hrp0092fc8.3 | Pituitary, Neuroendocrinology and Puberty Session 1 | ESPE2019

Absence of Central Adrenal Insufficiency in Adults with Prader-Willi Syndrome

Rosenberg Anna , Davidse Kirsten , Pellikaan Karlijn , Donze Stephany , Hokken-Koelega Anita , van der Lely Aart Jan , de Graaff Laura

Introduction: Individuals with Prader-Willi syndrome (PWS) suffer from hyperphagia, hypotonia and hypothalamic dysfunction, leading to a variety of pituitary hormone deficiencies. Central adrenal insufficiency (CAI) has been reported in PWS, while each of these studies used different testing modalities and cut-off values. Therefore, reported prevalence of CAI ranges from 0% to 60%. It has been speculated that CAI might be responsible, at least in part,...

hrp0092rfc9.4 | Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia) | ESPE2019

Correlation Between Genotype and Phenotype Characteristics in Children with Congenital Hyperinsulinism (CHI) in a Specialist Centre

Bosch Laura , Kallefullah Mohammad Jasmina , Dastamani Antonia , Gilbert Clare , Morgan Kate , Houghton Jayne , Ellard Sian , Flanagan Sarah , Shah Pratik

Introduction and Aim: Congenital hyperinsulinism (CHI) is the most common cause of hypoglycaemia in early infancy and represents a heterogeneous disorder with respect to clinical presentation, histology and genetics. The aim of our study is to review correlation between genotype and phenotypic characteristics of children with CHI.Methods: Retrospective review of CHI patients with positive genetics during the last 8 years...

hrp0092rfc9.6 | Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia) | ESPE2019

Extra Uterine Growth Restriction (EUGR) in Very Low Birth Weight Infants: Growth Recovery and Neurodevelopment by the Corrected Age of 2 Years Old

Lucaccioni Laura , Arrigoni Marta , Della Casa Elisa , Bertoncelli Natascia , Predieri Barbara , Berardi Alberto , Pugliese Marisa , Ferrari Fabrizio , Iughetti Lorenzo

Background: Extra Uterine Growth Restriction (EUGR) represents a serious comorbidity in infants born very low birth weight (VLBW). In fact, failure in postnatal growth and malnutrition at vulnerable ages can interfere with growth recovery and neurodevelopment at older ages.Hypothesis: Aim of the study was to assess whether and how the postnatal early growth patterns of VLBW may affect later growth, spontaneous motility a...

hrp0092p1-14 | Bone, Growth Plate and Mineral Metabolism | ESPE2019

Refractory Hypercalcemia After Denosumab Treatment in Pediatric Age: A Case Report

Deodati Annalisa , Ubertini Graziamaria , Grossi Armando , Inzaghi Elena , Paone Laura , Maria Milano Giuseppe , Cappa Marco , Fintini Danilo

Background: Denosumab is a new class of antiresorptive medication and a fully human monoclonal antibody of the IgG2 immunoglobulin isotype to RANKL. In fact, it binds with high affinity and specificity to RANKL, mimicking the inhibitory effects of Osteoprotegerin, resulting in rapid suppression of bone resorption. Denosumab is commonly used also in pediatric age for treatment of osteoporosis, malignancies, and other benign bone lesions, such as fibrous dysplas...

hrp0092p1-364 | GH and IGFs (2) | ESPE2019

GH Values in Serum and Blood Spots on Filter Paper Samples in Neonates Until 30 Days of Life by Electrochemiluminescence (ECLIA).

