Acute pancreatitis after anesthesia with propofol in a child with glycogen storage disease type IA.

Glycogen storage disorder type 1A (GSD 1A) is an inherited disorder of glycogen metabolism characterized by fasting hypoglycemia, lactic acidosis, hyperuricemia, and hyperlipidemia. These children have a higher risk of developing pancreatitis because of hypertriglyceridemia. Drug-induced pancreatitis accounts for a small proportion of cases of pancreatitis. The mechanism of drug-induced pancreatitis include hypersensitivity, direct toxic injury or indirectly by inducing hypertriglyceridemia. Propofol is often the drug of choice for induction of anesthesia in ambulatory surgical procedures. There are various reports in the literature describing pancreatitis induced by propofol. We present a 4-year-old girl with GSD 1A, who required tonsillectomy and adenoidectomy under general anesthesia. She developed acute pancreatitis in the postoperative period. Propofol was used as a general anesthetic and the postoperative incidence of pancreatitis is discussed.