A 27-day-old girl presented with abnormal bicycling movement, lethargy, poor feeding, and repeated vomiting for the last 7 days

(B). Treatment of the acute state of maple syrup urine disease is aimed at hydration and rapid removal of the branched-chain amino acids and their metabolites from the tissues and body fluids. Because renal clearance of these compounds is poor, hydration alone may not produce a rapid improvement. Peritoneal dialysis or, preferably, hemodialysis is the most effective mode of therapy in critically ill infants