HMG-CoA synthase deficiency affects the first step of hepatic ketogenesis. Patients typically present during infancy with hypoketotic hypoglycemic coma. Acute crises occur during prolonged fasting, often triggered by concomitant infectious illness. sequelae may occur due to prolonged hypoketotic hypoglycemia.
No information available from this source.
This disease is not (yet) listed on their website.