Abstract
Very-high-dose biotin therapy is sometimes prescribed in newborns with pharmacoresistant neonatal epileptic encephalopathy or with suspicion of inherited metabolic disorders such as biotinidase deficiency, holocarboxylase synthetase deficiency, or Leigh syndrome (in biotin-thiamine-responsive basal ganglia disease due to SLC19A3 mutation).1,2 Regardless of the child’s weight (2-10 kg), tremendous doses of biotin are administrated (10-300 mg/day, e.g., 3-100 mg/kg/day for a 3-kg newborn).
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