Glucose and leucine kinetics in idiopathic ketotic hypoglycaemia

Olaf A Bodamer ^1*, Khalid Hussein ², Andrew A Morris ³, Claus-Dieter Langhans ⁴, Dietz Rating ⁴, Ertan Mayatepek ⁵ and James V Leonard ²

¹ University Children's Hospital Vienna, Austria
² Institute of Child Health London, United Kingdom
³ Royal Children's Hospital Manchester, United Kingdom
⁴ University Children's Hospital Heidelberg, Germany
⁵ University Children's Hospital Dusseldorf, Germany

^* To whom correspondence should be addressed. E-mail: olaf.bodamer{at}meduniwien.ac.at.

Accepted 16 January 2006

Abstract

Objective: Glucose and leucine kinetics were studied in association with metabolic and endocrine investigations in children with ketotic hypoglycaemia (KH) to elucidate the underlying pathophysiology. Design: Prospective interventional study using stable isotope tracer.

Patients: 9 children (age: 4.23 years, 0.9-9.8; mean and range, 7 males) with KH and 11 children (4.57 years, 0.16-12.3; 4 males) for comparision.

Results: Plasma insulin levels were significantly lower (p<0.05) in KH compared to subjects in the non-KH group. Plasma ketone body levels were significantly higher in KH (acetoacetate p<0.006; 3-hydroxy butyrate p<0.05) than in non-KH. Basal metabolic rate was significantly higher in subjects with KH (45.48+7.41 vs. 31.81+6.72 kcal/kg/day, p<0.02) but the respiratory quotients were similar in both groups; KH vs. non-KH, 0.84+0.05 vs. 0.8+0.04. Leucine oxidation rates were significantly lower in children with KH (12.25+6.25mol/kg/h vs. 31.96+8.59, p<0.05). Hepatic glucose production rates were also significantly lower in KH; 3.84+0.46 mg/kg/min vs.6.6+0.59 (p<0.0005).

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.

Keywords: energy expenditure, isotope, ketone body, mass spectrometry, tracer

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