hrp0097p1-311 | Growth and Syndromes | ESPE2023

Management of rhGH treatment in children with CKD in current clinical practice: a multicentric study

Labey Séverine , Hogan Julien , Salomon Rémi , Ulinski Tim , Boizeau Priscilla , Carel Jean-Claude , Simon Dominique

Introduction: Growth retardation < –2 SDS is frequently observed in children with chronic kidney disease (CKD) and is a marker of severity of CKD. Recombinant human growth hormone (rhGH) treatment has been approved since 1995 in CKD patients. The aim of this study was to describe the growth outcomes and treatment patterns in children with congenital CKD.Methods: Patients were recruited from transplantation recor...

hrp0095p1-4 | Adrenals and HPA Axis | ESPE2022

Glucocorticoid-induced adrenal insufficiency: morning plasma cortisol values safely assess recovery of the HPA axis in pediatrics

Laulhé Margaux , Dumaine Cécile , Chevenne Didier , Leye Fallou , Faye Albert , Dozières Blandine , Strullu Marion , Viala Jérôme , Hogan Julien , Houdouin Véronique , Léger Juliane , Simon Dominique , Claude Carel Jean , Storey Caroline , Guilmin-Crépon Sophie , Martinerie Laetitia

Background and Objectives: Chronic administration of synthetic glucocorticoids affects 1% of the world population and is responsible for secondary adrenal insufficiency for 40% of the patients. In pediatrics, glucocorticoid-induced adrenal insufficiency (GI-AI) diagnosis is based on the Low Dose Synacthen Test (LD-SST). Screening for glucocorticoid-induced adrenal insufficiency implies medical and economical costs due to the necessity of an intravenous line an...

hrp0097fc10.2 | Fetal, neonatal endocrinology and metabolism (to include hypoglycaemia) &amp; Multisystem endocrine disorders | ESPE2023

Immune profile response to rituximab in ROHHAD syndrome

Hawton Katherine , Hamilton-Shield Julian , Hogan Andrew , Giri Dinesh

Background: Rapid-onset obesity with hypoventilation, hypothalamic dysfunction, autonomic dysregulation (ROHHAD) is a rare syndrome associated with high morbidity and mortality presenting with rapid onset of obesity in early childhood. An immune-inflammatory aetiology has been postulated; however, the immune profile is not well described.Case report: We report the case of a five-year-old female who presented in respirato...

hrp0097p1-231 | Diabetes and Insulin | ESPE2023

Children with Type 1 Diabetes and Obesity show biochemical changes associated with insulin resistance

Hayes Eadaoin , Cody Declan , E Hogan Andrew

Children with Type 1 diabetes are experiencing higher levels of overweight and obesity as a consequence of intensive insulin therapy as well as background socioeconomic and environmental factors that are contributing to excess adiposity in the general population. The consequences of obesity in people with type 1 diabetes are of particular concern, as obesity in adults increases the risk of both diabetes-related and obesity-related complications, including cardiovascular diseas...

hrp0089p1-p087 | Diabetes &amp; Insulin P1 | ESPE2018

Investigation into β-cell Adaptation During Puberty

Castell Anne-Laure , Ethier Melanie , Fergusson Grace , Ghislain Julien , Poitout Vincent

Background: Puberty is a time of hormonal changes that are associated with insulin resistance. Although insulin sensitivity is restored at the end of puberty in healthy youth, it does not resolve in obese adolescents leading to an increase of cardio metabolic disease such as type 2 diabetes. In response to an increase in insulin demand, as during pregnancy or obesity-induced insulin resistance, β-cells increase their functional mass to maintain glucose homeostasis. Howeve...

hrp0097p2-246 | Late Breaking | ESPE2023

Results from a Multi-Stakeholder Meeting on Medical Devices in Paediatric Type I Diabetes

Biester Torben , Reschke Felix , Danne Thomas , Julien Marc , Lusar Irja , Cheng Katharine , Cavaller-Bellaubi Maria , Katz Michelle , Niemoeller Elisabeth , Renard Eric , Sturny Maren , Geertsma Robert , Vassal Gilles

Objectives: To discuss all challenges involved with providing children (including the very young) and adolescents with diabetes (CwD) with the latest appropriate technology, such as automated insulin delivery systems (AIDs), to manage their blood glucose and help improve their quality of life and suggest ways in which access to new types of devices available to adults can be improved for children with T1D.Methods: In con...

hrp0082p1-d3-128 | Fat Metabolism &amp; Obesity (2) | ESPE2014

Acylated and Unacylated Ghrelin Levels in Children and Young Adults with Prader–Willi Syndrome

Kuppens Renske , Diene Gwenaelle , Bakker Nienke , Molinas Catherine , Faye S , Nicolino Marc , Bernoux Delphine , Delhanty Patric , Jan van der Lelij Aart , Allas Soraya , Julien Michiel , Delale Thomas , Tauber Maithe , Hokken-Koelega Anita

Background: Prader–Willi syndrome (PWS) is characterized by a switch in early childhood from failure to thrive to excessive weight gain and hyperphagia with impaired satiety. The underlying mechanism for this switch may involve hyperghrelinemia, but only poor data exists regarding levels of acylated ghrelin (AG), unacylated ghrelin (UAG), and the AG/UAG ratio in PWS.Objective and hypotheses: To investigate plasma levels of AG and UAG in PWS, compare...

hrp0092p1-322 | Diabetes and Insulin (2) | ESPE2019

Neonatal Diabetes and Glis3 Mutation: A New Phenotype

Kamoun Thouraya , Chabchoub Imen , Kmiha Sana , Julier Cecile , Hachicha Mongia

Background: The transcription factor Gli-similar 3 (Glis3) is predominantly expressed in the pancreas and it has a critical role in the development of insulin producing β-cells, thyroid and kidney. Mutations in GLIS3 is a rare cause of neonatal diabetes associated with congenital hypothyroidism, congenital glaucoma and polycystic kidney. We report a new case from consanguineous parents with homozygous novel mutation in GLIS3 gene.<p class="abstext"...

hrp0094s6.1 | Bone Formation: The Growth Plate and Beyond | ESPE2021

Epigenetic regulation of the growth plate

Lui Julian ,

Longitudinal growth is driven by endochondral ossification at the growth plate, where chondrocytes undergo a well-organized cellular differentiation process from stem-like progenitors to rapidly-proliferating transit-amplifying cells and to hypertrophic chondrocytes. Numerous recent studies have demonstrated that epigenetic modifications play a crucial role in human development and childhood growth. For example, Brachydactyly mental retardation syndrome (BDMR) is caused by hap...

hrp0086p1-p366 | Gonads &amp; DSD P1 | ESPE2016

Consecutive Lynestrenol and Cross-Sex Hormone Treatment in Biological Female Adolescents with Gender Dysphoria: A Retrospective Analysis

Tack Lloyd , Craen Margarita , Dhondt Karlien , Vanden Bossche Heidi , Laridaen Jolien , Cools Martine

Background: Progestins such as lynestrenol (L) can be used in female to male (FtM) adolescents with gender dysphoria (GD) who have advanced pubertal development to reduce the psychological burden of menstruation. L can later be combined with cross-sex hormones (testosterone esters) (L+T). L is much cheaper and easier to administer than GnRHa. To date, few data exist on the (side) effects of progestins for this indication.Objective and hypotheses: To repo...