All medications should be divided into three to four equal daily doses taken with meals and distributed as far as possible throughout the day. Grade of recommendation, C-D.
a100 mg of each drug correspond to the following amounts in mmols: 0.694 sodium benzoate; 0.537 sodium PBA; 0.475 arginine hydrochloride; 0.574 arginine base; 0.571 citrulline; 0.532 carbamylglutamate.
b Sodium PBA was considered of second choice for long-term treatment by most guideline group members. It should be given together with sodium benzoate in patients in which benzoate alone is not enough.
c Serum/plasma levels of benzoate/PBA and plasma levels of arginine (aim are fasting levels of 70–120 μmol/L) should be monitored to adjust dosages and in case of high or repeated doses.
d In some patients higher doses are needed, according to expert advice. For PBA, FDA and EMA approved doses are 450-600mg/kg/d in children of <20kg, and, above 20 kg bw, 9.9-13 g/m2/d.
e Citrulline may be preferable. When given no need for concomitant use of L-arginine.