Adult height improved over decades in patients with X-linked hypophosphatemia : a cohort study
© The Author(s) 2023. Published by Oxford University Press on behalf of European Society of Endocrinology. All rights reserved. For permissions, please e-mail: journals.permissionsoup.com..
OBJECTIVES: The aim of this study is to analyze height after cessation of growth (final height [FH]) and its evolution over the last decades in X-linked hypophosphatemia (XLH) patients in France, as the data on natural history of FH in XLH are lacking.
DESIGN: We performed a retrospective observational study in a large cohort of French XLH patients with available data on FH measurements.
MATERIALS AND METHODS: We divided patients into 3 groups according to their birth year: group 1 born between 1950 and 1974, group 2 born between 1975 and 2000, and group 3 born between 2001 and 2006, respectively, and compared their FHs.
RESULTS: A total of 398 patients were included. Mean FHs were the following: for group 1, -2.31 ± 1.11 standard deviation score (SDS) (n = 127), 156.3 ± 9.7 cm in men and 148.6 ± 6.5 cm in women; for group 2, -1.63 ± 1.13 SDS (n = 193), 161.6 ± 8.5 cm in men and 153.1 ± 7.2 cm in women; and for group 3, -1.34 ± 0.87 SDS (n = 78), 165.1 ± 5.5 cm in men and 154.7 ± 6 cm in women. We report a significant increase in mean FH SDS over 3 generations of patients, for both men and women (P < .001). Final height SDS in male (-2.08 ± 1.18) was lower than in female (-1.70 ± 1.12) (P = .002).
CONCLUSION: The FH of XLH patients in France increased significantly over the last decades. Even though men's FHs improved more than women's, men with XLH remain shorter reflecting a more severe disease phenotype. While the results are promising, most patients with XLH remain short leaving room for improvement.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:189 |
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| Enthalten in: |
European journal of endocrinology - 189(2023), 4 vom: 17. Okt., Seite 469-475 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Boros, Emese [VerfasserIn] |
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| Links: |
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| Themen: |
EC 3.4.24.- |
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| Anmerkungen: |
Date Completed 27.10.2023 Date Revised 27.10.2023 published: Print Citation Status MEDLINE |
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| doi: |
10.1093/ejendo/lvad144 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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NLM363247459 |
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| 245 | 1 | 0 | |a Adult height improved over decades in patients with X-linked hypophosphatemia |b a cohort study |
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| 500 | |a Date Revised 27.10.2023 | ||
| 500 | |a published: Print | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © The Author(s) 2023. Published by Oxford University Press on behalf of European Society of Endocrinology. All rights reserved. For permissions, please e-mail: journals.permissionsoup.com. | ||
| 520 | |a OBJECTIVES: The aim of this study is to analyze height after cessation of growth (final height [FH]) and its evolution over the last decades in X-linked hypophosphatemia (XLH) patients in France, as the data on natural history of FH in XLH are lacking | ||
| 520 | |a DESIGN: We performed a retrospective observational study in a large cohort of French XLH patients with available data on FH measurements | ||
| 520 | |a MATERIALS AND METHODS: We divided patients into 3 groups according to their birth year: group 1 born between 1950 and 1974, group 2 born between 1975 and 2000, and group 3 born between 2001 and 2006, respectively, and compared their FHs | ||
| 520 | |a RESULTS: A total of 398 patients were included. Mean FHs were the following: for group 1, -2.31 ± 1.11 standard deviation score (SDS) (n = 127), 156.3 ± 9.7 cm in men and 148.6 ± 6.5 cm in women; for group 2, -1.63 ± 1.13 SDS (n = 193), 161.6 ± 8.5 cm in men and 153.1 ± 7.2 cm in women; and for group 3, -1.34 ± 0.87 SDS (n = 78), 165.1 ± 5.5 cm in men and 154.7 ± 6 cm in women. We report a significant increase in mean FH SDS over 3 generations of patients, for both men and women (P < .001). Final height SDS in male (-2.08 ± 1.18) was lower than in female (-1.70 ± 1.12) (P = .002) | ||
| 520 | |a CONCLUSION: The FH of XLH patients in France increased significantly over the last decades. Even though men's FHs improved more than women's, men with XLH remain shorter reflecting a more severe disease phenotype. While the results are promising, most patients with XLH remain short leaving room for improvement | ||
| 650 | 4 | |a Observational Study | |
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a PHEX | |
| 650 | 4 | |a XLH | |
| 650 | 4 | |a final height | |
| 650 | 4 | |a quality of life | |
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| 700 | 1 | |a Audrain, Christelle |e verfasserin |4 aut | |
| 700 | 1 | |a Lecoq, Anne Lise |e verfasserin |4 aut | |
| 700 | 1 | |a Kamenicky, Peter |e verfasserin |4 aut | |
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| 700 | 1 | |a Mignot, Brigitte |e verfasserin |4 aut | |
| 700 | 1 | |a Girerd, Barbara |e verfasserin |4 aut | |
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