Reduced Volumetric Bone Mineral Density of the Spine in Adolescent Rett Girls with Scoliosis
In advanced Rett syndrome (RTT), limited or complete loss of ambulation, nutritional problems and scoliosis are unfavorable factors for bone mineral density (BMD). Still, there are few data available in this research area. Spinal quantitative computed tomography (QCT) allows an exact measurement of...
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| Format: | Article |
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MDPI AG
2022-12-01
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| Series: | Children |
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| Online Access: | https://www.mdpi.com/2227-9067/9/12/1902 |
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| author | Konstantinos Tsaknakis Jan H. Kreuzer Friederike Luise Metzger Katharina Jäckle Katja A. Lüders Lena Braunschweig Heiko M. Lorenz Anna K. Hell |
| author_facet | Konstantinos Tsaknakis Jan H. Kreuzer Friederike Luise Metzger Katharina Jäckle Katja A. Lüders Lena Braunschweig Heiko M. Lorenz Anna K. Hell |
| author_sort | Konstantinos Tsaknakis |
| collection | DOAJ |
| description | In advanced Rett syndrome (RTT), limited or complete loss of ambulation, nutritional problems and scoliosis are unfavorable factors for bone mineral density (BMD). Still, there are few data available in this research area. Spinal quantitative computed tomography (QCT) allows an exact measurement of the volumetric BMD (vBMD) in this patient group. Two examiners measured vBMD of thoracic and lumbar vertebrae on asynchronous calibrated CTs that were acquired prior to surgical scoliosis correction (n = 21, age 13.6 ± 2.5 years). The values were compared to age- and sex-matched healthy controls to additionally derive Z-scores (n = 22, age 13.8 ± 2.0 years). The results showed the most significant reduction of vBMD values in non-ambulatory RTT patients, with <i>p</i> < 0.001 and average BMD-Z-score −1.5 ± 0.2. In the subgroup comparison, non-ambulatory patients with valproate treatment had significant lower values (<i>p</i> < 0.001) than ambulatory patients without valproate therapy, with an average BMD-Z-score of −2.3 ± 0.2. Comparison of the Z-scores to critical BMD thresholds of 120 and 80 mg/cm<sup>3</sup> showed normal Z-scores in case of the ambulatory RTT subgroup, as opposed to BMD-Z-scores of the non-ambulatory RTT subgroups, which were partially below osteopenia-equivalent values. Furthermore, valproate treatment seems to have a direct effect on vBMD in RTT patients and when combined with loss of ambulation, BMD-Z-scores are reduced to osteoporosis-equivalent levels or even further. |
| first_indexed | 2024-03-09T17:11:41Z |
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| institution | Directory Open Access Journal |
| issn | 2227-9067 |
| language | English |
| last_indexed | 2024-03-09T17:11:41Z |
| publishDate | 2022-12-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Children |
| spelling | doaj.art-ac782325c15b4d47944f9d8cb938e1422023-11-24T14:01:42ZengMDPI AGChildren2227-90672022-12-01912190210.3390/children9121902Reduced Volumetric Bone Mineral Density of the Spine in Adolescent Rett Girls with ScoliosisKonstantinos Tsaknakis0Jan H. Kreuzer1Friederike Luise Metzger2Katharina Jäckle3Katja A. Lüders4Lena Braunschweig5Heiko M. Lorenz6Anna K. Hell7Paediatric Orthopaedics, Department of Trauma, Orthopaedic and Plastic Surgery, University Medical Center, 37077 Göttingen, GermanyPaediatric Orthopaedics, Department of Trauma, Orthopaedic and Plastic Surgery, University Medical Center, 37077 Göttingen, GermanyPaediatric Orthopaedics, Department of Trauma, Orthopaedic and Plastic Surgery, University Medical Center, 37077 Göttingen, GermanyPaediatric Orthopaedics, Department of Trauma, Orthopaedic and Plastic Surgery, University Medical Center, 37077 Göttingen, GermanyPaediatric Orthopaedics, Department of Trauma, Orthopaedic and Plastic Surgery, University Medical Center, 37077 Göttingen, GermanyPaediatric Orthopaedics, Department of Trauma, Orthopaedic and Plastic Surgery, University Medical Center, 37077 Göttingen, GermanyPaediatric Orthopaedics, Department of Trauma, Orthopaedic and Plastic Surgery, University Medical Center, 37077 Göttingen, GermanyPaediatric Orthopaedics, Department of Trauma, Orthopaedic and Plastic Surgery, University Medical Center, 37077 Göttingen, GermanyIn advanced Rett syndrome (RTT), limited or complete loss of ambulation, nutritional problems and scoliosis are unfavorable factors for bone mineral density (BMD). Still, there are few data available in this research area. Spinal quantitative computed tomography (QCT) allows an exact measurement of the volumetric BMD (vBMD) in this patient group. Two examiners measured vBMD of thoracic and lumbar vertebrae on asynchronous calibrated CTs that were acquired prior to surgical scoliosis correction (n = 21, age 13.6 ± 2.5 years). The values were compared to age- and sex-matched healthy controls to additionally derive Z-scores (n = 22, age 13.8 ± 2.0 years). The results showed the most significant reduction of vBMD values in non-ambulatory RTT patients, with <i>p</i> < 0.001 and average BMD-Z-score −1.5 ± 0.2. In the subgroup comparison, non-ambulatory patients with valproate treatment had significant lower values (<i>p</i> < 0.001) than ambulatory patients without valproate therapy, with an average BMD-Z-score of −2.3 ± 0.2. Comparison of the Z-scores to critical BMD thresholds of 120 and 80 mg/cm<sup>3</sup> showed normal Z-scores in case of the ambulatory RTT subgroup, as opposed to BMD-Z-scores of the non-ambulatory RTT subgroups, which were partially below osteopenia-equivalent values. Furthermore, valproate treatment seems to have a direct effect on vBMD in RTT patients and when combined with loss of ambulation, BMD-Z-scores are reduced to osteoporosis-equivalent levels or even further.https://www.mdpi.com/2227-9067/9/12/1902Rett syndromevBMDvolumetric bone mineral densityscoliosisZ-scorevalproate |
| spellingShingle | Konstantinos Tsaknakis Jan H. Kreuzer Friederike Luise Metzger Katharina Jäckle Katja A. Lüders Lena Braunschweig Heiko M. Lorenz Anna K. Hell Reduced Volumetric Bone Mineral Density of the Spine in Adolescent Rett Girls with Scoliosis Children Rett syndrome vBMD volumetric bone mineral density scoliosis Z-score valproate |
| title | Reduced Volumetric Bone Mineral Density of the Spine in Adolescent Rett Girls with Scoliosis |
| title_full | Reduced Volumetric Bone Mineral Density of the Spine in Adolescent Rett Girls with Scoliosis |
| title_fullStr | Reduced Volumetric Bone Mineral Density of the Spine in Adolescent Rett Girls with Scoliosis |
| title_full_unstemmed | Reduced Volumetric Bone Mineral Density of the Spine in Adolescent Rett Girls with Scoliosis |
| title_short | Reduced Volumetric Bone Mineral Density of the Spine in Adolescent Rett Girls with Scoliosis |
| title_sort | reduced volumetric bone mineral density of the spine in adolescent rett girls with scoliosis |
| topic | Rett syndrome vBMD volumetric bone mineral density scoliosis Z-score valproate |
| url | https://www.mdpi.com/2227-9067/9/12/1902 |
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