Miras Mirta , Silvano Liliana , Campi Veronica , Ochetti Mariana , Sobrero Gabriela , Castro Laura , Martin Silvia , Testa Graciela , Franchioni Liliana

Growth Hormone deficiency (GHD) in newborn is an infrequent condition, which can cause threat to life due mainly to hypoglycemia that begins in the first week of life. A GH basal level (whether random or associated with spontaneous hypoglycemia) that distinguishes infants with GHD from those with GH sufficiency in the neonatal period is not conclusive. Few data have been reported about the GH measurements in serum and dried blood spots on filter paper samples in healthy neonat...

hrp0092p1-378 | Growth and Syndromes (to include Turner Syndrome) (2) | ESPE2019

Vascular Anomalies and Aortic Dilatation in Turner Syndrome Study in a Large Cohort of Young-Adult Patients

Scarano Emanuela , Varini Susanna , Tamburrino Federica , Perri Annamaria , Costa Margherita , Prandstraller Daniela , Lovato Luigi , Balducci Anna , Gibertoni Dino , Mazzanti Laura

Background: Patients with Turner Syndrome (TS) are at high risk for congenital heart disease (CHD), aortic dilatation and dissection with increased mortality and morbidity. Thoracic gadolinium-enhanced MRI angiography allows clear imaging of all great vessels and revealed a spectrum of silent vascular anomalies (VA), both venous and arterial, undetected at Echocardiography.The aim of this study is to retrospectively evaluate the prevalen...

hrp0092p2-102 | Diabetes and Insulin | ESPE2019

Flash Glucose Monitoring System Versus Blood Sugar Test Strips: Cost Comparison and Satisfaction During a Year in a Northern Spain Region

Bertholt Zuber Maria Laura , Freijo Martin Concepción , Naranjo Gonzalez Cristina , Palenzuela Revuelta Inmaculada , Mariscal Sara Pozas

Background: The flash glucose monitoring system (FGMS) has been a great advance in quality of life for patients diagnosed with type 1 diabetes (T1D). However, it is a more expensive method.Objectives: To describe the characteristics of the pediatric population (<18 years) diagnosed with T1D using FGMS, during one year in our community. To assess the degree of satisfaction and possible inconveniences. To evaluate the ...

hrp0092p3-238 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology | ESPE2019

Bilateral Testicular Atrophy and Normal Inhibin B level: A Paradoxal Clinical Finding For A Rare Biochemical Cause !

ESSADDAM Leïla , PIKETTY Marie , KALLALI Wafa , GUEDRI Rahma , GONZALEZ Laura , MATTOUSSI Nadia , POLAK Michel , BEN BECHER Saayda

Background: Testicular atrophy is a rare complication following inguinal hernia repair particularly in children<2 years and those with an undescended testis at highest risk> with an undescended testis. Inhibin B is secreted from the testis as a product of Sertoli cells, and has been suggested as a good marker for spermatogenesis. Its value is expected to be very low in children with bilateral testicular atrophyCase Report...

hrp0092p3-286 | Late Breaking Abstracts | ESPE2019

Alterations in Ambulatory Blood Pressure in Adolescents with Obesity

Diaz-Escobar Laura A , Lopez-Gonzalez Desiree , Garibay-Nieto Nayely , Villanueva-Ortega Erendira , Hernandez Ana Maria , Medeiros Mara

Introduction: Obesity is associated with comorbidities such as hypertension (HTN), and other alterations in blood pressure (BP) such as: masked hypertension and alterations in the circadian cycle variability, that only can be detect through ambulatory blood pressure monitoring (ABPM).A higher prevalence of masked hypertension has been reported in obese subjects, up to 4.3%. Also a loss in drop from mean daytime to mean night-time lev...

hrp0089p1-p012 | Adrenals and HPA Axis P1 | ESPE2018

Pediatric Adrenocortical Tumors. A Single Tertiary Center Experience: Clinical, Biological and Pathologic Characteristics Analysis

Mattone Maria Celeste , Gil Silvia , Mutti Maria Laura Galluzzo , Casanovas Alejandra , Lazzati Juan Manuel , Zaidman Veronica , Belgorosky Alicia , Guercio Gabriela

Background: Adrenocortical Tumors (ACT) is a rare endocrine malignancy with heterogeneous presentation.Aim: To evaluate the clinical, biochemical and pathologic characteristics of pediatric ACT in a single tertiary center.Subjects and methods: Review of 28 medical records with childhood ACT (chronological age (CA) <18 years (y) treated between 1987 and 2017. Clinical, biochemical, and histological features (Wieneke index), stag...