hrp0084p3-1105 | Pituitary | ESPE2015

Off-label Use of Vaptans in Children with Severe Symptomatic Hyponatremia due to SIADH

Tuli Gerdi , Tessaris Daniele , Di Taranto Serena , Giorgis Alberto , Einaudi Silvia , Matarazzo Patrizia

Background: Vaptans, vasopressin receptor 2 antagonist, are used in adults to treat hyponatremia associated with congestive heart failure, cirrhosis, and the syndrome of inappropriate antidiuretic hormone (SIADH). To date, in paediatric patients with SIADH there are few data about use of vaptans, still considered off-label.Case presentation 1: A 9-yo female with surgically treated suprasellar astrocitoma developed chronic hyponatremia (121–128 mmol/...

hrp0084p3-1237 | Turner | ESPE2015

Clinical Features and Genetic Considerations of Turner Syndrome: A Review of Our Cases

Berrade Sara , Chueca Maria , Zarikian Sada , Mosquera Arantxa , Ulibarrena Noelia , Sola Alberto , Garcia Cristina , Oyarzabal Mirentxu

Background: Turner syndrome (TS) involves a partial or complete loss of an X chromosome. TS patients have an increased susceptibility to various disorders.Objective and hypotheses: To describe the clinical presentation, genotype and follow-up of TS patients controlled in the Pediatric endocrinology department of our hospital.Method: Retrospective study of patients diagnosed with TS at the ‘Navarra Hospital’ between 1980&#...

hrp0094p1-169 | Growth B | ESPE2021

Safety of growth hormone and cardiovascular outcomes in patients with Noonan syndrome enrolled in NordiNet® International Outcome Study (IOS) and the ANSWER Program

Romano Alicia , Kaski Juan Pablo , Dahlgren Jovanna , Kelepouris Nicky , Pietropoli Alberto , Rohrer Tilman R. , Polak Michel ,

Introduction: Growth hormone (GH) treatment has been shown to increase height velocity and adult height in patients with Noonan syndrome (NS). NS is also associated with cardiovascular (CV) anomalies, namely pulmonary stenosis and hypertrophic cardiomyopathy. Concerns persist about the role of GH in progression of CV conditions despite data, albeit limited, showing low rates of CV events and left ventricular wall thickness remaining normal. This pooled analysi...

hrp0097p1-289 | GH and IGFs | ESPE2023

Long-term effectiveness and safety of growth hormone therapy in Japanese children with short stature due to Noonan syndrome (NS): real-world data

Muroya Koji , Kawai Masanobu , Yamagishi Hiroyuki , Endo Takaaki , Pietropoli Alberto , Horikawa Reiko

Norditropin® was approved for children with short stature due to NS in Japan in 2017. The aim of this post-marketing surveillance study was to evaluate long-term safety and effectiveness of Norditropin® for the approved indication. This real-world non-interventional study (NCT03435627) was conducted February 2018–January 2022. Seventy patients enrolled: 35 received Norditropin® after study initiation (new patients); 35 were previously ra...

hrp0097p2-115 | GH and IGFs | ESPE2023

Are there sex differences among patients to whom a growth hormone (GH) test with clonidina stimulus was required during the study of short stature?

Vela Amaya , GarcÍA Maria , Izquierdo Jon , Ugeso Alberto , Grau Gema , Rica Itxaso

Introduction: GH stimulus test is usually performed in pacientes With short stature and/or growth delayed study if GH deficit is suspected. Several articles concluded that male are more frequently diagnosed and treated of GH deficiency than femaleObjectives: To know gender's ratio and the possible anthropometric differences between patients that required a consult for short stature and to whom a stimulus test was pe...

hrp0084p2-429 | GH & IGF | ESPE2015

Effectiveness of Recombinant IGF1 Treatment in a Patient with Isolated GH IA Deficit Producer of Anti-GH Antibodies

Porcar Ines , Oriola Josep , Kratzsch Jurgen , Escribano Joaquin , Feliu Albert

Background: GH deficiency type IA represents the most serious form of isolated deficit GH (IDGH). It’s transmitted as an autosomal recessive pattern and in most cases there is a homozygous deletion of the GH1 gene. Good initial response to treatment is characteristic, although often could appear antibodies against recombinant GH.Case presentation: We report a case of a 5-year-old Pakistanist boy evaluated for severe growth failure (heigh <8.07 <...

hrp0095rfc11.3 | Late Breaking | ESPE2022

Predictors of kidney damage development in children with metabolically healthy and metabolically unhealthy obese phenotypes

Di Sessa Anna , Paride Passaro Antonio , Maria Colasante Alberto , Ferrara Serena , Rosaria Umano Giuseppina , Marzuillo Pierluigi , Miraglia del Giudice Emanuele

Background: A close association has been reported between obesity and renal damage in adults, but evidence in childhood is still limited.Methods: We retrospectively studied 396 children and adolescents with obesity. Clinical, biochemical, and instrumental evaluations were performed. Patients were stratified according to the metabolically healthy obesity (MHO) and metabolically unhealthy obesity (MUO) phenotypes. Kidney d...

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...

hrp0089p1-p135 | Fetal, Neonatal Endocrinology and Metabolism P1 | ESPE2018

Cardiac and Vascular Assessments in Small- vs Appropriate-for-Gestational-Age Infants at Ages 1 and 2 Years

Garcia-Beltran Cristina , Sebastiani Giorgia , Pie Stella , Guerra Alberto , Lopez-Bermejo Abel , de Zegher Francis , Roses Ferran , Ibanez Lourdes

Background: Children born small-for-gestational age (SGA), especially those who experience spontaneous postnatal catch-up growth, are at increased risk for developing insulin resistance, central adiposity and cardiovascular abnormalities later in life. By age 3-6 years, SGA children have a broader aortic and carotid intima media thickness (aIMT and cIMT) which are markers of preclinical atherosclerosis.Objective: To assess longitudinally – at age 12...

hrp0086p2-p146 | Bone &amp; Mineral Metabolism P2 | ESPE2016

Bone Mineral Status and Metabolism in Patients with Williams-Beuren Syndrome

Stagi Stefano , Scalini Perla , Chiarelli Francesco , Verrotti Alberto , Giglio Sabrina , Ricci Franco , Lapi Elisabetta , Romano Silvia , Martino Maurizio de

Background: Despite the multiple endocrine, cardiovascular, and gastroenterologic problems of patients with Williams-Beuren Syndrome (WBS), Studies considering metabolism and bone quality in WBS are almost entirely absent from the literature.Objective and hypotheses: We evaluate bone mineral status and metabolism in a cohort of patients with WBS.Method: Thirty-one children (15 females, 16 male...