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Table 4 Comparison of pharmacokinetics data of betaine with the litterature

From: Efficacy and pharmacokinetics of betaine in CBS and cblC deficiencies: a cross-over randomized controlled trial

  

Age (y)

Dose (mg/kg)

Cmax (µmol/L)

AUC (µmol h/L)

t1/2,elim (h)

CL/F (L/h kg−1)

V/F (L/h kg−1)

CBS deficiency

Matthews et al. [23]

n = 6

14.5 [6,7,8,9,10,11,12,13,14,15,16,17]a

100

na

na

11.8 [8.0 – 26.3]a

0.174 [0.054 – 0.210]a

na

Current study

n = 5

13.3 [6.8–14.4]a

100

849 [681–1048]a

5522 [4909; 9317]c

16.7 [13.8–20.3]a

0.15 [0.09–0.17]a

1.45 [1.2–1.98]a

 

250

2074 [1001–2563]a

13,276 [6454; 14907]c

12.3 [10.0–16.3]a

0.16 [0.14–0.33]a

1.29 [1.15–3.41]a

cblC/MTHFR deficiency

Schwahn et al. [18]

n = 1

5.8

50

506

1450

6.63

0.297

1.726

n = 1

10.2

50

556

4230

23.2

0.102

1.907

Current study

n = 6

5.4 [1.5–17.8]a

100

717 [585–1000]a

7485 [4843; 11907]a

18.3 [10.9–24.2]a

0.11 [0.07–0.18]a

1.56 [1.04–1.86]a

 

250

1136 [827–2356]a

10,389 [7826; 29243]a

18.4 [9.43–25.9]a

0.21 [0.07–0.27]a

2.34 [1.15–3.49]a

  1. Cmax: Betaine maximal concentration; AUC: betaine area under the curve; t1/2,elim: betaine elimination half-life; CL/F: betaine apparent clearance, V/F: betaine apparent volume of distribution; na: non available
  2. $Doses in mg/kg are calculated using the mean weight of the patients
  3. aMedian [range]