hrp0092p1-167 | Adrenals and HPA Axis (1) | ESPE2019

Development of an International Benchmark for Sick Day Episodes as a Core Clinical Outcome in People with Congenital Adrenal Hyperplasia

Ali Salma , Daniel Eleni , Bryce Jillian , Ikiroma Adalia , Lewsey James , Ross Richard , Krone Ruth , Acerini Carlo , Krone Nils , Das Urmi , Tomlinson Jeremy , Korbonits Marta , Higham Claire , Darendeliler Feyza , Guran Tulay , Guven Ayla , Attapatu Navoda , Milenkovic Tatjana , Raducanu-Lichiardopol Corina , Hannema Sabine , Claahsen Hedi , Finken Martijn , Baronio Federico , Balsamo Antonio , Einaudi Silvia , de Vries Liat , Luczay Andrea , Neumann Uta , Blankenstein Oliver , Mohnike Klaus , Bonfig Walter , Elsedfy Heba , Birkebaek Niels , Iotova Violeta , Bachega Tania , Mendonca Berenice , Cools Martine , Costa Eduardo Correa , Filho Guilherme Guaragna- , Rey Rodolfo , Ahmed S. Faisal

Background: Congenital adrenal hyperplasia (CAH) is a rare condition characterised by adrenal insufficiency and a life-long risk of adrenal crises. There is a paucity of information on the epidemiology of acute adverse events in this population.Objective: To investigate the frequency, aetiology and consequences of acute adverse events attributed to adrenal insufficiency in CAH.Methods</stro...

hrp0094p1-43 | Sex Endocrinology and Gonads A | ESPE2021

Broad range of phenotypes in an international cohort of 75 DSD individuals with SF-1/NR5A1 variants

Kouri Chrysanthi , Sommer Grit , Ahmed Faisal , Balsamo Antonio , Baronio Federico , Bryce Jillian , Camats Nuria , Cetinkaya Semra , van der Grinten Hedi L Claahsen , Cools Martine , Darendeliler Fatma Feyza , Davies Justin H , Fabbri-Scallet Helena , Globa Evgenia , Guerra-Junior Gil , Guran Tulay , Hannema Sabine , Hiort Olaf , Janner Marco , Kalinchenko Natalia , Lachlan Katherine , Kolesinska Zofia , l’Allemand Dagmar , Lang-Muritano Mariarosaria , Lucas-Herald Angela , Martin Idoia Martinez de Lapiscina , Mazen Inas , Moenig Isabel , Muhrer Julia , Niedziela Marek , Nordenstrom Anna , Orman Burce , Poyrazoglu Sukran , Tack Lloyd , Tadokoro-Cuccaro Rieko , Wasniewska Malgorzata , Yavas Zehra , Zelinska Nataliya , Fluck Christa E ,

Background: Steroidogenic Factor 1 (NR5A1/SF-1) is essential for the development and function of human sex and steroid organs. Variants of SF-1 lead to a broad spectrum of phenotypes including adrenal insufficiency and differences of sex development (DSD), but data on the whole picture of phenotypes in individuals with SF-1 variants are currently lacking. We aim to investigate the phenotype of individuals with SF-1 variants in a large interna...

hrp0084p3-896 | Fat | ESPE2015

Prevalence of Excess Weight in Adolescents at Primary Health Care Units in South Brazil

Vargas Deisi Maria , de Medeiros Ana Carolina Santin , Klieman Elis , Eberhardt Isabel , Piesanti Vera Janete , Pasa Simone , da Silva Claudia Regina Lima Duarte , Coutinho Luciane Azevedo , Simao Vilma Margarete

Background: In the past decades Brazil has experienced a nutritional transition process characterised by a significant reduction in malnutrition and progressive increase in overweight and obesity. Nutritional education and precocious interventions are useful strategies to combat excess weight in childhood and adolescence. According to the World Health Organization (WHO), there were more than 40 million children overweight in the world in 2011. In Brazil, the prevalence of exce...

hrp0095p1-362 | Pituitary, Neuroendocrinology and Puberty | ESPE2022

Negative impact of brain tumors on the neurocognitive functions of childhood cancer survivors

Mavrea Kalliopi , Katsibardi Aikaterini , Roka Kleoniki , Pons Roser , Antoniou Alexandros-Stamatios , Kanaka-Gantenbein Christina , Kattamis Antonis , Bacopoulou Flora

Introduction – Scope: Childhood cancer has an impact on the neurocognitive functions of growing children and adolescents. The aim of this study, which is the first of its kind in Greece, was to examine the differences in neurocognitive abilities between children and adolescents survivors of brain tumors (BT), and survivors of other types of cancer.Method: Study participants were cancer survivors aged 7 to 15 years,...

hrp0095p1-482 | Fat, Metabolism and Obesity | ESPE2022

Liraglutide SC in the treatment of severe obesity in Pediatrics: a missed therapeutic opportunity?

