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The association of recurrent attacks of severe ketoacidosis with blood glucose levels generally high or normal, low lactacidemia and low ammonemia.
(Ketoacidosis is an extreme and uncontrolled form of ketosis, which is a normal response to prolonged fasting. In ketoacidosis, the body fails to adequately regulate ketone production causing such a severe accumulation of keto acids that the pH of the blood is substantially decreased)
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