IMPAIRED KETOGENESIS IN FRUCTOSE-1,6-BISPHOSPHATASE DEFICIENCY - A PITFALL IN THE INVESTIGATION OF HYPOGLYCEMIA

Authors
MORRIS AAM DESHPANDE S WARDPLATT MP WHITFIELD AE AYNSLEYGREEN A LEONARD JV POURFARZAM M BARTLETT K
Citation
Aam. Morris et al., IMPAIRED KETOGENESIS IN FRUCTOSE-1,6-BISPHOSPHATASE DEFICIENCY - A PITFALL IN THE INVESTIGATION OF HYPOGLYCEMIA, Journal of inherited metabolic disease, 18(1), 1995, pp. 28-32
Citations number
8
Categorie Soggetti
Endocrynology & Metabolism
Journal title
Journal of inherited metabolic disease → ACNP
ISSN journal
01418955
Volume
18
Issue
1
Year of publication
1995
Pages
28 - 32
Database
ISI
SICI code
0141-8955(1995)18:1<28:IKIFD->2.0.ZU;2-G
Abstract
Intermediary metabolite concentrations were measured in blood during f asting in two patients with fructose-1,6-bisphosphatase deficiency. Hy poglycaemia was accompanied by markedly raised levels of plasma free f atty acids, without the expected degree of ketosis. This suggests that there is secondary impairment of ketogenesis in this condition, and c ould lead to diagnostic confusion.