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Normal bone density but altered geometry in girls with Turner syndrome

  • Roland Schweizer ORCID logo EMAIL logo , Judith Mayer and Gerhard Binder
Published/Copyright: February 2, 2023
Journal of Pediatric Endocrinology and Metabolism
From the journal Journal of Pediatric Endocrinology and Metabolism Volume 36 Issue 3

Abstract

Objectives

Data on bone density and stability in Turner syndrome (TS) are contradictory. A confounding factor for interpretation is short stature. The aim was to measure bone density, geometry and stability in girls with TS compared to idiopathic short stature (ISS).

Methods

From 1999 to 2008, 59 girls with TS (35 prepubertal) were evaluated by pQCT. Mean age was 8.9 in prepubertal and 17.3 years in adolescent girls. Mean height was −3.1 and −1.8 SDS in prepubertal treatment-free and in adolescent, formerly rhGH-treated girls. For comparison, 18 prepubertal ISS girls were studied (age 7.7 years; height −3.3 SDS). Examination of radius with pQCT (XCT 2000). Cortical (CD) and trabecular density (TD), total bone area (TBA), cortical area (CA), cortical thickness, muscle area and strength strain index (SSI) were determined and compared with height related references.

Results

In prepubertal girls with TS, TD and CD were normal (0.55 and 0.90 SDS) and comparable to ISS (0.95 and 1.53 SDS). TBA was greater in girls with TS than in ISS (0.87 vs. −0.33 SDS) whereas CA was similar (1.48 vs. 1.43 SDS). The SSI was comparable (1.61 vs. 1.56 SDS). Adolescent girls with TS showed similar results with a TD of 0.48 SDS, a CD of −0.32, TBA of 1.99, a CA of −0.05 and an SSI of 0.88 SDS.

Conclusions

The observations are consistent with normal bone density and stability but altered bone geometry in prepubertal and substituted adolescent girls with TS. This peculiarity may reflect SHOX deficiency. We therefore think that timely and adequate estrogen substitution could prevent bone loss in TS.

Keywords: bone development; muscle; osteoporosis; SHOX deficiency; Turner syndrome
Corresponding author: Prof. Dr. Roland Schweizer, Universität Tübingen, Klinik für Kinder- und Jugendmedizin, Hoppe-Seyler-Str. 1, 72076 Tübingen, Germany, Phone: +49 7071 2983781, Fax: +49 7071 294157, E-mail:

Acknowledgments

The authors thank Margarete Kaipf for her help in surveying the patients. The authors thank Michael B. Ranke for the establishment of the observational study on bone in children with GH treatment in Tübingen; and all girls and young women with Turner syndrome who participated in the study.

  1. Research funding: The study was conducted with help of on-board resources.

  2. Author contributions: Roland Schweizer: Idea for the study, conception, data analysis, manuscript preparation; Judith Mayer: patient recruitment, discussion, manuscript correction; Gerhard Binder: discussion, manuscript preparation, study supervision.

  3. Competing interests: The authors declare no conflict of interest.

  4. Informed consent: Written informed consent were obtained from the children’s legal guardians.

  5. Ethical approval: The study protocol was reviewed and approved by the Ethics Committee of the University Tübingen (approval number 96/2002) and was conducted in accordance with the Declaration of Helsinki.

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Supplementary Material

This article contains supplementary material (https://doi.org/10.1515/jpem-2022-0516).

Received: 2022-10-10
Accepted: 2023-01-09
Published Online: 2023-02-02
Published in Print: 2023-03-28

© 2023 Walter de Gruyter GmbH, Berlin/Boston

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  2. Editorial
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  10. Evaluation of a nurse-led counselling intervention on selected outcome variables for parents of children with congenital adrenal hyperplasia
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  12. An open-label extension of a phase 2 dose-finding study of once-weekly somatrogon vs. once-daily Genotropin in children with short stature due to growth hormone deficiency: results following 5 years of treatment
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  21. Case Reports
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https://doi.org/10.1515/jpem-2022-0516
Keywords for this article
bone development; muscle; osteoporosis; SHOX deficiency; Turner syndrome

Articles in the same Issue

  1. Frontmatter
  2. Editorial
  3. Thyroid – what is a healthy thyroid function test?
  4. Review
  5. Thyroid storm in pediatrics: a systematic review
  6. Opinion Paper
  7. DSD/intersex: historical context and current perspectives
  8. Original Articles
  9. Predictive value of 6 h postoperative parathyroid hormone level on permanent hypoparathyroidism in pediatric total thyroidectomy: a pilot study
  10. Evaluation of a nurse-led counselling intervention on selected outcome variables for parents of children with congenital adrenal hyperplasia
  11. The relationship between estrogen and subsequent growth restriction among adolescents with heavy menstrual bleeding at menarche
  12. An open-label extension of a phase 2 dose-finding study of once-weekly somatrogon vs. once-daily Genotropin in children with short stature due to growth hormone deficiency: results following 5 years of treatment
  13. Normal bone density but altered geometry in girls with Turner syndrome
  14. Newborn screening for inborn errors of metabolism in a northern Chinese population
  15. Evaluation of serum telomerase activity in normal-weight young girls with polycystic ovary syndrome and its relation to metabolic parameters
  16. Early onset of puberty during COVID-19 pandemic lockdown: experience from two Pediatric Endocrinology Italian Centers
  17. Using change in predicted adult height during GnRH agonist treatment for individualized treatment decisions in girls with central precocious puberty
  18. Short Communications
  19. Long-term experience with the use of a single histrelin implant beyond one year in patients with central precocious puberty
  20. Relationships among biochemical measures in children with diabetic ketoacidosis
  21. Case Reports
  22. Hyperthyroidism in McCune–Albright Syndrome – a case report
  23. Sexual precocity in the setting of parental use of a compounded testosterone cream: case report and review of the literature
  24. Intracardiac thrombosis following intravenous zoledronate treatment in a child with steroid-induced osteoporosis
  25. Identification of a novel mutation in the ALDOB gene in hereditary fructose intolerance
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