hrp0089p2-p218 | GH & IGFs P2 | ESPE2018

The Rationale and Design of TransCon GH

Sprogoe Kennett , Beckert Michael , Mortensen Eva , Karpf David B , Leff Jonathan A

Background: The fundamental challenge of developing a long-acting growth hormone (LAGH) is to create a more convenient growth hormone (GH) dosing profile while retaining the excellent safety, efficacy, and tolerability of daily GH. With GH receptors on virtually all cells, replacement therapy should achieve the same tissue distribution and effects of daily (and endogenous) GH while maintaining levels of GH and resulting IGF1 within the physiologic range....

hrp0084p3-1157 | Puberty | ESPE2015

Assessment of Endocrine Function in Egyptian Adolescent B-Thalassemia Major Patients

Dayem Soha Abd El , Kader Mona Abd El

Objective: To evaluate the endocrine function among Egyptian adolescent thalassemic patients.Patients and methods: 54 patients and 28 age-matched normal controls was included. Anthropometric measurements, clinical pubertal assessment were done for all patients. Oral glucose tolerance test (OGTT) was done to all patients and controls with measuring serum insulin level at 0.120 minutes. Insulin sensitivity and release index were calculated. 32 patients had...

hrp0084p3-1179 | Thyroid | ESPE2015

Plasma Visfatin Level and Its Association with Apolipoproteins A1 and B in Hypothyroid Children

Dehkordi Elham Hashemi , Rostampour Noushin , Kasiri Karamali , Moafi Mohammad , Aramesh Mohammad Esmail , Hashemipour Mahin

Background and aim: Hypothyroidism could be accountable for cardiovascular diseases; hence, necessity of novel biomarkers being capable to predict patient’s status is indispensable. The aim of this study was to appraise alteration of plasma visfatin levels (as a newly discovered proteins) and its association with lipid profiles of hypothyroid patients.Materials and methods: In this cross-sectional, descriptive, and analytical study, 30 children bein...

hrp0089p3-p141 | Fat, Metabolism and Obesity P3 | ESPE2018

Serum Hepcidin and Ferritin in Prepubertal Obese Children

Gajewska Joanna , Klemarczyk Witold , Ambroszkiewicz Jadwiga , Gła¸b-Jabłońska Ewa , Chełchowska Magdalena

Introduction: Obesity is the direct cause of a number of immediate problems during childhood. Recently, fat mass was described as a significant negative predictor of serum iron. Hepcidin is a hormone stimulated by an increase in plasma iron levels and iron deposits in tissues, and decreases iron release from macrophages and duodenal enterocytes into the plasma. This protein prevents excessive iron absorption and iron accumulation in tissues. Ferritin is the most commonly deplo...

hrp0084p2-330 | Fat | ESPE2015

Abdominal fat Distribution Measured by Magnetic Resonance Imaging in 197 Children Aged 10–15 Years – Correlation to Anthropometry and Dual X-Ray Absorptiometry

Tinggaard Jeanette , Hagen Casper P , Mouritsen Annette , Mieritz Mikkel G , Wohlfahrt-Veje Christine , Fallentin Eva , Larsen Rasmus , Christensen Anders N , Jensen Rikke B , Juul Anders , Main Katharina

Background: Obesity in childhood is defined by age- and sex-specific BMI cut-off values. However, BMI does not disclose the distribution of fat mass. Increased abdominal adipose tissue is associated with a higher risk of cardio-metabolic disease in adulthood. Thus, precise measurements of abdominal adipose tissue in children may enable early prevention of disease.Objective and hypotheses: To validate measurements of abdominal adipose tissue by anthropome...

hrp0092p1-139 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology | ESPE2019

Gonadal Function of Female Patients with Noonan Syndrome

Moniez Sophie , Capri Yline , Pienkowski Catherine , Lepage Benoit , Hamdi Safouane , Cartault Audrey , Oliver Isabelle , Jouret Béatrice , Diene Gwenaelle , Salles Jean-Pierre , Cavé Hélène , Verloes Alain , Tauber Maithé , Yart Armelle , Edouard Thomas

