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Table 2: Laboratory and MRS measurements of the patients and of the control subjects
Patients Cr per Os (mg/kg/bw/day) Plasmatic Cr (μmol/L) Plasmatic GAA (μmol/L) Brain Cr (mmol/L) PCr/PDE P_i/PDE ATP/PDE pH
GAMT-d 
    Patient 1 800 487 11.4 3.86 1.03 0.76 0.58 7.22
400^* 598 11.1 3.10 0.91 0.75 0.59 6.85
400^** 512 7.9 3.31 1.19 0.70 0.65 7.43
    Patient 2 0 24 40.3 0.93 0.37^† 0.72 0.80 7.08
400^** 431 7.4 3.76 1.02 0.78 0.57 6.75
AGAT-d 
    Patient 3 0 6 1.10 0.73 0.84 0.63 7.31
400 776 3.87 1.24 0.85 0.61 7.21
200 203 3.65 1.19 0.82 0.70 7.20
    Patient 4 0 6 1.05 0.76 0.87 0.64 7.31
400 588 4.00 1.49 0.90 0.65 7.17
200 178 3.85 1.15 0.81 0.77 7.36
    Patient 5 0 8 1.33 0.76 0.87 0.64 7.22
400 283 3.99 1.32 0.87 0.64 7.04
200 93 3.52 0.83 0.76 0.62 7.54
300 246 3.61 0.93 0.77 0.54 7.22
Reference values n.v. ± SD 64 ± 14 1.48 ± 0.51 4.37 ± 0.44 1.40 ± 0.14 0.87 ± 0.08 0.64 ± 0.06 7.15 ± 0.12
Note:—Os indicates oral intake; Cr, creatine; bw, body weight; GAA, guanidinoacetate; PCr, phosphocreatine; PDE, phosphodiester; P_i, inorganic phosphate; GAMT-d indicates guanidinoacetate methyltransferase defect; AGAT-d, arginine:glycine amidinotransferase defect; n.v., normal value.
* Coupled with ornithine supplementation.
** Coupled with ornithine supplementation and arginine restriction.
† Resonance frequency of this signal was at −0.5 ppm and was assigned to GAA-P.