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The mild benefits in muscle strength experienced by HIBM patients after IVIG …


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- Intravenous immune globulin in hereditary inclusion body myopathy: a pilot study

Table 1
Laboratory values at baseline, after loading with IVIG (1 g/kg × 2), and at the end of IVIG treatment.

Baseline
Post-load
End of study
Normal range
Patient
1
2
3
4
1
2
3
4
1
2
3
4

Hemoglobin (g/dL)
13.1
14.4
13.2
14.3
11.2
13.8
11.2
13
12.1
13.8
12.3
11.4
11.1–16.7
WBC (K/uL)
8.94
7.57
7.39
7.28
6.82
6.11
8.06
2.99
6.94
6.16
6.34
4.85
3.3–9.6
Platelets (K/uL)
282
230
284
225
226
209
262
207
267
222
256
220
154–380
ESR (mm/h)
17
7
23
14
39
38
96
86
30
20
57
73
0–42
Creatinine (mg/dL)
0.3
0.7
0.5
0.4
0.4
0.8
0.6
0.6
0.3
0.7
0.6
0.5
0.7–1.4
BUN (mg/dL)
15
14
12
13
12
7
8
8
16
15
13
17
8–22
ALT (U/L)
42
36
18
36
31
37
16
30
60
81
18
62
6–41
AST (U/L)
30
31
22
23
21
31
15
23
36
68
31
52
9–34
CPK (U/L)
320
475
98
130
244
451
50
87
229
629
78
94
38–386
Cholesterol (mg/dL)
195
203
195
207
133
153
144
156
168
179
175
170
100–200
Total protein (g/dL)
6.4
6.1
6.1
6.3
7.8
8.7
9.5
9.1
7.1
7.2
7.3
7
6.0–7.6
Albumin (g/dL)
3.7
3.7
3.2
3.5
2.9
3.4
2.6
3
3.3
3.5
2.8
3.1
3.7–4.7
IgG (mg/dL)
1280
1180
955
997
3550
4280
5670
4440
2150
2350
2340
2310
642–1730
Urine protein (mg/d)
150
92
294
97
79
95
364
142
30–150
Urine glucose (g/d)
0.07
0.07
0.07
0.09
14.4
30.8
6.4
12.2
0.07
0.07
0.10
0.08
0–0.49
Abnormal values are in bold. Normal range encompasses values for males and females.
Baseline values are average of results on two different days.
Table 2
Strength of muscle groups of 4 HIBM patients at baseline and after IVIG treatment.

Hip Flexion
Ankle Dorsiflexion
Elbow
Wrist
Hand






Flexion
Extension
Flexion
Extension
Grip
Pinch
Baselinea,b
3.21 ± 0.84
3.50 ± 2.15
4.71 ± 1.42
3.36 ± 1.09
5.24 ± 0.86
4.55 ± 0.46
7.43 ± 1.63
1.02 ± 0.22
Post-Loadb
3.93 ± 0.74
(+22%)
2.53 ± 1.55
(-28%)
5.56 ± 1.60
(+18%)
3.30 + 0.98
(-2%)
5.81 ± 1.07
(+11%)
5.15 ± 0.58
(+13%)
7.85 ± 1.65
(+6%)
1.03 ± 0.16
(+1%)
End of studyb
3.63 ± 0.62
(+13%)
4.28 ± 2.87
(+22%)
5.45 ± 1.55
(+16%)
3.36 ± 1.03
(0%)
6.25 ± 1.15
(+19%)
5.78 ± 0.93
(+27%)
7.83 ± 1.59
(+5%)
1.37 ± 0.25
(+35%)
aValues are given in kg as means ± SEM. Percent changes are in parentheses. Values for both the right and left sides were included in the analyses.
bBaseline: Before treatment; Post-Load: Day 1 after IVIG load (1 g/kg × 2); End of study: 4 weeks after the initial IVIG load.

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