A 6-year-old child with abdominal pain

A 6-year-old female presented to her local emergency room with a history of vomiting and fever. She had had 20-30 episodes of emesis over the previous 2 days and an acute onset of abdominal distension and also gave a 1-day history of fever to 101°F. Two weeks before her ER visit she had had a spica cast placed because of a right femur fracture resulting from a motor vehicle accident. Her medical history was significant for Schwartz-Jampel syndrome, type 1. She demonstrated the following characteristics of this syndrome: mild developmental delay, skeletal muscle abnormalities including muscle weakness and stiffness, short stature, small mouth, flat nose, short neck, muscular hypertrophy, and stiffness in her wrists and fingers. Both Tegretol® (started at 3 years of age) and Dilantin® (begun at 5 years of age) had controlled her muscle stiffness. She had had no difficulty related to her spica cast until 2 days before her visit to the ER. On presentation to the ER, her abdomen was so distended that the anterior portion of her spica cast needed to be removed. Laboratory findings included the following: elevated concentrations of amylase (488 mu/mL) and lipase (761 U/dL), a peripheral white cell count of 28,900/cm3 with 87% segmented polys, therapeutic concentrations of Tegretol® and DilantinO, and normal results from liver function tests: aspartate aminotransferase (AST): 47 mU/mL; alanine