The high increase in ammonium (Figure 2, ammonium increase of 25-30X, normal: 5-10X) can be explained as the result of the increased activity of AMPD, to compensate for the muscle energetic deficit during intense exertion in the context of LDH deficiency [3,19,20]. Here, AMPD1 is linked to hyperinsulinemic hypoglycemia, familial, 4.