hrp0095p1-449 | Diabetes and Insulin | ESPE2022

Do-It-Yourself Artificial Pancreas Systems in a PediatricPopulation with Type 1 Diabetes in a Real-life Setting: The AWeSoMe Study Group Experience

Nir Judith , Rachmiel Marianna , Fraser Abigail , Lebenthal Yael , Brener Avivit , Pinhas-Hamiel Orit , Haim Alon , Stern Eve , Levek Noa , Ben-Ari Tal , Landau Zohar

Aims: Do-It-Yourself Artificial Pancreas Systems (DIYAPS) represent a unique patient-initiated treatment in which commercially available and approved medical devices such as continuous glucose monitoring systems (CGMs) and insulin pumps are connected by an off-label algorithm, and are remotely controlled by open-source algorithms to automate insulin delivery. While these systems are co-created by the DIYAPS community, and access is open to everyone, users have...

hrp0095p1-368 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2022

Gender dimorphism in transgender youth – hormonal therapy and the balance between muscle, adipose tissue and cardiometabolic alterations

Borger Ophir , Oren Asaf , Perl Liat , Yackobovitch-Gavan Michal , Sheppes Tamar , Brener Avivit , Elkon-Tamir Erella , Israeli Galit , Segev-Becker Anat , Lebenthal Ophir Yael

Context: Given the importance of sex hormones in metabolic regulation, dynamics in body composition and cardiometabolic alterations may occur in transgender persons receiving gender-affirming hormone (GAH) therapy.Objectives: Our aim was to explore the association between muscle-to-fat ratio (MFR) and the risk for metabolic syndrome components in transgender youth.Methods: In 71 tr...

hrp0097rfc1.1 | Adrenals and HPA Axis | ESPE2023

Unfavorable Body Composition in Children with Premature Adrenarche: Implications for Metabolic Health

Ben Simon Asaf , Uretzky Adi , Yackobovitch-Gavan Michal , Segev-Becker Anat , Midlij Eyas , Borger Ophir , Laurian Irina , Dorfman Anna , Brener Avivit , Lebenthal Yael

Context: Premature adrenarche (PA) could pose an increased risk of obesity and metabolic derangements in adolescence and early adulthood. However, little is known about the body composition of children with PA and its association to the development of adverse metabolic outcomes.Aim: To describe the body composition of children with PA and explore the interaction between PA and metabolic syndrome components.<p class="...

hrp0097p1-238 | Diabetes and Insulin | ESPE2023

MiniMed 780G Advanced Hybrid Closed Loop System Outcomes According to Pubertal Status - Awesome Study Group Real-Life Experience

Rachmiel Marianna , Lebenthal Yael , Kineret Mazor-Aronovitch Kineret , Brener Avivit , Levek Noah , Polishuk-Yakobi Talia , Tal Ben Tal , Abiri Shirli , Landau Zohar , Pinhas-Hamiel Orit

Background and aims: Achieving good glycemic control is a major challenge for adolescents with type 1 diabetes (TID). The introduction of the MiniMed 780G system, an advanced hybrid closed-loop (AHCL), that enables an automatic correction of insulin, gave hope for improved glycemic outcomes in adolescents. We assessed specific characteristics associated with glycemic measures in youth with T1D switching to Minimed 780G.Methods:</...

hrp0097p1-259 | Fat, Metabolism and Obesity | ESPE2023

Exploring the Relationship between Muscle-to-Fat Ratio and Surrogate Markers of Nonalcoholic Fatty Liver Disease in Children with Overweight and Obesity

Uretzky Adi , Zaitoon Hussein , Ben Simon Asaf , Cohen-Sela Eyal , Yackobovitch-Gavan Michal , Midlij Eyas , Borger Ophir , Interator Hagar , Amir Achiya , Moran-Lev Hadar , Brener Avivit , Lebenthal Yael

Background: We previously reported on the strong predictive value of the muscle-to-fat ratio (MFR) z-score in the development of early-onset metabolic syndrome components in children with overweight/obesity. Data on the role of the balance between muscle and fat in the development of childhood onset nonalcoholic fatty liver disease (NAFLD) are sparse. In the current study we explored the interplay between MFR and surrogate markers of NAFLD in children with ove...

hrp0095rfc4.2 | Fat, Metabolism and Obesity | ESPE2022

Effect of Setmelanotide Treatment in Children and Adolescents With Proopiomelanocortin (POMC) Deficiency, Leptin Receptor (LEPR) Deficiency, and Bardet-Biedl Syndrome (BBS)

