Background: Low bone mass is a frequent and early complication of girls with Rett syndrome. As a consequence of the low bone mass, Rett patients are at an increased risk of fragility fractures. This study aimed to investigate the long-term influences of mobility on bone status in girls with Rett syndrome. Methods: In 58 girls with Rett syndrome, biochemical parameters and quantitative ultrasound parameters at phalanges (amplitude-dependent speed of sound: AD-SoS and bone transmission time: BTT) were measured at baseline and after 5 and 10 years. The subjects were divided into two groups: nonambulatory (n = 28) and ambulatory (n = 30). Results: In nonambulatory Rett subjects, the values of AD-SoS and BTT were significantly lower than in ambulatory Rett subjects at each time point. However, during the 10-year follow-up both ambulatory and nonambulatory Rett patients showed a similar worsening in their bone status. Conclusion: This longitudinal study suggests that both ambulatory and nonambulatory Rett subjects present a progressive deterioration of bone status as assessed by quantitative ultrasound parameters, and the ambulatory impairment and the nutritional status seem to play a key role in the deterioration of bone status. © 2018, International Pediatric Research Foundation, Inc.

Caffarelli, C., Francolini, V., Hayek, J., Valacchi, G., Giannotti, S., Nuti, R., et al. (2019). Bone status in relation to ambulatory performance in girls with Rett syndrome: a 10-year longitudinal study. PEDIATRIC RESEARCH, 85(5), 639-643 [10.1038/s41390-018-0111-z].

Bone status in relation to ambulatory performance in girls with Rett syndrome: a 10-year longitudinal study

Caffarelli C.;Francolini V.;Valacchi G.;Giannotti S.;Nuti R.;Gonnelli S.
2019-01-01

Abstract

Background: Low bone mass is a frequent and early complication of girls with Rett syndrome. As a consequence of the low bone mass, Rett patients are at an increased risk of fragility fractures. This study aimed to investigate the long-term influences of mobility on bone status in girls with Rett syndrome. Methods: In 58 girls with Rett syndrome, biochemical parameters and quantitative ultrasound parameters at phalanges (amplitude-dependent speed of sound: AD-SoS and bone transmission time: BTT) were measured at baseline and after 5 and 10 years. The subjects were divided into two groups: nonambulatory (n = 28) and ambulatory (n = 30). Results: In nonambulatory Rett subjects, the values of AD-SoS and BTT were significantly lower than in ambulatory Rett subjects at each time point. However, during the 10-year follow-up both ambulatory and nonambulatory Rett patients showed a similar worsening in their bone status. Conclusion: This longitudinal study suggests that both ambulatory and nonambulatory Rett subjects present a progressive deterioration of bone status as assessed by quantitative ultrasound parameters, and the ambulatory impairment and the nutritional status seem to play a key role in the deterioration of bone status. © 2018, International Pediatric Research Foundation, Inc.
Caffarelli, C., Francolini, V., Hayek, J., Valacchi, G., Giannotti, S., Nuti, R., et al. (2019). Bone status in relation to ambulatory performance in girls with Rett syndrome: a 10-year longitudinal study. PEDIATRIC RESEARCH, 85(5), 639-643 [10.1038/s41390-018-0111-z].
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11365/1124140