Introduction
Methods
Material
Data collection
Fracture history
Bone mineral density
Assessment of calcium metabolism and biochemical markers of bone turnover
All OI patients | OI type 1 | OI type 3 | OI type 4 | Unclassified | |
---|---|---|---|---|---|
N
| 97 | 75 | 9 | 11 | 2 |
Sex (male/female) | 41/56 | 28/47 | 3/6 | 9/2 | 1/1 |
Age (mean ± SD) | 44 ± 12 | 45 ± 13 | 35 ± 7 | 47 ± 7 | 49 |
Weight (kg), (mean ± SD) | 66 ± 17 | 69 ± 13 | 36 ± 14 | 70 (12) | 69 |
Height (cm), (mean ± SD) | 156 ± 20a
| 163 ± 10 | 106 ± 13 | 157 ± 17 | 157 |
Ambulation using wheelchair | 19 (20%) | 7 (9%) | 9 (100%) | 3 (27%) | 0 |
Drug use | |||||
HRT/bisphosphonates | 17 (18%) | 13 (17%) | 1 (11%) | 2 (18%) | 1 (50%) |
Kalsium/vitamin D | 24 (25%) | 21 (28%) | 0 | 3 (27%) | 0 |
Fractures or orthopaedic operations last 12 months | 12 (12%) | 9 (12%) | 0 | 2 (18%) | 1 (50%) |
Bone turnover markers
Calcium metabolism
Statistical methods
Results
Prevalence and localisation of fractures
Age |
N, 39 | Men median (range) |
N, 55 | Women median (range) |
N, 94 | Total median (range) |
N, 74 | OI type I median (range) |
N, 7 | OI type III median (range) |
N, 11 | OI type IV median (range) |
---|---|---|---|---|---|---|---|---|---|---|---|---|
25–30 | 2 | 28 (20–35) | 10 | 12 (2–50) | 12 | 17 (2–50) | 9 | 10 (2–35) | 3 | 28 (25–50) | ||
30–35 | 6 | 36 (3–250) | 6 | 10 (1–15) | 12 | 13 (1–250) | 10 | 10 (1–37) | 1 | 250 | 1 | 57 |
35–40 | 7 | 10 (2–30) | 9 | 15 (5–40) | 16 | 14 (2–40) | 13 | 13 (5–40) | 1 | 25 | 1 | 28 |
40–45 | 5 | 60 (17–300) | 5 | 5 (1–100) | 10 | 38 (1–300) | 7 | 17 (1–61) | 2 | 75 (50–100) | 1 | 300 |
45–50 | 5 | 10 (4–170) | 6 | 11 (4–20) | 11 | 10 (4–170) | 9 | 10 (4–170) | 2 | 10 (4–16) | ||
50–55 | 9 | 20 (7–40) | 6 | 12 (1–20) | 15 | 13 (1–40) | 9 | 11 (1–40) | 6 | 21 (13–25) | ||
55–60 | 2 | 20 (20–20) | 5 | 14 (4–100) | 7 | 15 (4–100) | 6 | 17 (4–100) | ||||
50–65 | 1 | 55 | 5 | 30 (13–74) | 6 | 35 (13–74) | 6 | 35 (13–74) | ||||
65–70 | 1 | 4 | 2 | 32 (9–55) | 3 | 9 (4–55) | 3 | 9 (4–55) | ||||
70+ | 1 | 7 | 1 | 15 | 2 | 11 (7–15) | 2 | 11 (7–15) |
Determinants of fracture susceptibility
Bone mineral density
Total (mean ± SD) | Type I (mean ± SD) | Type III (mean ± SD) | Type IV (mean ± SD) |
P value | |
---|---|---|---|---|---|
