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Intravenous glycerol therapy should not be used in patients with unrecognized fructose-1,6-bisphosphatase deficiency.

AbstractBACKGROUND:
In Asian countries, glycerol solution that contains fructose (5%) is often used for management of brain edema. However, glycerol and fructose may cause severe hypoglycemia and metabolic acidosis in patients with fructose-1,6-bisphosphatase (FBPase) deficiency, even under stable conditions. The aim of the present study was to determine whether glycerol solution was used for brain edema during acute metabolic decompensation of hypoglycemia and metabolic acidosis in patients with unrecognized FBPase deficiency in Japan and to examine a long-term prognosis of the patients who had this kind of severe metabolic decompensation with or without glycerol therapy.
METHODS:
A retrospective study of 20 children with FBPase deficiency was conducted, based on their medical records.
RESULTS:
Six of the 20 children were given glycerol solution for the presence or possibility of brain edema during acute metabolic decompensation of hypoglycemia and metabolic acidosis; two of the six patients administered with glycerol were given dialysis. In four patients treated with glycerol alone without dialysis, two had no brain edema before glycerol administration but it developed later after the administration. These four patients treated with glycerol alone died or developed severe neurological complications. Fourteen patients who were not treated with glycerol solution had no brain edema and showed good prognosis.
CONCLUSIONS:
Glycerol solution, which contains fructose in Asian countries including Japan, should not be used as an osmotic agent for treatment of brain edema in patients who have hypoglycemia and retention-type metabolic acidosis, until FBPase deficiency is ruled out by measuring blood concentration of lactate.
AuthorsYukihiro Hasegawa, Yoshiharu Kikawa, Junko Miyamaoto, Shuji Sugimoto, Masanori Adachi, Toshihiro Ohura, Mitsufumi Mayumi
JournalPediatrics international : official journal of the Japan Pediatric Society (Pediatr Int) Vol. 45 Issue 1 Pg. 5-9 (Feb 2003) ISSN: 1328-8067 [Print] Australia
PMID12654061 (Publication Type: Journal Article)
Chemical References
  • Cryoprotective Agents
  • Glycerol
Topics
  • Brain Edema (epidemiology, therapy)
  • Child, Preschool
  • Comorbidity
  • Cryoprotective Agents (adverse effects, therapeutic use)
  • Female
  • Fructose-1,6-Diphosphatase Deficiency (epidemiology)
  • Glycerol (adverse effects, therapeutic use)
  • Humans
  • Hypoglycemia (etiology)
  • Infant
  • Infant, Newborn
  • Male
  • Prognosis
  • Retrospective Studies

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