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Table 1 Comparison of lipid profile, markers of LDL quality and HDL functionality, and markers of inflammation between patients with Niemann–Pick disease type B and healthy controls at baseline

From: Changes in PCSK 9 and apolipoprotein B100 in Niemann–Pick disease after enzyme replacement therapy with olipudase alfa

 

Controls (n = 15)

Niemann–Pick Disease type B (n = 5)

P value

Age

35.3 ± 16.3

32.6 ± 9.4

0.363

Total cholesterol (mmol/l)

5.1 ± 1.5

4.5 ± 0.8

0.426

Triglyceride (mmol/l)

1.0 ± 0.3

2.2 ± 1.5

0.003

HDL-C (mmol/l)

1.29 ± 0.27

0.79 ± 0.28

0.002

LDL-C (mmol/l)

3.4 ± 1.3

2.9 ± 0.5

0.469

ApoA1 (g/l)

1.39 ± 0.17

0.84 ± 0.25

< 0.001

ApoB100 (g/l)

0.72 ± 0.26

0.94 ± 0.21

0.098

ApoB48 (µg/ml)

61.0 ± 12.3

89.8 ± 11.9

< 0.001

sdLDL-C (mg/dl)

29.0 ± 18.5

44.7 ± 18.2

0.118

oxLDL (ng/ml)

88.2 ± 21.4

131.1 ± 22.8

0.001

PCSK9 mass (ng/ml)

366.8 ± 127.8

599.0 ± 237.6

0.011

TNF-α (pg/ml)

27.7 ± 8.8

76.0 ± 10.7

< 0.001

PON1 activity (nmol/ml/min)

158.1 ± 128.8

98.5 ± 79.0

0.347

Cholesterol efflux capacity (%)

13.1 ± 1.4

11.9 ± 1.4

0.150

  1. Data are presented as mean ± SD
  2. P value for comparison with controls using independent samples t-test
  3. apoA1, apolipoprotein A1; apoB-100, apolipoprotein B-100; apoB-48, apolipoprotein B-48; HDL-C, high-density lipoprotein cholesterol; LDL-C, low-density lipoprotein cholesterol; oxLDL, oxidised low-density lipoprotein; PCSK9, proprotein convertase subtilisin/kexin type 9; PON1, paraoxonase-1; sdLDL-C, small dense low-density lipoprotein cholesterol; TNF-α, tumour necrosis factor α