Diez-Lopez Ignacio , Sarasua-Miranda Ainhoa , Jose Espina-Diez Mari

Liraglutide 3.0 mg has been shown to improve body mass index (BMI) and weight in obese adolescents. And it has done so in a randomized, double-blind, phase 3 clinical trial that has investigated the effect of liraglutide 3mg. compared with placebo for weight control in 251 obese minors, and as a complement to a healthy lifestyle. Adverse events associated with the use of liraglutide 3.0 in pediatric patients are not very different from those observed in adults. There may be ca...

hrp0086p2-p306 | Diabetes P2 | ESPE2016

Maturity-Onset Diabetes of the Young (MODY): Tracking and Clinical Follow-up

Bezerra Arthur Pires , Ramos Alberto Jose Santos , Braz Adriana Farrant

Background: Maturity-onset diabetes of the young (MODY) is a monogenic form of diabetes, with an autosomal dominant mode of inheritance and high penetrance. To this date, it is known 13 subtypes of MODY with different genetic etiologies. It is characterized by high incidence in the family, an early onset and primary defect in pancreatic β-cell function.Objective and hypotheses: The primary objective of this study is to identify patients with MODY an...

hrp0084p2-208 | Bone | ESPE2015

Bone Mineral Density in Children and Adolescents with Vertical HIV Infection

Vargas Deisi Maria , Prust Daniela Oliveira , Galvao Jose Carlos

Background: Chronic diseases are the main causes of bone mass reduction in childhood and adolescence. Different aspects related to the process of bone acquisition and maintenance may be affected. Studies had point out the presence of bone mass reduction in children and adolescents with HIV infection with association to antiretroviral use, chronological age (CA), weight and serum CD4 T-cell counts. However, others do not.Objective: To evaluate bone minera...

hrp0086p2-p518 | Fat Metabolism and Obesity P2 | ESPE2016

Vitamin D Deficiency in Obese Children and the Relationship with Insulin Resistance and Metabolic Syndrome

Fernandez Viseras Irene , Angeles Santos Mata Maria , Lechuga Sancho Alfonso , Pedro Novalbos Ruiz Jose , Jose Macias Lopez Francisco

Background: Vitamin D is a pleiotropic hormone the deficiency of which is related with extraskeletal manifestations such as insulin resistance and cardiovascular risk disease.Objective and hypotheses: To investigate the levels of VitaminD in a sample of children with obesity and to evaluate the relationship between carbohydrate metabolism and metabolic syndrome (MS).Method: In this prospective cross-sectional study, 189 children ag...

hrp0084p3-773 | Diabetes | ESPE2015

Mauriac Syndrome, a Rare Complication of Type 1 Diabetes Mellitus

Rivero-Martin Maria Jose , Perez-Segura M Pilar , Alcazar-Villar Maria Jose , Montes-Bentura David , Milian M Eugenia Oros

Background: Mauriac syndrome (MS) classically involves hepatomegaly (hepatic glycogenosis), growth impairment and cushingoid features in a patient with poorly controlled type 1 diabetes mellitus (T1DM). The typical age of presentation is adolescence. With the advent of improved insulin regimens, MS has seen less frequently. However, new cases appear each year in medical literature.Clinical case: We report the case of a 9 years and 9 months of age male, w...

hrp0084p3-964 | GH &amp; IGF | ESPE2015

Evaluation of the Facility of Use of a New GH Administration Device – Study DAGH2014

Rivero-Martin Maria Jose , Ontanon-Nasarre Ana , Alcazar-Villar Maria Jose , Carrasco-Torrents America , Andres-Rosado Ana , Montes-Bentura David

Background: One of the limiting factors in adherence to GH therapy, is satisfaction with the administration device used.Objective and hypotheses: With the emergence of a new biosmiliar GH (BGH) administration device, we will assess the simplicity, ease of use, management and pain perceived by the patients.Method: Prospective through survey caregivers and children treated with BGH at least for 6 months. Signed informed consent was r...