FIGURE 1.

Exploratory measurements in two patients with multiple acyl‐CoA dehydrogenase deficiency after an oral dose of D,L‐3‐hydroxybutyrate. The concentration‐time and semi‐log concentration‐time plots of 3‐HB, D‐3‐HB and L‐3‐HB, and AcAc (if available), determined in plasma after an oral dose of D,L‐3‐HB. For measurements of <0.050, a cutoff value of 0.050 was used. Data of patient 1 is not depicted because the peak concentrations of both enantiomers could not be determined due to measurements at two time points only; she received a salt‐free D,L‐3‐HB dose of 1123 mg/kg/day divided in 5 daily doses; body weight 17.3 kg; 5 years old. Patient 2, A/B, received a multiple salt solution of D,L‐3‐HB at a salt‐free dose of 736 mg/kg/day divided in eight daily doses; body weight 4.5 kg; 3 months old. Patient 3, C/D, received sodium‐D,L‐3‐HB at a salt‐free dose of 820 mg/kg/day of in 4 daily doses; body weight 40.0 kg; 16 years old. Data are presented in absolute concentrations