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Near Adult Height in Girls with Turner Syndrome Treated with Growth Hormone Following Either Induced or Spontaneous Puberty - 19/08/19

Doi : 10.1016/j.jpeds.2019.04.056 
Roberto Lanes, MD 1, , Anders Lindberg, MS 2, Martin Carlsson, MS 3, Dionisios Chrysis, MD 4, Ferah Aydin, RN 2, Cecilia Camacho-Hübner, MD 3, Donna King, PhD 3, Marsha Davenport, MD 5
1 Pediatric Endocrine Unit, Hospital de Clinica Caracas, Caracas, Venezuela 
2 Pfizer Health AB, Endocrine Care, Sollentuna, Sweden 
3 Pfizer Inc., Endocrine Care, New York, NY 
4 Division of Endocrinology and Diabetes, Department of Pediatrics, Medical School, University of Patras, Patras, Greece 
5 Division of Pediatric Endocrinology, University of North Carolina, Chapel Hill, NC 

Reprint requests: Roberto Lanes, MD

Abstract

Objective

To understand whether spontaneous vs induced puberty and the type and route of estrogen influence the height of girls with Turner syndrome on growth hormone (GH).

Study design

Search of an international database of children treated with GH revealed 772 girls with Turner syndrome followed from GH initiation to near adult height. Data from girls with sustained spontaneous puberty (n = 145) were compared with those requiring estrogens for induction or maintenance of puberty (n = 627).

Results

At GH start, mean age (7.5 vs 7.9 years), weight (−1.7 vs −1.7 SDS), and body mass index (0.2 SDS vs 0.1 SDS) were similar for girls with spontaneous puberty and with induced puberty. Although those girls with spontaneous puberty were shorter than those with induced puberty, when midparental height was taken into consideration, starting heights in both groups averaged −2.8 SDS. Both groups received approximately 0.3 mg/kg/week of GH. Girls with spontaneous puberty initiated puberty and reached near adult height earlier than girls with induced puberty (12.6 ± 1.8 years vs 13.4 ± 1.4 years and 16.0 ± 1.3 years vs 16.9 ± 1.4 years, respectively). Although girls with spontaneous puberty grew more in the first year of GH therapy and between the onset of puberty and near adult height (11.0 cm vs 9.3 cm), height SDS at near adult height and the length of time in puberty before reaching near adult height were comparable. A 45,X karyotype was detected in 22.1% of girls with spontaneous puberty and in 58.4% of girls with induced puberty. Patients receiving transdermal estrogens did not grow better than those on oral estrogens. Adverse event reporting was comparable between groups.

Conclusions

Girls with Turner syndrome with spontaneous puberty tended to grow better in response to GH than girls with induced puberty, but not enough to produce a difference in height SDS at near adult height.

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Keywords : Turner syndrome, spontaneous puberty, induced puberty, near adult height, growth hormone, estrogen replacement, karyotype

Abbreviations : CE, E2, EE, GH, Ht-SDS, SAE, TD


Plan


 KIGS is sponsored by Pfizer Inc. C.C.-H. and M.C. are full-time employees of Pfizer Inc. D.K. was employed by Pfizer Inc. at the time of this study. A.L. and F.A. were employed by Pfizer Health AB. R.L. and D.C. were members of the KIGS Steering Committee at the time of this study. No authors were compensated for their contributions to this manuscript.


© 2019  Elsevier Inc. Tous droits réservés.
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Vol 212

P. 172 - septembre 2019 Retour au numéro
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