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Affected infants are symptomatic at birth with hypoketotic hypoglycemia, seizures, cardiomyopathy/arrythmias, liver failure and death occurring within the first few weeks of life, (2) severe infantile (hepatocardiomuscular) form, with symptomatology appearing from 6 to 24 months of life in a similar presentation but less severe than LNF form and (3) myopathic form, which is the least severe type and they become symptomatic in infancy to adulthood with myalgia and weakness following exercise or fasting ( Lethal neonatal form of CPT II remains a challenge in early diagnosis and treatment

2 test) (Table 1)

32 Di CerboA.PalmieriB.AponteM.Morales-MedinaJ