title: Glucose and leucine kinetics in idiopathic ketotic hypoglycaemia.
abstract: OBJECTIVE
  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.
authors.affiliation: Biochemical Genetics and National Neonatal Screening Laboratories, University Children's Hospital Vienna, Vienna, Austria. olaf.bodamer@meduniwien.ac.at
authors.forename: O A
authors.initials: OA
authors.lastname: Bodamer
authors.forename: K
authors.initials: K
authors.lastname: Hussein
authors.forename: A A
authors.initials: AA
authors.lastname: Morris
authors.forename: C-D
authors.initials: CD
authors.lastname: Langhans
authors.forename: D
authors.initials: D
authors.lastname: Rating
authors.forename: E
authors.initials: E
authors.lastname: Mayatepek
authors.forename: J V
authors.initials: JV
authors.lastname: Leonard
mesh_terms: D000409:Alanine / Q000097:blood; D000597:Amino Acids, Branched-Chain / Q000097:blood; D001481:Basal Metabolism; D002153:Calorimetry, Indirect; D002331:Carnitine / Q000031:analogs & derivatives / Q000097:blood; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D005215:Fasting; D005260:Female; D005947:Glucose / Q000378:metabolism*; D006801:Humans; D007003:Hypoglycemia / Q000378:metabolism*; D007223:Infant; D007328:Insulin / Q000097:blood; D007662:Ketosis / Q000378:metabolism*; D007930:Leucine / Q000378:metabolism*; D008297:Male
chemical_list: D000597:Amino Acids, Branched-Chain; D007328:Insulin; C116917:acylcarnitine; D007930:Leucine; D005947:Glucose; D000409:Alanine; D002331:Carnitine