Metabolism
on-line - the virtual tutorial room
copyright © 2008 - 2015 David A Bender
Two boys with profound fasting hypoglycaemia and no ketone bodies
LF is an 18 month old boy. He is the first child of healthy non-consanguineous parents, delivered after a normal pregnancy. He was well until he had an episode of gastroenteritis, leading to diarrhoea and vomiting for several days, when he was admitted to hospital in a coma, with a blood glucose concentration of 1.0 mmol /L. There was no smell of acetone on his breath, and on testing his urine with a dipstick there were no detectable ketone bodies. He regained consciousness after an intravenous infusion of glucose.
Later during his stay in hospital he was fasted under close supervision, with blood samples taken at intervals over 18 hours, by which time he had become hypoglycaemic, and was given an intravenous infusion of glucose. The following results were obtained on analysis of blood samples taken during his fast (all in mmol /L):
LF |
control subjects |
||||
13 h fasting |
15 h fasting |
17 h fasting |
18 h fasting |
24 h fasting |
|
| glucose | 4.1 |
3.8 |
2.9 |
2.3 |
3.6 ± 0.4 |
| non-esterified fatty acids | 2.38 |
2.48 |
3.34 |
3.96 |
1.2 ± 0.2 |
| beta-hydroxybutyrate | 0.03 |
0.05 |
0.05 |
0.02 |
2.0 ± 0.2 |
| acetoacetate | nd |
nd |
nd |
nd |
1.0 ± 0.2 |
nd = not detectable
(From data reported by Morris AAM, et al, Pediatr Res 44:3
392-6, 1998)