hrp0082p1-d3-51 | Bone (1) | ESPE2014

Bone Geometry, Volumetric Density, Microarchitecture and Estimated Bone Strength Assessed by HR-pQCT in Adult Patients with Hypophosphatemic Rickets

Shanbhogue Vikram v. , Hansen Stinus , Folkestad Lars , Brixen Kim , Beck-Nielsen Signe Sparre

Background: Hypophosphatemic rickets (HR) are rare, inheritable disorders caused by excessive renal phosphate wasting. Despite a generalized mineralization defect, patients with HR are reported with a lower risk of fracture.Objective and hypotheses: The aim of this study was to evaluate the effect of bone -geometry, -microarchitecture and volumetric BMD (vBMD) on the estimated bone strength in adult patients with HR using high-resolution peripheral quant...

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 ...

hrp0097p1-283 | Fetal, Neonatal Endocrinology and Metabolism | ESPE2023

Developing a Collaborative Research Network to Accelerate the Understanding and Treatment of the Rare Disease Congenital Hyperinsulinism

Pasquini Pasquini Tai , Raskin Julie , De León-Crutchlow Diva , Banerjee Indi , Christesen Henrik , Conwell Louise , Dastamani Antonia , Flanagan Sarah , Gillis David , Kalish Jennifer , Lord Katherine , Stanley Charles , Zangen David , Thornton Paul

Background: Congenital Hyperinsulinism International (CHI) is an international non-profit organization focused on improving the lives of patients and families living with hyperinsulinism (HI). Despite many advances in the care of patients with HI, long term neurologic outcomes have not significantly improved, highlighting the need for CHI’s goals for robust and rapidly translatable research. We describe the development of a collaborative research network...

hrp0082fc7.3 | Growth promoting therapies | ESPE2014

Gene Expression Networks Associated with Changes in Serum Markers of Metabolism and Growth in GH-Treated Children with GH Deficiency

Stevens Adam , De Leonibus Chiara , Chatelain Pierre , Clayton Peter

Introduction: Growth promoting effects of GH occur in parallel with its impact on insulin sensitivity and lipid metabolism; underlying biological networks that link these actions are not defined. Our objective was to identify gene expression (GE) networks linking growth with metabolic responses in GH-treated children with GHD.Methods/design: Pre-pubertal children with GH Deficiency GHD (n=125) were enrolled from the PREDICT short-term (NCT002561...

hrp0084p3-901 | Fat | ESPE2015

Metabolic Syndrome Rates Among Adolescents of the Greek School Community

Kotanidou Eleni , Kleisarchaki Angeliki , Mouzaki Konstantina , Kokka Paraskevi , Kyrgios Ioannis , Tsara Iro , Efstratiou Efthimia , Haidich Anna-Bettina , Papageorgiou Vagia , Eboriadou-Petikopoulou Maria , Galli-Tsinopoulou Assimina

Background: Adolescent obesity constitutes a phenomenon with epidemic prevalence in a worldwide context. Increased rates of metabolic syndrome represent a consequence of this epidemic, leading to high risk for chronic diseases in adolescence. The study herein, presents the primary outcomes of a school-based intervention trial regarding the control of the metabolic syndrome among adolescents of Northern Greece.Aims and objectives: To investigate the preva...

hrp0082s1.3 | Disorders of Gsalpha Signaling | ESPE2014

New Therapies in Metabolic Bone Disease: PTH

Linglart Agnes

Hypoparathyroidism is a rare disease manifesting as hypocalcemic seizures, developmental delay, sluggishness, paresthesias and/or dyskinesia. Symptoms reveal hypocalcaemia associate with increased serum phosphorus and undetectable, low or unadapted PTH levels. Known causes of hypoparathyroidism are cervical surgeries causing irreversible damage to the parathyroid tissue, toxic attack of the parathyroid cells by antibodies, and genetic defects affecting parathyroid development,...

hrp0086s6.3 | Prevention of childhood obesity | ESPE2016

Childhood Obesity Prevention: What Do We Need and How Are We Doing? A European Perspective

Brug Johannes

The worldwide prevalence of overweight and obesity has been steadily increasing, also among children and adolescents, and has reached alarming pandemic proportions in Europe and elsewhere. Childhood overweight is a main predictor of adult overweight and obesity. Obesity treatment is largely ineffective, and a still stronger focus on primary prevention is needed. Such prevention should take a population and a life course approach and should focus on promoting healthy energy bal...

hrp0086p1-p107 | Bone & Mineral Metabolism P1 | ESPE2016

Bone Mineral Status in Children and Adolescents with Klinefelter Syndrome

Stagi Stefano , Scalini Perla , Di Tommaso Mariarosaria , Chiarelli Francesco , Manoni Cristina , Parpagnoli Maria , Verrotti Alberto , Lapi Elisabetta , Giglio Sabrina , Dosa Laura , de Martino Maurizio

Background: Klinefelter syndrome (KS) has long-term consequences on bone health. However, studies regarding bone status and metabolism in childhood and adolescence are very rare.Objective and hypotheses: The purpose of our study was to evaluate bone status and metabolism in a cohort of KS children and adolescents.Method: This cross-sectional study involves 40 (mean age 13.7±3.8 years) KS children and adolescents and 80 age-mat...

hrp0082p2-d2-374 | Fat Metabolism & Obesity (1) | ESPE2014

The Role of Bone and Fat Tissue in Glucose Metabolism in Two Different Metabolic Conditions: Obesity and Diabetes Mellitus Type 1: Pilot Study

Wedrychowicz Anna , Sztefko Krystyna , Starzyk Jerzy

Background: Recent studies have shown a new link between skeleton, fat tissue, and insulin action. However, clinical data are still limited, especially in children.Objective: The aim of the presented study was to investigate the relationship between bone and fat hormones and glucose metabolism in children with type 1 diabetes mellitus (T1DM) and obesity.Methods: Forty-six T1DM children, mean age 12.2±4.6 years, mean BMI 20.0&#...

hrp0089fc12.3 | Diabetes and Insulin 2 | ESPE2018

Impact of Insulin Sensitivity and β-cell Function on the Development of Impaired Glucose Tolerance (IGT) in Obese European Children and Adolescents

Denzer Christian , Vogt Josef , Kohlsdorf Katja , von Schnurbein Julia , Wabitsch Martin

Objectives: Compared to the US, prevalence rates of T2DM in obese children are significantly lower in European countries. Data from cohorts of obese children living in the US suggest a concurrent worsening of insulin sensitivity and ß-cell function over the spectrum of glucose tolerance. If these results can be applied to European populations is currently unknown.Methods: A combination of our novel method for mathematical modelling ...