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Table 6 MIP, MEP, and peripheral muscle strength measurements (mean ± SD and [95% confidence interval]) in BSCL subjects without and with metreleptin replacement

From: Impairment of respiratory muscle strength in Berardinelli-Seip congenital lipodystrophy subjects

(n = 7)

2015 (n/%)

2016 (n/%)

2017 (n/%)

P

MIP (predicted %)

 No Metreleptin

7 (100)

4 (57.2)

3 (42.8)

–

 

70.24 ± 14.89

72.62 ± 16.14

100.4 ± 33.27

b0.0719

 

[60.24–80.24]

[46.93–98.30]

[17.71–183.00]

–

 Metreleptin

0 (0)

3 (42.8)

4 (57.2)

–

 

–

54.93 ± 14.93

81.35 ± 17.36

a0.0892

 

–

[17.84–92.03]

[53.73–109]

 

 pa

–

0.7524

0.3644

 

MEP (predicted %)

 No Metreleptin

7 (100)

4 (57.2)

3 (42.8)

–

 

55.24 ± 18.42

50.56 ± 21.34

57.1 ± 16.19

b0.8881

 

[38.2–72.28]

[16.6–84.52]

[16.87–97.33]

–

 Metreleptin

0 (0)

3 (42.8)

4 (57.2)

–

 

–

51.31 ± 4.67

44.11 ± 14.51

a0.4541

 

–

[39.69–62.93]

[21.03–67.19]

–

 pa

–

0.9556

0.3142

–

PMS (predicted %)

 No Metreleptin

7 (100)

4 (57.2)

3 (42.8)

–

 

85.34 ± 25

85.87 ± 30.34

92.03 ± 18.78

b0.9270

 

[62.22–108.5]

[37.6–134.1]

[45.38–138.7]

–

 Metreleptin

0 (0)

3 (42.8)

4 (57.2)

–

 

–

84.8 ± 26.27

79.81 ± 28.39

a0.8219

 

–

[19.53–150.1]

[34.63–125]

–

 pa

–

0.9632

0.5504

–

  1. ap values were based on independent unpaired t-tests
  2. bp values were based on repeated measures ANOVA for the group without metreleptin