In a KH-patient, the glycogen stores are somehow insufficient. This leads to decreased fasting tolerance with earlier onset of fat burn and hence ketone bodies.
In most patients, the hypoglycemia is relatively mild, and the ketone bodies help to provide fuel to the brain, which prevents loss of consciousness and convulsions.
However, in relatively few patients, the condition is more severe, but still without an identified cause despite intense investigations in hormones and cell metabolism. Such patients are said to have “idiopathic” KH, or IKH, which simply means KH without any known cause