hrp0092rfc12.5 | Growth and Syndromes (to include Turner syndrome) | ESPE2019

Cognitive and Neuroradiological Assessments in Silver Russell Patients

Patti Giuseppa , De Mori Letizia , Tortora Domenico , Savina Severino Maria , Calevo Mariagrazia , Morana Giovanni , Rossi Andrea , Casalini Emilio , Napoli Flavia , Di Iorgi Natascia , Maghnie Mohamad

Silver-Russell syndrome (SRS) is an epigenetic disorder characterized by severe intrauterine and postnatal growth retardation and typical dysmorphic features. The most common genetic abnormalities are 11p15 ICR1 loss of methylation, (11p15 LOM) and maternal uniparental disomy of chromosome 7 (mUPD7). There is little information on cognitive development in SRS patients and no neuroradiological studies are available so far. Global developmental delay and requirement for speech t...

hrp0092p1-179 | Diabetes and Insulin (1) | ESPE2019

Longitudinal Metabolic Control After Initiation of Insulin Pump in 5,040 Pediatric Type-1-Diabetes Subjects – Heterogeneous HbA1c Trajectories Over Three Years from the DPV Registry

Prinz Nicole , Schwandt Anke , Becker Marianne , Biester Torben , Hess Melanie , Holder Martin , Karges Beate , Näke Andrea , von Sengbusch Simone , Tauschmann Martin , Holl Reinhard W.

Objectives: Continuous subcutaneous insulin infusion (CSII) has been associated with lower HbA1c. To explore whether CSII initiation leads to HbA1c improvement in each individual with type-1 diabetes and to identify co-variates which might influence change in HbA1c.Methods: 5,040 pediatric type-1-diabetes subjects (δ20 y, 49% boys, median age at diabetes onset [Q1;Q3]: 5.9 [3.5;8.4] years) ...

hrp0092p1-252 | Pituitary, Neuroendocrinology and Puberty (1) | ESPE2019

Brain Malformations and Sellar Spine as Possible Causes of Central Precocious Puberty in a Large Monocentric Study

Fava Daniela , Calandrino Andrea , Morana Giovanni , Gastaldi Roberto , Allegri Anna Elsa Maria , Napoli Flavia , Roviglione Barbara , Di Iorgi Natascia , Maghnie Mohamad

Background: Central precocious puberty (CPP) is defined as the secondary sexual characteristics onset before 8 years of age in females and before 9 in males, due to premature activation of the hypothalamic-pituitary-gonadal axis. The underlying cause remains idiopathic in the great majority; based on the 2009 Consensus, 2% to 7% of girls who have onset of CPP between the ages of 6 and 8 years have unsuspected pathology and only 1% have a tumor such...

hrp0092p1-338 | Fat, Metabolism and Obesity (2) | ESPE2019

Continuous Score of Metabolic Syndrome (sSMp) in Chilean Pediatric Population is Associated with Insulin Resistance Parameters and Subclinical Endothelial Inflammation

Loureiro Carolina , Cavada Gabriel , Bancalari Rodrigo , Vecchiola Andrea , Tapia Alejandra , Baudrand René , Campino Carmen , Carvajal Cristian , Fardella Carlos , Martíinez Alejandro , García Hernán

Introduction: The dichotomous nature of the definition of Metabolic Syndrome (MS) in both children and adults can under-diagnose subjects at risk and prevents adequate follow-up of therapeutic interventions. Recently, a continuous score of MS (sSMp) was validated in the pediatric population based on the IDF criteria for a population> 16 years.Objectives: To apply sSMp in a Chilean pediatric population cohort and corr...

hrp0092p2-8 | Adrenals and HPA Axis | ESPE2019

Novel TBX19 Mutation as Cause of Hypoglicemia in Two Siblings

Castagna Andrea , Bottero Arianna , Ruggiero Jessica , Viola Anna , Perrotti Chiara , Oprandi Daniela , Rossi Stefano , Badolato Raffaele , Cutrì Maria Rosa , Mingotti Chiara , Grazzani Livia , Buzi Fabio , Pilotta Alba

