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- W2890941456 abstract "We aim to evaluate the pattern of bone mass acquisition and fracture determinants in type 2/3 patients. DXA measurements of total body less head bone mineral density (TB-BMD and Z-score), bone mineral content (TB-BMC), fat mass (FM) and fat free mass (FFM) were obtained in 19 subjects with SMA (10 SMA2, 9 SMA3) and 19 controls, at baseline and every 12 months for 36 months. At baseline, the median age was 9.4 years. All patients underwent anthropometric (HT SDS, BMI SDS) and biochemical bone turnover evaluations. Six subjects (3 SMA2, 3 SMA3) reported fragility fractures. SMA2/3 subjects did not differ in age, HT SDS, BMI SDS, although SMA2 tended to be lower, thinner and with greater muscle atrophy than SMA3 from T0 to T36; controls were significantly higher than both SMA types at all times (p <0.0001). The BMI SDS was reduced over time in SMA2 with the same FMI (fat mass index). TB-BMD (T24 and T36) and TB-BMD Z-score (at all times) were significantly reduced in SMA2 compared to SMA3 (p <0.05) and significantly lower in SMA compared to controls (p <0.0001). BMC was significantly reduced in SMA2 compared to SMA3 (p <0.05) and in both groups compared to controls; in the upper limbs there was a significant reduction compared to controls only in SMA2. There was no significant difference in bone turnover parameters between controls and SMA2/3. DXA parameters did not discriminate between fractured and not fractured SMA patients. However, multiple regression analysis showed that visceral adiposity predicted fractures after correction for lean mass, skeletal dimensions, or the BMD-Z-score (R2 0.466-0.341, P <0.05). SMA2 have a generalized impairment of DXA parameters, although bone mass acquisition is also reduced in SMA3 compared to controls. In SMA patients, body composition and visceral adiposity would appear to be the major determinants of bone fragility." @default.
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- W2890941456 date "2018-10-01" @default.
- W2890941456 modified "2023-09-26" @default.
- W2890941456 title "SMA CLINICAL DATA, OUTCOME MEASURES AND REGISTRIES" @default.
- W2890941456 doi "https://doi.org/10.1016/j.nmd.2018.06.111" @default.
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