Hemorrhagic pancreatitis in a patient with glycogen storage disease type I

A 17-year-old female with glycogen storage disease type I (GSD-I) died suddenly with hemorrhagic pancreatitis. She had a long-standing history of hyperlipidemia that did not respond to a regimen of frequent daytime and nocturnal intragastric feeding. Although pancreatitis is a well-known complication of hyperlipidemia, there are no reports to our knowledge of pancreatitis causing sudden death in patients with GSD-I. Pancreatitis must be added to the growing list of complications that can occur in long-term survivors with GSD-I, and should be considered when these patients present with abdominal pain.