hrp0095hdi2.2 | How Do I… Session 2 | ESPE2022

How do I replace oestrogens in Turner syndrome?

Sas Theo

In Turner Syndrome (TS) the ovaries usually start to involute within 4 or 5 months of gestation. Consequently, the majority of patients have diminished ovarian estrogen production leading to the (Partial) absence of puberty. These girls need estrogen therapy to induce pubertal maturation. For many parents of girls with TS, talking about ovaries, puberty and, particularly, infertility is a stressful thing. However, timely age-appropriate disclosure about these subjects, startin...

hrp0086wg3.5 | ESPE Turner Syndrome Working Group (TS) | ESPE2016

Estradiol Supplementation in Turner Syndrome: An Update

Sas Theo

Background: Most Turner syndrome patients will need estrogen replacement therapy – first for induction of puberty and later for maintaining secondary sex characteristics, attaining peak bone mass, and normalizing uterine growth for possible pregnancy later.Results: An updated overview of the different estrogen replacement therapy regimen will be presented....

hrp0082p1-d1-198 | Reproduction | ESPE2014

Stability Conditions in Estradiol Matrix Patches; in vitro Studies for Application in Pediatrics

Ankarberg-Lindgren Carina , Sas Theo

Background: We have previously shown that estradiol (E2) matrix patches for adults could be cut in smaller pieces to administer low doses for pubertal induction in girls with hypogonadism. With a slow increase of the patch size during a few years, serum E2 levels in normal girls undergoing puberty can be closely mimicked.Objective and hypotheses: To evaluate storage conditions once the patch has been cut.Metho...

hrp0084wg6.5 | Turner Syndrome | ESPE2015

Report on the Progress and Difficulties on Transdermal Estradiol Supplementation in Europe

Sas Theo , Ankarberg-Lindgren Carina

Background: The Turner syndrome (TS) Working Group of the ESPE intends to start an international study comparing transdermal estradiol (E2) vs oral E2 in Europe in girls with TS. For this, stable and reliable products for children are needed, for use in different countries with different climates. It has previously been shown that E2 matrix patches for adults can be cut in smaller pieces to administer low doses for pubertal induction in girls. The Turner Working Group plan to ...

hrp0089p1-p239 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology P1 | ESPE2018

Sustainability of Estradiol Drug Concentrations in Cut Matrix Patches; A Study of Different Brands with Potential Use for Pubertal Induction

Ankarberg-Lindgren Carina , Gawlik Aneta , Kristrom Berit , Mazzanti Laura , Sas Theo CJ

Background: The estimated estradiol (E2) dose required for pubertal induction in hypogonadal girls is about 1/10 of the adult dose. Previous studies have shown that it is possible to provide an individualized and physiological dose for pubertal induction by cutting the patch into smaller pieces. However, the manufacturers do not guarantee stability or utility of cut E2 patches, primarily designed for postmenopausal women, and have no interest in that eval...

hrp0097p1-127 | Growth and Syndromes | ESPE2023

Effects and safety of growth hormone (GH) treatment in 6 children with pycnodysostosis

Renes Judith , Sas Theo , Clement-de Boer Agnes , Zwaveling-Soonawala Nitash , van der Kaay Danielle , Hokken-Koelega Anita

Background: Pycnodysostosis is a very rare autosomal recessive skeletal dysplasia caused by cathepsin K deficiency. It is characterized by extreme short stature resulting in an adult height in males typically <150 cm and in females <134 cm. Bone-fragility and frequent fractures are present. There are few case-reports on the effects of GH treatment.Objective: To evaluate the effect and safety of GH in 6 patients wit...

hrp0095p2-158 | Growth and Syndromes | ESPE2022

Global recall of a growth hormone jet delivered device: experiences with children forced to switch from a needle free system to a growth hormone device with needle.

P. van Eck Judith , S. Renes Judith , J.C. Sas Theo , L.T. van den Akker Erica , C.M. van der Kaay Daniëlle

Introduction: Growth hormone (GH) treatment is essential for growth in children with GH deficiency. Also short children born small for gestational age (SGA), and children with syndromes like Turner Syndrome (TS), Noonan Syndrome (NS) and Silver Russel Syndrome (SRS) can benefit from GH treatment. For children with needle anxiety GH delivered by a jet device can be a solution for the daily subcutaneous treatment for many years. In 2021, a global recall of the o...

hrp0092rfc2.5 | Bone, Growth Plate and Mineral Metabolism Session 1 | ESPE2019

Long Term Effects of Treatment with Oxandrolone (Ox) in Addition to Growth Hormone (GH) in Girls with Turner Syndrome (TS) on Bone Mineral Density in Adulthood

Breunis Leonieke , Boer Pieter , Freriks Kim , Menke Leonie , Boot Annemieke , Wit Jan Maarten , Otten Barto , Muinck Keizer-Schrama Sabine de , Hermus Ad , Timmers Henri , Sas Theo

Introduction: Ox in a dose of 0.03-0.05 mg/kg per day in addition to GH treatment significantly increases adult height in TS more than GH alone. To date, the long term effects of Ox in childhood on bone mineral density (BMD) in adulthood are unknown.Methods: This is a follow-up study of a previous randomized controlled trial, performed in the Netherlands. In the original trial, 133 girls were treated with GH. Placebo (Pl...