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In the classical form there are two main presentations with the onset in infancy with acute nonketotic hypoglycemic episodes, poor feeding, failure to thrive, hypertrophic cardiomyopathy, hepatomegaly, elevated liver transaminases, hyperammonemia triggered by fasting or common illnesses such as upper respiratory tract infection or gastroenteritis, hypotonia, lethargy or encephalopathy associated with hypoglycemia, Reye syndrome and a delay in gross motor development due to muscle weakness
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