TABLE 1

Metabolic Parameters in Blood and Urine During the Different Phases of 3-HB Treatment

Parameter3-HB dose
A = NoB = 450 mg/kg per dayC = 900 mg/kg per dayD = 1400 mg/kg per dayE = 2600 mg/kg per day
FFAs, mmol/L535 (407–737)345 (63–467)194 (68–389)181 (176–189)236 (156–755)
AcA, mmol/L0.05 (0.03–0.07)0.03 (0.01–0.04)0.02 (0.01–0.03)0.02 (0.00–0.11)e0.10 (0.02–0.29)d
BOHB, mmol/L0.06 (0.05–0.10)0.02 (0.01–0.03)0.02 (0.00–0.03)0.02 (0.01–0.16)e0.15 (0.02–0.46)d
NT-proBNP, ng/L1217 (689–1744)65263 (33 685–90 427)c,d,e11954 (5466–47 039)b,d,e7790 (2515–13 941)b,c,e527 (241–2370)b,c,d
Plasma acylcarnitines, μmol/L
 Total carnitine38 (23–137)e41 (23–58)e45 (25–99)e51 (27–113)e101 (74–124)a,b,c,d
 Free carnitine19 (12–102)b,e14 (8–17)a,c,d,e28 (16–70)b,e27 (15–69)b,e68 (42–87)a,b,c,d
 C52.71 (1.20–4.94)c,d,e2.34 (1.03–6.29)1.67 (0.59–2.38)a1.46 (0.85–2.74)a1.86 (1.28–2.41)a
 C6–C185.32 (3.45–15.47)e9.89 (5.41–17.40)5.78 (3.56–12.91)6.32 (4.25–14.65)8.85 (7.10–14.60)a
Urinary organic acids
 Ethylmalonic acid0.27 (0.10–0.77)0.150.39 (0.27–0.64)0.28 (0.09–0.38)0.33 (0.02–0.41)
 Glutaric acid2.11 (0.74–3.32)c,d,e5.640.58 (0.24–0.99)a0.77 (0.25–1.12)a0.45 (0.05–0.66)a
 Adipic acid0.03 (0.01–0.21)5.550.07 (0.03–0.54)0.12 (0.03–0.23)0.13 (0.01–0.29)
 2-OH-glutaric acid0.86 (0.31–0.91)0.290.70 (0.52–0.97)0.63 (0.39–1.38)0.55 (0.07–0.72)
 Isovalerylglycine0.06 (0.03–0.18)e0.160.09 (0.03–0.12)e0.05 (0.02–0.06)0.02 (0.00–0.08)a,c
 Hexanoylglycine0.02 (0.00–0.10)0.000.06 (0.02–0.17)e0.06 (0.04–0.08)e0.01 (0.00–0.05)c,d
 3-HB0.00 (0.00–0.00)d,e0.000.00 (0.00–2.37)e2.68 (0.18–5.32)a16.70 (1.46–27.03)a,c
 Suberic acid0.01 (0.00–0.03)0.250.02 (0.01–0.07)0.02 (0.01–0.06)0.01 (0.00–0.03)
  • Data are presented as medians (ranges). Acute metabolic decompensation at the age of 7 mo occurred during 3-HB supplementation at a dose of 450 mg/kg per day. Differences between groups were analyzed by Kruskal-Wallis test and subsequently by Mann-Whitney U test and were considered significant at P < .05. AcA, acetoacetate; BOHB, β-hydroxybutyrate; C5, isovaleryl-/2-methylbutyrylcarnitine; C6–C18, the sum of acylcarnitines of C6–C18; FFA, free fatty acid.

  • a Significantly different from A.

  • b Significantly different from B.

  • c Significantly different from C.

  • d Significantly different from D.

  • e Significantly different from E.