OBJECTIVES: I. Study the metabolism of pyruvate and related problems in patients with
II. Define the nature of the metabolic defect.
PROTOCOL OUTLINE: Patients fast for 6 hours. Glucagon IM is administered after the 6 hour
fast. Glucose level is measured at 0, 15, 30, 45, 60, and 90 minutes.
In children of sufficient size, alanine and lactic acid should also be measured at each or
most of these time points.
Fasting continues for at least 18 hours. Glucagon IM is administered again at end of fast.
Glucose level is measured at time 0, 15, 30, 45, 60, and 90 minutes.
PROTOCOL ENTRY CRITERIA:
- Diagnostically documented elevation in lactate, pyruvate, and/or alanine levels in
lactic acidemia patients