hrp0094p1-159 | Fetal Endocrinology and Multisystem Disorders B | ESPE2021

Clinical features and body mineral density in Italian patients with rapid onset obesity with hypoventilation, hypothalamic dysfunction, autonomic disregulation and neural tumor (ROHHADNET): a single center observational study

Angelelli Alessia , Fava Daniela , Thiabat Hanan F. , Guglielmi Davide , Ceradelli Francesca Lorenzini , Maiorano Nadia Gabriella , Marasini Lucia , Pistorio Angela , Ceccherini Isabella , Napoli Flavia , Iorgi Natascia Di , Maghnie Mohamad ,

Background: ROHHAD (rapid-onset obesity with hypoventilation, hypothalamic dysfunction, autonomic dysregulation) syndrome is a rare and underdiagnosed disease with high mortality rate. When a neural crest tumor (NET) is detected (56% of patients) the acronym used is ROHHADNET.Aim: To analyze the relationship between clinical variables, hypothalamic-pituitary disorders, DXA parameters and neural tumors, in ROHHADNET patie...

hrp0094p2-176 | Fat, metabolism and obesity | ESPE2021

Exploring Urinary Bile Acids as Potential Markers of Metabolism: Reference Values in Children by Targeted LC-MS/MS

Schauermann Marcel , Wang Rong , Hartmann Michaela F. , Remer Thomas , Hua Yifan , Shmoish Michael , Bereket Abdullah , Wasniewska Malgorzata , Hochberg Ze’ev , Gawlik Aneta , Wudy Stefan A. ,

Bile acids (BA) are C24 steroids synthesized from cholesterol in liver. Originally, they were mainly considered to function as emulsifiers supporting resorption of lipophilic food compounds and excretion of metabolic products. Recently, their additional biological and endocrine functions in the regulation of metabolism have aroused interest of researchers. In contrast to BA in blood, it is surprising that hardly any data exist on BA in the most accessible human biof...

hrp0097fc14.5 | Late Breaking | ESPE2023

Predicting Average IGF-I Concentration for Once-Weekly Somapacitan in Children with Growth Hormone Deficiency

F. Backeljauw Philippe , Kamal Lyauk Yassine , C. Blair Joanne , S. Miller Bradley , Mori Jun , Højby Rasmussen Michael , Juul Kildemoes Rasmus

Growth hormone (GH) stimulates insulin like growth factor I (IGF I) release. IGF-I is the standard biomarker for monitoring GH effects during treatment and to achieve optimal long-term safety, and to a limited extent, monitor efficacy in children with GH deficiency (GHD). The IGF I profile during treatment with a long acting GH (LAGH), such as once weekly somapacitan (Novo Nordisk), differs from the daily GH profile by exhibiting larger peaks and troughs over the dosing interv...

hrp0097p1-256 | Fat, Metabolism and Obesity | ESPE2023

Serum leptin concentrations in a pooled cohort of 6.105 children and adolescents: Reference values as a function of dependence on sex, age, pubertal stage and BMI-SDS

Brandt Stephanie , Vogel Mandy , Kratzsch Jürgen , Tews Daniel , Pridzun Lutz , Flehmig Bertram , B Ranke Michael , Körner Antje , Kiess Wieland , F Blum Werner , Wabitsch Martin

Background: Current reference values for leptin in childhood and adolescence are presented separately for girls and boys, and as a function of age without considering the variability of fat mass. This complicates the interpretation of measured serum leptin concentrations since fat mass is the major determinant of circulating leptin concentrations. To fill this gap, we aimed at pooling existing data (age, BMI, sex, tanner stage (TS), serum leptin concentrations...

hrp0097p1-92 | Fetal, Neonatal Endocrinology and Metabolism | ESPE2023

Relationship between birth body weight< centile (sga) and insulin-like growth factor binding protein-3: relevance of birth chest circumference / birth body weight ratio independently of birth gestational age, insulin-like growth factor binding protein-1 and -2 in the not-life threatened newborn

Terzi Cesare , Virdis Raffaele , Magnani Cristiana , Tridenti Gabriele , Cerioli Andrea , Riani Marco , Garavelli Lidia , Luigi De Angelis Gian , Bernasconi Sergio , F. Blum Werner , Banchini Giacomo

Birth chest circumference(CC) shows often, like birth gestational age(GA), tight direct relations to birth body weight(BW). However distinct connections of hypoxia/undernutrition with different body structures might be suspected based on brain-, heart- and adrenal-sparing following intrauterine growth restriction and, postnatally, on higher chest size for body mass observed at high altitude. Growth retarded fetuses gestated by hypoxic pregnant animals may present increments of...

