hrp0089p2-p144 | Fat, Metabolism and Obesity P2 | ESPE2018

Efficacy, Safety and Tolerability of Liraglutide, GLP-1 Analogue, in Indian Adolescent Population with Obesity

Sethi Aashish , Ramachandran Smita , Kochar Inderpal

Introduction: Liraglutide is a long-acting glucagon-like peptide-1 receptor agonist which directly stimulate POMC neurons and inhibit neuropeptide-Y and Agouti-related peptide neurons of the arcuate nucleus resulting in appetite suppression. It has been approved in adult population for obesity.Aim: To assess the Efficacy and Safety of Liraglutide in Indian Adolescent obese population.Materia...

hrp0089p3-p328 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology P3 | ESPE2018

A 45X0/46XY Girl Diagnosed with Prepubertal FSH Elevation

Haliloglu Belma

Aim: The 45,X/46,XY karyotype is rare with an estimated incidence rate of less than 1/15.000 live births. It represents from Turner females to phenotypically normal males with varying degrees of genital ambiguity. Although, high gonadotropin levels have been described in 0–5 years old girls with Turner syndrome, high FSH level is not well known finding in prepubertal girls older than 6 years.Case: A 6 y 8 m girl presented with lipomastia. She was bo...

hrp0089p3-p354 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology P3 | ESPE2018

Emblematic Case CAH

Chahin Silvia , Mejia Federico

Introduction: The National Registry of Civil Status through circular 33 of February 24, 2015 instructed the guidelines for the allocation of a sex through an inscription on the Civil Registry of Birth for intersexual minors.Objective: To create clinical and medical awareness on the importance of making an accurate diagnosis of Congenital Adrenal Hyperplasia CAH in order to avoid adverse effects due the omission of the due diligence by violating of the pr...

hrp0086wg5.2 | ESPE Diabetes Technology and Therapeutics Working Group | ESPE2016

SWEET: Developing Centers of Excellence (CoR)

Danne Thomas

Background: SWEET (www.sweet-project.eu) is a non-profit entity endorsed by ISPAD aiming to create an extensive network of certified CoRs for childhood diabetes in order to ensure high quality care.Objective and hypotheses: SWEET aims at an improved and more uniform care for people with diabetes through comparing processes and outcomes among participating members. The results of data analysis are conveyed to memb...

hrp0086fc10.4 | Perinatal Endocrinology | ESPE2016

In Utero and Postnatal Consequences of Psychological Maternal Stress have Different Effects On Longevity: Studies in World War 1 Orphans

Todd Nicolas , Valleron Alain-Jacques , Bougneres Pierre

Background: Early life stress (ELS) are known to have a deleterious impact on health, but their potential long-term consequences on mortality remain to be assessed. World War 1 (WW1) has caused major stress and bereavement to millions of European mothers.Objectives and hypotheses: To compare the effects of a maternal stress occurring during intra uterine or in postnatal life upon long-term mortality.Patients: Thanks to recently dig...

hrp0086rfc7.3 | Gonads & DSD | ESPE2016

Harmonisation of Serum Dihydrotestosterone Analysis: Establishment of an External Quality Assurance Program

Wudy Stefan , Hartmann Michaela , Jolly Lisa , Shun Ho Chung , Kam Richard , Joseph John , Boyder Conchita , Greaves Ronda

Background: Serum dihydrotestosterone (DHT) is an important analyte for the clinical assessment of disorders of sex development. It is also reportedly a difficult analyte to measure. Currently there are significant gaps in the standardisation of this analyte, including no external quality assurance (EQA) program available worldwide to allow for peer performance review of DHT.Objective and hypotheses: We therefore proposed to establish an EQA program for ...

hrp0086p1-p39 | Adrenal P1 | ESPE2016

Chronic Adrenal Insufficiency Due to a Mutation of Nicotinamide Nucleotide Transhydrogenase 1 (NNT1): Case Report

Lefevre Christine , Derquenne Helene , Ister Marielle , Weill Jacques , Gueorguieva Iva , Stuckens Chantal , Cartigny Maryse

Background: Congenital adrenal insufficiency represents a life-threatening condition. Among its multiples causes, mutation of NNT1 is the most recently discovered. We had the opportunity to observe one case. NNT1 is a gene coding for a membrane protein which protects cells from oxidative stress.Objective and hypotheses: As few cases have been published until now, we describe a new case.Results: In a consanguineous (second grade) Al...

hrp0086p1-p232 | Diabetes P1 | ESPE2016

Extrahepatic Biliary Atresia in Combination with Toxic Cholestasis Due to Glibenclamide in a Case of Neonatal Diabetes

Kapellen Thomas , Flemming Gunter , Bartelt Heike , Wachowiak Robin , Kiess Wieland

Background: More than 20 gene loci are known to cause monogenic neonatal diabetes today. A definite mutation can be found in 65–70% of all cases. Mutations in the ATP sensitive potassium channel can frequently be treated by sulfonylurea. Glibenclamide is on of the drugs known to inhibit the bile salt export pump (BSEP). However most drug induced cholestasis cases are reported in adults.Objective and hypotheses: Glibenclamide is used frequently to tr...

hrp0086p2-p270 | Diabetes P2 | ESPE2016

Seasonality of Type 1 Diabetes in Children and Adolescents According to Date of Diagnosis and Date of Birth in a Large Diabetes Centre

Vasilakis Ioannis Anargyros , Kosteria Ioanna , Tzifi Flora , Christopoulos-Timogiannakis Evangelos , Kotrogianni Paraskevi , Chrousos George , Kanaka-Gantenbein Christina

Background: There are studies supporting the seasonality of diabetes according to date of diagnosis and date of birth, with most T1D children being diagnosed in fall and winter and most of them been born in the same period. That is in concordance with previously published data from 1978 to 2008 in our country.Objective and hypotheses: To assess whether there is a seasonal variation of diabetes onset according to date of diagnosis and date of birth of inc...

hrp0086p1-p375 | Gonads & DSD P1 | ESPE2016

Can we Standardize Sex Assignment in 45,X/46,XY Mixed Gonadal Dysgenesis?

Colindres Johanna Viau , Eugster Erica , Smith O'Brian , Gunn Sheila , Mendiratta Meenal , Karaviti Lefkothea

Background: Patients with 45,X/46,XY mixed gonadal dysgenesis (MGD) have variable phenotypes leading to both male and female sex assignments. A multidisciplinary team should guide the process of sex assignment, however a standardized approach may provide objective guidance for the team as well as a tool for settings without such resources. One of the most important considerations for sex assignment is the hormonal production, which often determines the degree of virilization. ...