hrp0092p2-114 | Fat, Metabolism and Obesity | ESPE2019

Five Years' Follow-up of the Effect of Sex Steroid Hormone on Lipid and Glucose Metabolism in Girls with Turner Syndrome

Guo Song , Li Yanhong , Zhang Jun , Chen Qiuli , Ma Huamei , Chen Hongshan , Du Minlian

Objective: We sought to evaluate the role of long-term HRT on the lipid profile and glucose metabolism in girls with TS.Design: A pre-test/post-test observational study.Seting: Pediatric TS clinic in The First Affiated Hospital, Sun Yat-sen University.Patients: 56 girls with TS had accurate maintenance HRT data....

hrp0084p1-137 | Turner & Puberty | ESPE2015

Normalization of Puberty and Adult Height in Girls with Turner Syndrome, Randomised Trials vs Age and Dose at GH-Start

Kristrom Berit , Ankarberg-Lindgren Carina , Barrenas Marie-Louise , Nilsson Karlolof , Albertsson-Wikland Kerstin

Background: Early TS diagnosis permits early GH start and estradiol (E2) supplementation approaching adult height (AH) at normal age and within a normal range. However, higher age at diagnosis is still a challenge.Objective and hypotheses: The hypothesis from our long-term trials will result in knowledge for personalized treatment in order to obtain a psychological acceptable age at onset of puberty and still an attained AH within normal range...

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...

hrp0092fc2.6 | Bone, Growth Plate and Mineral Metabolism Session 1 | ESPE2019

Validation of a New Version of BoneXpert Bone Age in Children with Congenital Adrenal Hyperplasia (CAH), Precocious Puberty (PP), Growth Hormone Deficiency (GHD), Turner Syndrome (TS), and other Short Stature Diagnoses

Thodberg Hans Henrik , Martin David D

Background: The BoneXpert method for automated determination of bone age from hand X-rays is based on machine learning, so it lends itself naturally to be improved by adding more training data and using better learning algorithms. Currently, version 2 is running in 145 hospitals across Europe, and a new version 3 is rolled out in 2019.Objective and Hypotheses: The aim was to validate version 3 against manual ratings in r...

hrp0086p1-p638 | Growth P1 | ESPE2016

Retrospective Analysis of Growth Hormone (GH) Treatment Results in Children with Idiopathic Growth Hormone Deficiency (IGHD), Turner Syndrome (TS) and Small for Gestational Age (SGA) using iGRO* in a Pediatric Endocrine Practice

Partsch Carl-Joachim , Jakisch Bele , Ostendorf Anne , Stahnke Nikolaus , Wusthof Achim

Background: Quality management of GH treatment in children is important to ensure optimal treatment outcome and to save resources in the health care system. iGRO is a new internet based Medical Device to compare treatment results with predicted results according to published prediction models.Objective and hypotheses: Growth data were analyzed by iGRO for 1st and 4th year prediction in comparison to treatment results. All eligible patients of our practic...

hrp0084p2-547 | Puberty | ESPE2015

The Impact of Growth Hormone (GH) Therapy Combined with Estrogens on Blood Pressure (BP), Cardiac Left Ventricular (LV) Dimensions and Lipid Metabolism in Pubertal Girls with Turner’s Syndrome (TS)

Nagaeva Elena , Shiryaeva Tatiana , Volevodz Natalia , Chikulaeva Olga , Pankratova Maria , Gavrilova Anna , Peterkova Valentina

Background: The risk of hypertension is estimated to occur in 7–17% of children and adolescents with TS. Even girls with TS who are normotensive have been shown an abnormal circadian BP rhythm, increasing the risk of end-organ hypertensive damage.Objective and hypotheses: We performed this study to assess the effects of GH treatment combined with estrogens for short stature on LV dimensions, systemic BP and lipid metabolism in girls with TS without ...

hrp0084p3-972 | GH & IGF | ESPE2015

Time Trends in Baseline Characteristics (2006–2014) in Short Children with Growth Hormone Deficiency (GHD), Born Small for Gestational Age (SGA) and with Ullrich-Turner Syndrome (TS) Enrolled in Nordinet® International Outcomes Study (IOS) in Germany and Czech Republic

Dorr Helmuth Gunther , Bramswig Jurgen , Meckes-Ferber Stefanie , Pournara Effie , Pedersen Birgitte Tonnes , Snajderova Marta

Background: Early diagnosis of growth disorders and initiation of GH therapy at a younger age improves clinical outcomes.Aims and objectives: To analyse time trends in baseline parameters at GH treatment start (2006 – 2014) in short children with GHD, SGA and TS from Germany and Czech Republic enrolled in NordiNet® IOS (NCT00960128).Method: Baseline data (chronological age, height, weight, BMI, GH dose) from pa...

hrp0092p3-325 | Late Breaking Abstracts | ESPE2019

45X/47XXX Mosaicism and Progressive Puberty

liu ziqin , chen xiaobo

Objective: To report a case girl with 45X/47XXX mosaic Turner syndrome exhibiting a progressive puberty.Design: Case reportResult: A 9 years 4 months old girl was presented with growth retardation. Chromosome analysis revealed a mosaic karyotype 45X/47XXX. She presented with normal height but much lower than the mid-parental height. During annual check-ups, her growth rate was acce...

hrp0089wg1.3 | ESPE Disorders of Sex Development & Turner Syndrome Joint Session | ESPE2018

Cardiovascular Pathology in Males and Females with 45,X/46,XY Mosaicism

De Groote Katya

The phenotype of 45,X/46,XY mosaicism is heterogeneous ranging from females with Turner syndrome (TS) to apparently normal males. Cardinal features of TS include reduced final height and infertility. Apart from endocrine, renal and neurocognitive disorders, structural heart defects are frequently present (in 25% to 50%), also in TS patients with mosaicism 45,X/46,XY. Males with 45,X/46,XY frequently show stigmata typically associated with TS but data on cardiovascular patholog...

hrp0082wg6.1 | Turner | ESPE2014

Liver Involvement in Tuner Syndrome

Roulot-Marullo Dominique

Liver test abnormalities are frequent in adult patients with Turner syndrome, corresponding to various pathophysiological mechanisms. Steatosis, steatofibrosis and steatohepatitis are the most frequently reported lesions, caused by metabolic disorders, which are commonly related to overweight. Marked architectural changes, including nodular regenerative hyperplasia, multiple focal nodular hyperplasia and cirrhosis, found in some patients, are associated with a risk of severe l...