemia
  1. O A Bodamer1,
  2. K Hussein2,
  3. A A Morris3,
  4. C-D Langhans4,
  5. D Rating4,
  6. E Mayatepek5,
  7. J V Leonard2
  1. 1Biochemical Genetics and National Neonatal Screening Laboratories, University Children’s Hospital Vienna, Vienna, Austria
  2. 2Endocrinology, Metabolism, Nutrition & Genetics, Institute of Child Health, London, UK
  3. 3Willink Biochemical Unit, Royal Manchester Children’s Hospital, Manchester, UK
  4. 4Department of Paediatric Neurology, University Children’s Hospital, Heidelberg, Germany
  5. 5Department of General Paediatrics, University Children’s Hospital, Düsseldorf, Germany
  1. Correspondence to:
    Prof. Dr O Bodamer
    Biochemical Genetics and National Neonatal Screening Laboratories, Department of General Pediatrics, University Children’s Hospital Vienna, Währinger Gürtel 18-20, A-1090 Vienna, Austria; olaf.bodamer{at}meduniwien.ac.at

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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Footnotes

  • Published Online First 27 January 2006

  • Competing interests: none declared

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