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Table 4 Initial biochemical data of among MMA mutase, MMA cblB, PA and false positive patients upon their admission

From: Methylmalonic acidemia/propionic acidemia – the biochemical presentation and comparing the outcome between liver transplantation versus non-liver transplantation groups

 

False positive (n = 10)

MMA

PA (n = 4)

mut (n = 18)

cbl (n = 1)

ALT(IU) (0–40)

15.00 ± 4.83 (6–21)

43.64 ± 48.34* (11-166)

14

18.00 ± 9.83a (6–30)

AST(IU) (5–45)

30.5 ± 21.89 (19–92)

98.64 ± 115.96* (19-451)

34

44.50 ± 20.94a (21–69)

Bun(mg/dl) (7–20)

10.67 ± 4.37 (6–18)

13.18 ± 8.88 (2–35)

8

13.08 ± 10.65 (7–29)

Cre(mg/dl) (0.5–1.5)

0.23 ± 0.14 (0.1–0.39)

0.62 ± 0.47* (0.1-2.0)

0.42

0.50 ± 0.16* (0.3-0.69)

NH3(μg/dl) (5–150, newborn)

70.58 ± 27.25 (35.8–132)

388.89 ± 299.07* (92-1244)

253

341.00 ± 227.28* (84-637)

pH value (7.40 ± 0.08)

7.43 ± 0.08 (7.305–7.54)

7.28 ± 0.14* (6.94-7.47)

7.55

7.37 ± 0.11 (7.229–7.476)

HCO3 (20 ± 4)

20.54 ± 3.19 (14.5–26.3)

13.97 ± 6.38 (2.0–25.3)

8.7

18.10 ± 4.07 (13.5–22)

Glycine (μM) (110–240)

227.7 ± 52.61 (190.5–264.9)

533.49 ± 221.21* (238-1163.5)

148.9

592 ± 106.06 (84–667)

  1. *indicated significant difference (p < 0.05) compared to false positive group
  2. aindicated significant difference compared to mutase type patients