Background: Abnormalities in the hypothalamo-pituitary-gonadal axis have been reported in Noonan syndrome (NS) males but few data are available in female patients. Objective: The aim of this retrospective study was to evaluate the gonadal function of female patients with NS and to look for genotype-phenotype correlations. Patients and Methods: The study population included 19 girls/adolescents with a genetically confirmed diagnosis of NS and with available cli...

hrp0084fc5.3 | Endocrine Oncology/Turner | ESPE2015

Screening in Children with Succinate Dehydrogenase B (SDHB) Mutations: a Single Centre’s Family Clinic Experience

Shapiro Lucy , Srirangalingam Umasuthan , McAndrew Lorraine , Martin Lee , Tufton Nicola , Kumar Ajith , Drake William , Akker Scott , Storr Helen

Background: Germline mutations in SDHB gene are associated with the familial paraganglioma (PGL) syndrome that carries the highest malignant potential. Although penetrance is lower than initially described, lack of effective treatments for metastatic PGLs makes screening essential for early tumour detection, surgical removal and improved outcome. However, no consensus exists in relation to timing and mode of screening.Objective: To assess publis...

hrp0082fc9.5 | Beta cells | ESPE2014

Transient Neonatal Diabetes in Adulthood: Metabolic and Neurodevelopmental Outcomes

Le Bourgeois Fleur , Busiah Kanetee , Abu-Amara-Olivieri Sawsan , Bachere Nadege , Bertrand Anne-Marie , Bourron Olivier , David Paul , De Boisvilliers Fabienne , Deumier Bernard , deVries Liat , Jelliman Stephanie , Le Tallec Claire , Martin-Dessila Amelie , Nimri Paul , Paoli Anne , Perrin Mireille , Stuckens Chantal , Ythier Hubert , Pouvreaux Nathalie , Bellane-Chantelot Christine

Background: Transient neonatal diabetes mellitus (TNDM) is a rare genetic β-cell dysfunction leading to hyperglycaemia that resolves in early childhood. About 80% of patients relapse during adolescence or adulthood. Some of these patients suffer from neurodevelopmental defect. Long-term outcome has been poorly investigated.Objective and Hypotheses: To investigate metabolic and neurologic outcomes in adults affected with TNDM.M...

hrp0092p3-300 | Late Breaking Abstracts | ESPE2019

Height and Upper/Lower Body Ratio in Turner Syndrome Adolescents in Indonesia; Is There any Significant Difference Based on Karyotype?

Novina Novina , Gunardi Hartono , Pulungan Aman B.

2Department of Pediatrics, Faculty of Medicine, University of Indonesia, Jakarta, IndonesiaBackground: Short stature is one of the most common findings in Turner syndrome. There are two types of Turner syndrome based on karyotype: classical and mosaic. It is often marked by the body disproportion and dysmorphic profile of the patients. There are still not many data available regarding upper lower body segment ratio (U/L body s...

hrp0095fc5.1 | Adrenals and HPA Axis | ESPE2022

Transcriptome profiling of adrenocortical tumors from children with unfavorable disease presentation.

Bueno Ana C , da Silva Jr Rui M P , Stecchini Mônica F , Cardinalli Izilda A , Junqueira Thais , A Scrideli Carlos , AF Molina Carlos , Tucci Silvio , Coeli-Lacchini Fernanda B , Moreira Ayrton C , Ramalho Leandra NZ , Brandalise Silvia R , Yunes José A , Vêncio Ricardo ZN , de Castro Margaret , Antonini Sonir R

Children diagnosed with adrenocortical tumors (ACT) have variable prognosis depending on disease presentation at diagnosis. Available therapeutic options render excellent outcome for a subset of patients, but limited improvement in the survival of those diagnosed with non-localized/advanced disease. ACT DNA methylation was recently demonstrated to be a robust and independent prognostic biomarker. Aiming to identify potential therapeutic targets for these children, we compared ...