Optimal Pubertal Induction in Girls with Turner Syndrome Using Either Oral or Transdermal Estradiol: A Proposed Modern Strategy
| Autor: | Donaldson, M., Kristrom, B., Ankarberg-Lindgren, C., Verlinde, S., Van Alfen-Van Der Velden, J., Gawlik, A., Van Gelder, M. M. H. J., Sas, T., Agota, M., Akulevich, N., Albertsson-Wikland, K., Bober, E., Buyukgebiz, A., Carel, J. -C., Dacou-Voutetakis, C., De Muinck Keizer-Schrama, S., Gault, E. J., Ghizzoni, L., Kanaka-Gantenbein, C., Kurtev, A., Malecka-Tendera, E., Mazzanti, L., Norjavaara, E., Popovic, J., Ranke, M., Sallai, A., Stagi, S., Wasniewska, M., Zenaty, D., Zuckerman-Levin, N. |
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| Přispěvatelé: | Pediatrics |
| Rok vydání: | 2019 |
| Předmět: |
Pediatrics
medicine.medical_specialty Adolescent medicine.drug_class Endocrinology Diabetes and Metabolism Administration Oral Turner Syndrome 030209 endocrinology & metabolism Administration Cutaneous Healthcare improvement science Radboud Institute for Health Sciences [Radboudumc 18] 03 medical and health sciences 0302 clinical medicine Endocrinology 17β-estradiol Oral induction Puberty Transdermal induction Turner syndrome Humans Medicine Sexual Maturation Child Prospective cohort study Transdermal 030219 obstetrics & reproductive medicine Estradiol medicine.diagnostic_test business.industry Vascular disease Estrogen Replacement Therapy Vascular damage Radboud Institute for Molecular Life Sciences [Radboudumc 16] medicine.disease Regimen Blood pressure Estrogen Pediatrics Perinatology and Child Health Female business Lipid profile |
| Zdroj: | Hormone Research in Paediatrics, 91, 3, pp. 153-163 Hormone Research in Paediatrics, 91(3), 153-163. Karger Hormone Research in Paediatrics, 91, 153-163 |
| ISSN: | 1663-2826 1663-2818 |
| DOI: | 10.1159/000500050 |
| Popis: | Background: Most girls with Turner syndrome (TS) require pubertal induction with estrogen, followed by long term replacement. However, no adequately powered prospective studies comparing transdermal with oral 17β-estradiol administration exist. This reflects the difficulty of securing funding to study a rare condition with relatively low morbidity/mortality when competing against conditions such as cancer and vascular disease. Protocol Consensus: The TS Working Group of the European Society for Paediatric Endocrinology (ESPE) has agreed to both a 3-year oral and a 3-year transdermal regimen for pubertal induction. Prerequisites include suitable 17β-estradiol tablets and matrix patches to allow the delivery of incremental doses based on body weight. Study Proposal: An international prospective cohort study with single centre analysis is proposed in which clinicians and families are invited to choose either of the agreed regimens, usually starting at 11 years. We hypothesise that pubertal induction with transdermal estradiol will result in better outcomes for some key parameters. The primary outcome measure chosen is height gain during the induction period. Analysis: Assessment of the demographics and drop-out rates of patients choosing either oral or transdermal preparations; and appropriate analysis of outcomes including pubertal height gain, final height, liver enzyme and lipid profile, adherence/acceptability, cardiovascular health, including systolic and diastolic blood pressure and aortic root diameter and bone health. Conclusion: The proposed model of prospective data collection according to internationally agreed protocols aims to break the current impasse in obtaining evidence-based management for TS and could be applied to other rare paediatric endocrine conditions. |
| Databáze: | OpenAIRE |
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