hrp0097p1-51 | Fat, Metabolism and Obesity | ESPE2023

GLP1 agonists improve glycaemic dysregulation, satiety levels and quality of life in adolescents with obesity

Apperley Louise , Parkinson Jennifer , Senniappan Senthil , Patrizia Bruzzi

Introduction: GLP-1 receptor agonists (GLP1A) have been shown to be effective in achieving weight loss in adolescents with obesity and improve glycaemic control in type 2 diabetes mellitus (T2DM). We aimed to investigate the glycaemic alterations and satiety levels in patients treated with the GLP1A, liraglutide. To the best of our knowledge, this is the first study of its kind.Methods: In total, 22 patients managed in a...

hrp0097p1-260 | Fat, Metabolism and Obesity | ESPE2023

Early onset obesity due to Melanocortin 4 receptor (MC4R) defect; Successful treatment with Semaglutide

Ramya Gokul Pon , Apperley Louise , Parkinson Jennifer , Seniappan Senthil

Introduction: Childhood obesity is a growing concern worldwide, and it has been linked to several nutritional and genetic factors. In some patients, monogenic causes can be identified, which are due to single gene mutations in specific pathways related to appetite regulation. One of the most common monogenic causes of obesity is heterozygous mutations in Melanocortin 4 receptor (MC4R), with a prevalence ranging from 2% to 6% in juvenile-onset obesity. We repor...

hrp0095rfc4.5 | Fat, Metabolism and Obesity | ESPE2022

Cerebral perfusion following childhood-onset craniopharyngioma and the relationship with metabolic rate

Elsworth Rebecca L. , Naeem Nimra , Hawton Katherine , Narayan Kruthika , Elson Ruth , Taylor-Miller Tashunka , Lithander Fiona E. , Hamilton-Shield Julian P. , Crowne Elizabeth C. , Hinton Elanor C.

Background: Craniopharyngioma is a non-malignant embryonic tumour in the pituitary-hypothalamic area, associated with hypothalamic obesity. Dysfunctional parasympathetic nervous system activity has been proposed as one mechanism underlying alterations in energy metabolism. Arterial spin labelling (ASL) is a non-invasive MRI technique that quantifies brain tissue perfusion as a proxy for functional activity. Here, we measure cerebral perfusion in patients with ...

hrp0095rfc6.2 | Sex Development and Gonads | ESPE2022

Dynamic changes of reproductive hormones in male minipuberty: Temporal dissociation of Leydig- and Sertoli-cell activity

S. Busch Alexander , Lindhardt Ljubicic Marie , N Upners Emmie , Bistrup Fischer Margit , Lau Raket Lars , Frederiksen Hanne , Albrethsen Jakob , Holm Johannsen Trine , P Hagen Casper , Juul Anders

Background: The male Hypothalamic-Pituitary-Gonadal (HPG) axis is transiently active during the first months of life with surging serum concentrations of reproductive hormones. This period, termed minipuberty, promotes masculinization, including testicular, and penile growth, as well as further testicular descent in males. It appears to be essential for priming specific cell types, i.e. Sertoli-, Leydig-, and germ cells, including subsequent growth and maturat...

hrp0095rfc8.4 | Diabetes and Insulin | ESPE2022

Developing a regional Southeast Asia diabetes healthcare professional network and webinars programme initiative during the pandemic

Sze May Ng , Lek Ngee , Sahakitrungruang Ngee , Pheng Chan Siew P , Yong Lai Mee , Yazid Jalaludin Muhammad , Thao Bui Muhammad , Nyi Nyi Soe , Ficheroulle Anne-Charlotte , Toomey Charles

Background: In 2020, the average glycaemic index of Type 1 diabetes (T1D) in Southeast Asia (SEA) countries were reported to be at around 83mmol/mol. In many low-middle-income countries (LMICs) in SEA, insufficient infrastructure, lack of universal health coverage and professional knowledge are factors that affect T1D outcomes that adversely affect mortality and morbidity. Action4diabetes (A4D) currently provides comprehensive partnership programmes with defin...

hrp0095p2-158 | Growth and Syndromes | ESPE2022

Global recall of a growth hormone jet delivered device: experiences with children forced to switch from a needle free system to a growth hormone device with needle.

