Evaluation of Growth Characteristics and Final Height of Cases Diagnosed with Noonan Syndrome on Growth Hormone Treatment

Zeynep ����klar, Merih Berbero��lu, Sirmen K��z��lcan ��etin, Melek Y��ld��z, Serap Turan, ����kran Darcan, Semra ��etinkaya, Nihal Hatipo��lu, Ruken Y��ld��r��m, Korcan Demir, ��znur Vermezo��lu, Zehra Yava�� Abal��, Deniz ��zalp K��z��lay, Nilay G��rkem Erdo��an, ��lk�� G��l ��iraz, Zerrin Orbak, ��lker Tolga ��zgen, Aysun Bideci, Beray Selver Eklio��lu, Esin Karak��l���� ��zturan, G��rkan Tar����n, Abdullah Bereket, Feyza Darendeliler
Author Information
  1. Zeynep ����klar: Ankara University Faculty of Medicine, Department of Pediatric Endocrinology, Ankara, T��rkiye ORCID
  2. Merih Berbero��lu: Ankara University Faculty of Medicine, Department of Pediatric Endocrinology, Ankara, T��rkiye ORCID
  3. Sirmen K��z��lcan ��etin: Ankara University Faculty of Medicine, Department of Pediatric Endocrinology, Ankara, T��rkiye ORCID
  4. Melek Y��ld��z: ��stanbul University-��stanbul Faculty of Medicine, Department of Pediatric Endocrinology, ��stanbul, T��rkiye ORCID
  5. Serap Turan: Marmara University Faculty of Medicine, Department of Pediatric Endocrinology, ��stanbul, T��rkiye ORCID
  6. ����kran Darcan: Ege University Faculty of Medicine, Department of Pediatric Endocrinology, ��zmir, T��rkiye ORCID
  7. Semra ��etinkaya: University of Health Sciences T��rkiye, Ankara Dr. Sami Ulus Child Health and Diseases Health Implementation and Research Center, Ankara, T��rkiye ORCID
  8. Nihal Hatipo��lu: Erciyes University Faculty of Medicine, Department of Pediatric Endocrinology, Kayseri, T��rkiye ORCID
  9. Ruken Y��ld��r��m: Diyarbak��r Child Diseases Hospital, Clinic of Pediatric Endocrinology, Diyarbak��r, T��rkiye ORCID
  10. Korcan Demir: Dokuz Eyl��l University Faculty of Medicine, Department of Pediatric Endocrinology, ��zmir, T��rkiye ORCID
  11. ��znur Vermezo��lu: Trakya University Faculty of Medicine, Department of Pediatric Endocrinology, Edirne, T��rkiye ORCID
  12. Zehra Yava�� Abal��: Marmara University Faculty of Medicine, Department of Pediatric Endocrinology, ��stanbul, T��rkiye ORCID
  13. Deniz ��zalp K��z��lay: Ege University Faculty of Medicine, Department of Pediatric Endocrinology, ��zmir, T��rkiye ORCID
  14. Nilay G��rkem Erdo��an: University of Health Sciences T��rkiye, Ankara Dr. Sami Ulus Child Health and Diseases Health Implementation and Research Center, Ankara, T��rkiye ORCID
  15. ��lk�� G��l ��iraz: Erciyes University Faculty of Medicine, Department of Pediatric Endocrinology, Kayseri, T��rkiye ORCID
  16. Zerrin Orbak: Atat��rk University Faculty of Medicine, Department of Pediatric Endocrinology and Diabetes, Ankara, T��rkiye ORCID
  17. ��lker Tolga ��zgen: Biruni University Faculty of Medicine, Department of Pediatric Endocrinology, ��stanbul, T��rkiye ORCID
  18. Aysun Bideci: Gazi University Faculty of Medicine, Department of Pediatric Endocrinology, Ankara, T��rkiye ORCID
  19. Beray Selver Eklio��lu: Necmettin Erbakan University Faculty of Medicine, Department of Pediatric Endocrinology, Konya, T��rkiye ORCID
  20. Esin Karak��l���� ��zturan: Marmara University Faculty of Medicine, Department of Pediatric Endocrinology, ��stanbul, T��rkiye ORCID
  21. G��rkan Tar����n: Adana City Training and Research Hospital, Clinic of Pediatric Endocrinology Adana, T��rkiye ORCID
  22. Abdullah Bereket: Marmara University Faculty of Medicine, Department of Pediatric Endocrinology, ��stanbul, T��rkiye ORCID
  23. Feyza Darendeliler: ��stanbul University-��stanbul Faculty of Medicine, Department of Pediatric Endocrinology, ��stanbul, T��rkiye ORCID

Abstract

Objective: Proportional short stature is one of the most important features of Noonan syndrome (NS), and adult height often remains below the third percentile. Although the pathophysiology of short stature in NS patients is not fully understood, it has been shown that growth hormone (GH) treatment is beneficial in NS, significantly improving height in respect to the results of short and long-term GH treatment.
Methods: In this national retrospective cohort study, patients with NS who reached final height from 14 centers were evaluated. patients were stratified by sex and treatment with or without GH and final height outcomes were compared.
Results: The study included 67 patients with NS, of whom 53 (79.1%) with mean follow-up 5.6 years, received GH treatment. At presentation height standard deviation scores (SDS) of the subjects who were started on GH tended to be shorter than those who did not (-3.26��1.07 vs. -2.53��1.23). In girls mean final height and final height SDS in those using GH vs not using GH were 150.1 cm (-2.17 SDS) vs. 147.4 cm (-2.8 SDS), respectively, and for boys these values were 162.48 cm (-1.81 SDS) vs 157.46 cm (-2.68 SDS), respectively. The ��height SDS value of the cases was significantly higher in the group receiving GH compared to those not receiving GH (1.36��1.12 SD vs. -0.2��1.24, p<0.001). Cardiac findings remained stable in two patients with hypertrophic cardiomyopathy who received GH treatment. No significant side effects were observed in any patient during follow-up.
Conclusion: In patients with NS who reach their final height, a significant increase in height was observed with GH treatment. An increase of approximately +1.4 SDS may be achieved. GH treatment appears to be safe and effective in NS.

Keywords

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MeSH Term

Humans
Noonan Syndrome
Male
Female
Body Height
Retrospective Studies
Human Growth Hormone
Child
Child, Preschool
Adolescent
Growth Disorders
Follow-Up Studies
Treatment Outcome

Chemicals

Human Growth Hormone

Word Cloud

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