Table 1.

Differential diagnoses of spontaneous ketosis

Inborn errors of metabolism

– Glycogen storage disease types 0, III, IV, IX

– Gluconeogenesis defects

– Branched chain organic acidemias

– Mitochondrial respiratory chain disorders

– Defects of ketolysis

– GLUT-1-deficiency syndrome

Others

– Fasting states, catabolism

– Diabetes

– Corticosteroids

– Growth hormone deficiency

– Adrenal insufficiency