hrp0097p1-279 | Fetal, Neonatal Endocrinology and Metabolism | ESPE2023

A Year-Long, National Trial of Prospective CGM Use in Families with Hyperinsulinism

Worth Chris , Worthington Sarah , Auckburally Sameera , Ahmad Sumera , O'Shea Elaine , Ferrera-Cook Chris , F Betz Stephen , Salomon-Estebanez Maria , Banerjee Indraneel

Introduction: Congenital hyperinsulinism (CHI) is the commonest cause of severe hypoglycaemia in early childhood but glycaemic characterisation remains scarce. Continuous glucose monitoring (CGM) offers a deep understanding of glycaemic control to understand disease burden, individualise patient care and inform therapeutic trials in CHI. Preliminary studies suggest inadequate accuracy and no efficacy of standalone CGM to reduce hypoglycaemia. Provision is hist...

hrp0097p1-484 | Fetal, Neonatal Endocrinology and Metabolism | ESPE2023

Relationships between birth body weight< centile (SGA) and insulin-like growth factor-ii / insulin-like growth factor binding protein-3 ratio in the not-life threatened newborn: relevance of birth chest circumference / birth body weight ratio and oxygen supplementation

Terzi Cesare , F. Blum Werner , Magnani Cristiana , Tridenti Gabriele , Cerioli Andrea , Riani Marco , Chesi Elena , Luigi De Angelis Gian , Bernasconi Sergio , Virdis Raffaele , Banchini Giacomo

Direct relationships of estimated birth brain weight(BRW) to birth body weight (BW) ratios (BBR) and of BW< centile for GA(SGA) with blood serum Insulin-like Growth Factor-II(IG2) to blood serum Insulin-like Growth Factor Binding Protein-3 (IB3) ratios (IG2/IB3R), and inverse relations between BW-SDS and birth chest circumference(CC) / BW ratio (i.e., CC through BW; CC/BWR) have been detected by our group in the human newborn(NWB). We evaluated the possibility that CC...

hrp0097p1-168 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2023

Parental perceptions and concerns in a cohort of infants with unoperated hypospadias

L. Leunbach Tina , Ernst Andreas , Wisniewski Amy , Berglund Agnethe , H. Gravholt Claus , Faisal Ahmed Syed , M. Hvistendahl Gitte , F. Rawashdeh Yazan

Background: Hypospadias surgery is usually performed during infancy at which time parents act as proxy decision makers. Parental decisional regret, a common phenomenon after hypospadias surgery, may be affected by the process of informed consent where decision making between parents and healthcare provider is shared based on available evidence and individual concerns.Objective: To identify short- and long-term parental c...

hrp0097p1-567 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2023

An International Delphi Based Study For Developing A Core Outcome Set For Hypospadias Surgery

Leunbach Tina. , Yankovic Francisca , Springer Alexander , Wisniewski Amy , Burgu Berk , Shnorhavorian Margarett , Braga Luis , Ernst Andreas , Lucas-Herald Angela , O'Toole Stuart , Faisal Ahmed S. , F. Rawashdeh Yazan

Background: Heterogeneity in reported outcomes limits the ability to compare results of studies evaluating hypospadias surgery.Objective: To identify a core outcome set (COS), a minimal number of defined outcomes, to be routinely measured and reported in all trials across the age span following hypospadias surgery.Materials and Methods: A study protocol was drafted and the study re...

hrp0084p2-406 | GH &amp; IGF | ESPE2015

Metabolic Health in Short Children Born Small for Gestational Age Treated with GH and GnRHa: Results of a Randomised, Dose-response Trial

van der Steen M , Lem A J , van der Kaay D C M , Waarde W M Bakker-van , van der Hulst F J P C M , Neijens F S , Noordam C , Odink R J , Oostdijk W , Schroor E J , Sulkers E J , Westerlaken C , Hokken-Koelega A C S

Background: Previously we showed that pubertal children born small for gestational age (SGA) with a poor adult height (AH) expectation can benefit from treatment with GH 1 mg/m2 per day (~0.033 mg/kg per day) in combination with 2 years of GnRH analogue (GnRHa) and even more so with a double GH dose. GnRHa treatment is thought to have negative effects on body composition and blood pressure. Long-term effects and GH-dose effects on metabolic health in children treate...