The Glycerol Intolerance Syndrome (GIS) is a rare disorder of glycerol metabolism.
Some patients have fructose-1,6-diphosphatase (FDP) deficiency.
Clinical features:
(1) Intolerance of glycerol ingestion or infusion.
(2) Intolerance of fructose if FDP deficiency present.
(3) Episodic metabolic crisis with central nervous system deterioration, hypoglycemia, metabolic acidosis and ketosis.
General management:
(1) Avoid glycerol.
(2) Avoid fructose if FDP-deficient.
Management of an acute crisis:
(1) Intravenous infusion of glucose.
(2) Supportive care.
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Specialty: Endocrinology, Clinical Laboratory, Genetics
ICD-10: ,