Argente Jesús , Kühnen Peter , M. Haqq Andrea , Wabitsch Martin , K. Chung Wendy , van den Akker Erica , Á. Martos-Moreno Gabriel , Mohamed Iqbal Anoop , Forsythe Elizabeth , Dubern Béatrice , Malhotra Sonali , Yuan Goujun , Touchot Nicolas , Dollfus Hélène , Farooqi Sadaf , Clément Karine

Background: The melanocortin-4 receptor (MC4R) pathway is a key regulator of energy balance and satiety. Variants in genes upstream of MC4R encoding leptin receptor (LEPR), proopiomelanocortin (POMC), proprotein convertase subtilisin/kexin type 1(PCSK1) and those involved in Bardet-Biedl syndrome (BBS) can impair MC4R pathway signaling. Clinically, these variants are characterized by hyperphagia (Pathologic insatiable hunger) and early-onset, severe obesity. E...

hrp0095p2-3 | Adrenals and HPA Axis | ESPE2022

A Rare Cause of Peripheral Precocious Puberty

Çakır Gündoğan Seçil , Kılınç Uğurlu Aylin , Özdemir Gökçe Ayşe , Özyörük Derya , Yardımcı Gönül , Kasap Yusuf , Karakuş Esra , Sarı Neriman , Seçil Ekşioğlu Ayşe , Ölçücüoğlu Erkan , Boyraz Mehmet

Introduction: Peripheral precocious puberty is a non-gonadotropin-dependent early pubertal development. Exogenous intake of sex steroids, gonadal or adrenal pathologies, ectopic hCG-secreting lesions play a role in the etiology. Adrenal tumors is a rare cause of heterosexual precocious puberty.Case: An eight-year-old female patient was admitted with the complaints of genital hair growth and breast enlargement. The patien...

hrp0092rfc3.1 | Multi-system Endocrine Disorders | ESPE2019

European Registries For Rare Endocrine Conditions (EuRRECa): Results From The Pilot Phase Of The Platform For e-Reporting Of Rare Endocrine Conditions (e-REC)

Ali Salma , Bryce Jillian , Muir Tom , Okure Akanimo , Cools Martine , Danne Thomas , Dattani Mehul , Dekkers Olaf , Hiort Olaf , Linglart Agnès , Netchine Irène , Nordenström Anna , Patócs Attila , Pereira Alberto , Persani Luca , Reisch Nicole , Smyth Arlene , Šumnik Zdeněk , Taruscio Domenica , Visser W. Edward , Ahmed S.Faisal

Background: EuRRECa (European Registries for Rare Endocrine Conditions) is a new project incorporating the development of a core endocrine registry and the development of an e-reporting programme for rare endocrine conditions (e-REC) that are covered within Endo-ERN (https://eurreca.net/e-rec/).Methods: 24 Endo-ERN centres within 12 countries participated in a pilot phase of e-REC...

hrp0092rfc6.4 | Bone, Growth Plate and Mineral Metabolism Session 2 | ESPE2019

Targeted Molecular Genetic Diagnosis by Next Generation Sequence Analysis Method and Investigation of Responsible Candidate Genes in Patients with Osteogenesis Imperfecta

Özen Samim , Gökşen Damla , Işik Esra , Gürkan Ferda Evin , Onay Hüseyin , Akgün Bilçag , Ata Aysun , Atik Tahir , Özkinay Ferda , Darcan Şükran , Çogulu Özgür

Introduction: The aim of this study was to investigate the molecular genetic etiology and to determine the relationship between genotype and phenotype with targeted next-generation sequence (NGS)analysis.Method: Patients with a clinical diagnosis of OI were included in the study. Initially, mutations in COL1A1 and COL1A2 genes which are known to be most responsible for OI were investigated. In the second step, a targeted...

hrp0092p2-69 | Diabetes and Insulin | ESPE2019

Gluten-free Diet in Children with Recent Onset Type 1 Diabetes is Associated with Slower Pace of C-peptide Decline, Better Metabolic Control and Lower Insulin Requirement at 12 months

Neuman Vít , Pruhová Štepánka , Kulich Michal , Funda David P. , Koloušková Stanislava , Vosáhlo Jan , Romanová Martina , Petruželková Lenka , Obermannová Barbora , Cinek Ondrej , Šumník Zdenek

Objectives: Data on the role of gluten in type 1 diabetes (T1D) pathogenesis are scarce. We aimed to test whether gluten-free diet (GFD) can decelerate the decline in beta-cell capacity in newly diagnosed non-coeliac T1D children.Methods: Forty six children (aged 10.2±3.3 years) were recruited into this non-randomized self-selected intervention trial: 26 started with GFD, whereas 20 remained on standard diet. Main o...