Supplementary Tables
Table 1S. Biomarkers of metabolic control in females of reproductive age with PKU stratified by age group
PKU / Reference range / PKU / Reference range
n = 20 / n = 21
Plasma Phe, mean (SD), µmol/La / 843.1 (463.9) / 26-91 / 707.8 (464.8) / 49-76
Plasma Tyr, mean (SD), µmol/L / 42.1 (20.1) / 24-115 / 48.5 (24.5) / 41-78
Phe : Tyr, mean (SD), ratio / 23.9 (14.0) / 16.3 (11.3)
Abbreviations: PKU, phenylketonuria; Phe, phenylalanine; Tyr, tyrosine.
a NIH consensus statement (2000) treatment range goals: <12 years, preconception, and pregnancy: 120-360 µmol/L; adolescents: 120-600; adults: 120-900 (National Institutes of Health Consensus Development Panel 2001).
Table 2S. Select biomarkers of plasma total lipid fatty acid status in females of reproductive age with PKU compared with female laboratory controls without PKU
PKU / Lab controls / PKU / Lab controls
n = 20 / n = 5 / n = 21 / n = 17 / F testa / P value
Age, mean (SD), y / 15.1 (1.8) / 15.7 (0.97) / 32.3 (7.4) / 38.7 (8.1) / F1, 61 = 10.9 / .002
Plasma LA, mean (SD), % / 30.37 (4.24) / 27.97 (4.49) / 29.78 (3.71) / 29.33 (2.45) / F1, 61 = 1.2 / .28
Plasma AA, mean (SD), % / 5.96 (1.44) / 5.86 (1.09) / 6.86 (1.40) / 6.38 (0.970) / F1, 61 = 0.2 / .65
Plasma ALA, mean (SD), % / 0.841 (0.201) / 0.612 (0.085) / 0.839 (0.340) / 0.691 (0.289) / F1, 61 = 5.5 / .02
Plasma EPA, mean (SD), % / 0.271 (0.087) / 1.027 (0.436) / 0.365 (0.200) / 0.618 (0.459) / F1, 61 = 23.1 / <.001
Plasma DHA, mean (SD), % / 0.932 (0.299) / 3.177 (1.129) / 1.137 (0.326) / 2.889 (0.817) / F1, 61 = 157.8 / <.001
Plasma AA/DHA ratio, mean (SD) / 6.67 (1.47) / 2.16 (1.13) / 6.45 (2.10) / 2.36 (0.68) / F1, 58 = 110.6 / <.001
Plasma TLFA, mean (SD), µg/mL / 2476 (1100) / 2609 (322) / 2588 (786) / 2547 (382) / F1, 58 = 0.0 / .90
Abbreviations: PKU, phenylketonuria; DHA, docosahexaenoic acid; LA, linoleic acid; AA, arachidonic acid; ALA, alpha-linolenic acid; EPA, eicosapentaenoic acid; TLFA, total lipid fatty acids.
a 1-way ANOVA: all PKU (n = 41) vs. all lab controls (n = 22).
Table 3S. Select biomarkers of RBC total lipid fatty acid status in females of reproductive age with PKU compared with female laboratory controls without PKU
PKU / Lab controls / PKU / Lab controls
n = 20 / n = 5 / n = 21 / n = 20 / F testa / P value
Age, mean (SD), y / 15.1 (1.8) / 13 (2.1) / 32.3 (7.4) / 36.8 (9.9) / F1, 64 = 7.9 / .007
RBC LA, mean (SD), % / 9.85 (1.15) / 9.57 (1.09) / 9.73 (1.28) / 9.63 (1.22) / F1, 64 = 0.3 / .58
RBC AA, mean (SD), % / 12.45 (1.17) / 13.64 (0.49) / 12.71 (1.75) / 14.08 (1.34) / F1, 64 = 15.9 / <.001
RBC ALA, mean (SD), % / 0.130 (0.025) / 0.079 (0.021) / 0.126 (0.036) / 0.125 (0.048) / F1, 64 = 1.4 / .23
RBC EPA, mean (SD), % / 0.196 (0.030) / 0.332 (0.121) / 0.282 (0.133) / 0.494 (0.205) / F1, 64 = 34.6 / <.001
RBC DHA, mean (SD), % / 2.12 (0.50) / 3.66 (0.45) / 2.69 (0.82) / 3.92 (1.04) / F1, 64 = 48.8 / <.001
RBC AA/DHA ratio, mean (SD) / 6.27 (1.99) / 3.77 (0.48) / 5.13 (1.57) / 3.88 (1.21) / F1, 64 = 20.1 / <.001
RBC TLFA, mean (SD), µg/mL / 1329 (165) / 1423 (145) / 1453 (257) / 1328 (204) / F1, 64 = 0.7 / .41
Abbreviations: PKU, phenylketonuria; DHA, docosahexaenoic acid; LA, linoleic acid; AA, arachidonic acid; ALA, alpha-linolenic acid; EPA, eicosapentaenoic acid; TLFA, total lipid fatty acids.
a 1-way ANOVA: all PKU (n = 41) vs. all lab controls (n = 25).