Bone mineral density and massa, total body | |||||
Number of patients | 90 | 68 | 9 | 11 | |
BMC total body, kg | 2.26 ± 0.66 | 2.40 ± 0.50 | 0.99 ± 0.32 | 2.38 ± 0.50 | <0.001 |
BMD total body, g/cm² | 1.10 ± 0.11 | 1.11 ± 0.09 | 0.93 ± 0.09 | 1.14 ± 0.10 | <0.001 |
Z, BMD total body | −0.39 ± 1.03 | −0.29 ± 1.02 | −1.31 ± 0.92 | −0.45 ± 0.89 | 0.02 |
Bone mineral density and mass (L2–L4) and total hip without pathology | |||||
BMC L2–L4, g | 37.28 ± 8.86 (52) | 37.60 ± 8.78 (47) | –c
| 34.27 ± 10.07 (5) | 0.43 |
BMC total hip, g | 27.47 ± 7.69 (68)b
| 26.97 ± 7.68 (61) | –c
| 30.20 ± 8.74 (6) | 0.90 |
BMD L2–L4, g/cm² | 0.93 ± 0.14 (52) | 0.95 ± 0.14 (47) | –c
| 0.82 ± 0.12 (5) | 0.05 |
BMD total hip, g/cm² | 0.85 ± 0.17 (68)b
| 0.84 ± 0.16 (61) | –c
| 0.89 ± 0.22 (6) | 0.51 |
Z, BMD L2–L4 | −1.99 ± 1.09 (52) | −1.87 ± 1.06 (47) | –c
| −3.19 ± 0.66 (5) | 0.01 |
Z, BMD total hip | −1.15 ± 1.22 (68)b
| −1.55 ± 1.28 (61) | –c
| −1.12 ± 1.77 (6) | 0.32 |
Osteopenia and osteoporosis
N
| BMD T score [mean ± SD (range)] | Osteopenia T score = −1.0 > −2.5, number (%) | Osteoporosis T score ≤ −2.5, number (%) | |
---|---|---|---|---|
From total body | ||||
Total | 90a
| −0.83 ± 1.30 (−4.70–1.90) | 27 (30) | 9 (10) |
OI type I | 68 | −0.61 ± 1.11 (−3.70–1.90) | 19 (28) | 3 (5) |
OI type III | 9 | −2.74 ± 1.30 (−4.70–0.90) | 2 (22) | 6 (67) |
OI type IV | 11 | −0.73 ± 1.11 (−2.10−1.10) | 5 (46) | 0 |
From total hip | ||||
Total | 68b
| −1.52 ± 1.33 (−4.60–1.70) | 34 (50) | 10 (21) |
OI type I | 61 | −1.55 ± 1.28 (−4.60−1.70) | 30 (47) | 15 (23) |
OI type IV | 6 | −1.55 ± 1.72 (−3.80–0.80) | 5 (63) | 1 (13) |
Calcium metabolism
Total [mean ± SD (range)] | OI type I [mean ± SD (range)] | OI type III [mean ± SD (range)] | OI type IV [mean ± SD (range)] |
P value | |
---|---|---|---|---|---|
N
a
|
N
|
N
|
N
| ||
PTH (1.5–7.0 pmol/lb) | 4.0 ± 1.8 (1.0–8.5) | 3.8 ± 1.7 (1.0–8.1) | 4.3 ± 2.0 (1.2–7.3) | 4.9 ± 1.9 (1.7–8.5) | 0.13 |
89 | 68 | 8 | 11 | ||
s-iCa2+ (1.18–1.35 mmol/lb) | 1.24 ± 0.37 (1.17–1.32) | 1.23 ± 0.03 (1.17–1.31) | 1.24 ± 0.04 (1.20–1.31) | 1.25 ± 0.05 (1.17–1.32) | 0.43 |
88 | 67 | 8 | 11 | ||
25(OH)D (37–131 nmol/lb) | 75 ± 29 (22–198) | 78 ± 29 (27–198) | 52 ± 13 (34–73) | 74 ± 32 (22–131) | 0.05 |
91 | 70 | 8 | 11 |