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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