P. van Eck Judith , S. Renes Judith , J.C. Sas Theo , L.T. van den Akker Erica , C.M. van der Kaay Daniëlle

Introduction: Growth hormone (GH) treatment is essential for growth in children with GH deficiency. Also short children born small for gestational age (SGA), and children with syndromes like Turner Syndrome (TS), Noonan Syndrome (NS) and Silver Russel Syndrome (SRS) can benefit from GH treatment. For children with needle anxiety GH delivered by a jet device can be a solution for the daily subcutaneous treatment for many years. In 2021, a global recall of the o...

hrp0092fc8.5 | Pituitary, Neuroendocrinology and Puberty Session 1 | ESPE2019

LGR4-Wnt β-Catenin Signalling Directs GnRH Network Development, with Defects Leading to Self-Limited Delayed Puberty

Mancini Alessandra , Howard Sasha R. , Cabrera Claudia P. , Barnes Michael R. , David Alessia , Wehkalampi Karoliina , Vassart Gilbert , Cariboni Anna , Garcia Marie Isabelle , Guasti Leonardo , Dunkel Leo

Background: The initiation of puberty is dependent upon an augmentation of gonadotropin-releasing hormone (GnRH) secretion from the hypothalamus. Development of the GnRH neuroendocrine network in embryonic life depends on coordinated migration of neurons from the vomeronasal organ in the nose to the forebrain. We have previously demonstrated that dysregulation in GnRH neuronal migration leads to delayed pubertal onset. Late puberty affects up to 2% of the ...

hrp0092p1-100 | Pituitary, Neuroendocrinology and Puberty | ESPE2019

RNPC3 Mutations Associate Prolactin Deficiency and Ovarian Insufficiency, Expanding the Phenotype Beyond Isolated Growth Hormone Deficiency Type V (MIM#618860)

Martos-Moreno Gabriel Á. , Travieso-Suárez Lourdes , Pozo Jesús , Chowen Julie , Pérez-Jurado Luis A. , Argente Jesús

Background: The first three children reported to have biallelic mutations in RNPC3 presented with growth hormone (GH) deficiency and pituitary hypoplasia (MIM#618860). RNPC3 codes for a minor spliceosome protein required for U11/U12 small nuclear ribonucleoprotein formation and splicing of U12-type introns. The underlying mechanism causing GH deficiency in these patients is not fully understood. Moreover, whether the association of further ho...

hrp0092p1-110 | Pituitary, Neuroendocrinology and Puberty | ESPE2019

References for Testicular Volume Measured by Ultrasound and for Pubic Hair in 6-16 Year-Old Norwegian Boys

Oehme Ninnie B. , Roelants Mathieu , Bruserud Ingvild S. , Madsen André , Eide Geir Egil , Bjerknes Robert , Rosendahl Karen , Júlíusson Pétur B.

Objective: Recent studies have suggested earlier onset of pubertal development in boys. As assessment with orchidometer tends to overestimate true testicular volume (TV), and measurements based on ultrasound (US) have been proposed as a more reliable method, we present US based references in 6-16 year-old Norwegian boys. Our results are compared with data from Europe and the United States (U.S.) in order to establish evidence for an ongoing secular trend in ma...

hrp0092p1-367 | GH and IGFs (2) | ESPE2019

Normal IGF-Bioactivity and Low Free IGF-I in Patients with Prader-Willi Syndrome with High Total Serum IGF-I: Immunoreactive IGF-I Concentration Poorly Reflects IGF Bio-Activity and Bio-Availability.

Elizabeth Melitza , Donze Stephany , Pellikaan Karlijn , van den Berg Sjoerd , van Doorn Jaap , Peeters Robin P. , Hokken-Koelega Anita C.S. , de Graaff Laura C.G.

Introduction: Recombinant Growth Hormone (GH) has changed the lives of many patients with Prader-Willi Syndrome (PWS). GH treatment has beneficial effects on body composition, physical performance, cognition, psychomotor development, respiratory function and quality of life of patients with PWS. Due to the narrow therapeutic range, GH treatment is subject to strict limits. Clinicians measure serum immunoreactive Insulin-like Growth Factor 1 ('total IGF-I&#...