We describe a female neonate born from consanguineous parents who presented at birth with respiratory distress and severe hypoglycemia. At six months of age, the child was admitted to the Intensive care Unit because of two critical episodes characterized by fever and loss of consciousness. Child condition were critical and suggestive of sepsis, but blood tests showed severe hypoglycemia (19 mg/dl), hyponatremia (Na 132 mmol/l), compensated metabolic acidosis and increased infl...

hrp0092p2-150 | Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia) | ESPE2019

Relations of O2 Supplementation to Blood Serum Insulin-like Growth Factor-II / Insulin-like Growth Factor-binding Protein-3 Ratios in the Not-life-threatened Human Newborn; Role of Oral-enteral Caloric Intake Beyond Axillary Temperature

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

Introduction: Oral/enteral caloric intake (KOE) insufficiency may accompany human newborn (NWB) respiratory derangements. We detected axillary temperature(TEMP) relations to NWB blood serum Insulin-like Growth Factor-II(IG2)-blood serum Insulin-like Growth Factor-Binding Protein-3(IB3) ratios(IG2/IB3R). Here we evaluate the TEMP-independent relevance of birth gestational age(GA) and KOE to relationships of O2 supplementation in respiratory gases(O2S) with IG2/...

hrp0092p3-133 | Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia) | ESPE2019

Relations of O2 Supplementation to Blood Serum Insulin-Like Growth Factor-I in the Not-Life-Threatened Human Newborn; Role of Oral-Enteral Caloric Intake Beyond Axillary Temperature

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

Introduction: Human newborn(NWB) respiratory derangements may be concomitant to oral/enteral caloric intake (KOE) insufficiency. We evidenced a possible involvement of axillary temperature(TEMP) in relationships between preterm birth (PTB) and blood serum Insulin-like Growth Factor-I(IG1) in NWBs. Here we evaluate the TEMP-independent role of birth gestational age(GA) and KOE in relations of O2 supplementation in respiratory gases(O2S) to IG1 in the not-life-t...

hrp0089rfc10.4 | Late Breaking | ESPE2018

Comparative Analysis between Immunoassay and Tandem Mass Spectrometry for Androgens before and after Human Recombinant Gonadotrophin in Children with Genital Ambiguity and 46,XY Karyotype

Oliveira Leticia , Guerra-Junior Gil , Longui Carlos , Guaragna-Filho Guilherme , Costa Jose Luiz , Lanaro Rafael , Silva David , Mello Maricilda , Maciel-Guerra Andrea , Morcillo Andre

Liquid chromatography associated with tandem mass spectrometry (LC-MS/MS) is currently considered the gold standard for steroid measurement. The aim of this study was to compare traditional immunoassay and LC-MS/MS methods for androgens measurement before and after human recombinant chorionic gonadotrophin (hrCG) stimulation in children with diagnosis of disorder of sex development (DSD) with 46,XY karyotype and past of normal testosterone secretion. We evaluated 19 patients, ...

hrp0089p1-p122 | Fat, Metabolism and Obesity P1 | ESPE2018

Determinants of Attrition from a Healthy Lifestyle Intervention: Experience from the CIRCUIT Program

Daniels Prince Kevin , Ybarra Marina , Van Hulst Andrea , Barnett Tracie A , Mathieu Marie-Eve , Drouin Olivier , Kakinami Lisa , Bigras Jean-Luc , Henderson Melanie

Introduction: Pediatric obesity is a global public health problem that is associated with severe cardiometabolic consequences. Weight management interventions focusing on lifestyles have shown some promising results, but attrition rates are often high and reasons for dropout are poorly understood.Objectives: We aimed toestimate the prevalence, and identify the determinants of attrition among pediatric participants in the first year of a 2-year lifestyle ...

hrp0089p3-p176 | Fetal, Neonatal Endocrinology and Metabolism P3 | ESPE2018

Axillary Temperature Relation to Blood Serum Insulin-like Growth Factor-I in the Not-life-Threatened Newborn: Relevance of Preterm Birth

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

Introduction: Preterm delivery may comport blood serum Insulin-like Growth Factor-I (IG1) decrements and lower body temperature during the first days of postnatal life of the human newborn (NWB). We evaluated the role of preterm delivery in associations between axillary temperature (TEMP) and IG1 in NWBs without life-threatening disease.Methods: NWBs with any among total parenteral nutrition, blood transfusion, therapeutic hypothermia, life-threatening d...