Treatment including oral biotin and diet adjustment was commenced immediately after confirming the diagnosis. For patients during acute decompensation, the initial therapy included a low-protein diet, supplementation of biotin as well as L-carnitine, and intravenous fluid therapy with glucose as well as electrolytes. Then the treatment was adjusted according to their response to biotin and other personal condition. The recommended dosage of lifelong administered biotin was 10–40 mg/day [12 (link)].
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