Clinical manifestations are due to accumulation of toxic metabolites
Suspect in any sick neonate
Newborn screening varies by state
May present as late as early adulthood
Must rule-out sepsis (more common in these patients due to organic acid-induced bone marrow suppression) and other physiologic stressors that might be precipitating decompensation
Inborn errors of metabolism
THE MISFITS [1]
Thalassemia
Trauma
Retinoic acid syndrome
Heart
Neonatal jaundice
Congenital heart disease