title: Intravenous glycerol therapy should not be used in patients with unrecognized fructose-1,6-bisphosphatase deficiency.
abstract: BACKGROUND
  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.
authors.affiliation: Endocrinology, Metabolism and Genetics Unit, Tokyo Metropolitan Kiyose Children's Hospital, Umezono, Kiyose, Japan. eetyhaseg@1982.jukuin.keio.ac.jp
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Hasegawa
authors.forename: Yoshiharu
authors.initials: Y
authors.lastname: Kikawa
authors.forename: Junko
authors.initials: J
authors.lastname: Miyamaoto
authors.forename: Shuji
authors.initials: S
authors.lastname: Sugimoto
authors.forename: Masanori
authors.initials: M
authors.lastname: Adachi
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Ohura
authors.forename: Mitsufumi
authors.initials: M
authors.lastname: Mayumi
mesh_terms: D001929:Brain Edema / Q000453:epidemiology* / Q000628:therapy*; D002675:Child, Preschool; D015897:Comorbidity; D003451:Cryoprotective Agents / Q000009:adverse effects / Q000627:therapeutic use*; D005260:Female; D015319:Fructose-1,6-Diphosphatase Deficiency / Q000453:epidemiology*; D005990:Glycerol / Q000009:adverse effects / Q000627:therapeutic use*; D006801:Humans; D007003:Hypoglycemia / Q000209:etiology; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies
chemical_list: D003451:Cryoprotective Agents; D005990:Glycerol