hrp0086p1-p341 | Gonads & DSD P1 | ESPE2016

Application of on Line Learning in Assessment of Competencies of Fellows Pediatric Endocrinology

Kranenburg Laura , Reerds Sam , Cools Martine , Muscarella Miriam , Grijpink Kalinka , Quigley Charmian , Drop Stenvert

Background: The European Society for Paediatric Endocrinology (ESPE) developed an interactive e-learning portal for pre-and postdoctoral training: (www.espe-elearning.org).Objective and hypotheses: The aim of the study was to evaluate the role of e-learning in the formative assessment of competencies (medical expert, communicator) of fellows in pediatric endocrinology in informing patients and parents with a di...

hrp0086p2-p543 | Fat Metabolism and Obesity P2 | ESPE2016

25-Hydroxyvitamin D Concentrations in Pubertal Children with Obesity

Yordanova Desislava , Stefanova Elisaveta , Kazakova Krasimira , Todorova Zdravka , Dimitrova Mihaela

Background: The prevalence of childhood obesity has been rapidly increasing worldwide and the last report of World Health Organisation define it as epidemic and one of the most serious global public health challenges for the 21st century. Obese children and adolescents are at an increased risk of developing various health problems including type 2 diabetes mellitus, hypertension, osteoarthritis, cardiovascular disease. Obesity is a risk factor for vitamin D deficiency. Vitamin...

hrp0086p1-p594 | Growth P1 | ESPE2016

Somapacitan, a Once-Weekly Reversible Albumin-Binding Growth Hormone (GH) Derivative, Is Well Tolerated and Convenient in Adults with GH Deficiency (AGHD): Results from a 26-Week Randomised, Controlled Phase 3 Trial

Johannsson Gudmunder , Feldt-Rasmussen Ulla , Holme Haakonsson Ida , Biering Henrik , Rodien Patrice , Tahara Shigeyuki , Toogood Andrew , Hojby Michael

Background: Growth hormone (GH) replacement as daily s.c. injections for patients with adults with GH deficiency (AGHD) can be cumbersome. Somapacitan (Novo Nordisk), a once-weekly reversible albumin-binding GH derivative, has been shown in short-term trials to be well tolerated in healthy adults and in patients with AGHD.Objective and hypotheses: This trial was a multinational, multicentre, randomised (2:1), open-label, active-controlle...

hrp0086p2-p768 | Pituitary and Neuroendocrinology P2 | ESPE2016

10 Years Review of Endocrine Diseases in Spanish Patients Diagnosed with Primary Brain Tumors in a Tertiary Hospital

Portillo Nancy , Gonzalez Usune , Rubio Raquel , Ariza Miguel Garcia , Grau Gema , Vela Amaia , Rodriguez Amaia , Astigarraga Itziar , Rica Itxaso

Background: Pediatric Central Nervous System (CNS) neoplasms are the most frequent solid tumors in children. Since the increase in survival, the patients are in high risk of developing long term sequelae. Endocrinological sequelae may be due to the oncological disease itself but usually derived from the treatment received, and they affect 20–50% of patients long-term. We aimed to review our experience from 2005 to 2015.Objective and hypotheses: Desc...

hrp0086p1-p898 | Thyroid P1 | ESPE2016

Do Different Initial Doses of L-T4 within the Range of 10-15 mcg/kg/day Influence Neurodevelopment during the First Two Years of Life in Children with Congenital Hypothyroidism?

Esposito Andrea , Bravaccio Carmela , Bruzzese Dario , Cassio Alessandra , Gastaldi Roberto , Mussa Alessandro , Peroni Elena , Polizzi Miriam , Vigone Maria Cristina , Wasniewska Malgorzata Gabriela , Weber Giovanna , Salerno Mariacarolina

Background: The initial L-T4 dose currently recommended in the treatment of congenital hypothyroidism (CH) is 10–15 mcg/kg per day.Objective and hypotheses: We designed a multicenter randomized trial to evaluate the effects of different starting doses of L-T4 within the range 10–15 mcg/kg per day on neurocognitive development in children with CH.Method: Seventy-two children with CH diagnosed by neonatal screening were enr...

hrp0082fc9.6 | Beta cells | ESPE2014

Sulfonylurea Therapy Corrects Hypotonia, Attention Deficits, Improves Complex Neuropsychological Functions and Motricity in Patients with Neonatal Diabetes Secondary to Mutation in Potassium Channel Subunits, Through a CNS Effect

Beltrand Jacques , Vaivre-Douvret Laurence , Busiah Kanetee , Fournier Emmanuel , Boddaert Nathalie , Vera Myriam , Bahi-Buisson Nadia , Bui-Quoc Emmanuel , Ingster-Moati Isabelle , Flechtner Isabelle , Simon Albane , Scharfmann Raphael , Cave Helene , Elie Caroline , Polak Michel

Background: Sulfonylurea therapy (SU) allows a better metabolic control than insulin in patients with neonatal diabetes secondary to mutation in potassium channel subunits (ND-K). Most of these patients have neurological and neuromotor developmental impairments whose changes under SU has not been studied in a systematic and prospective way in a large cohort.Objective and Hypotheses: To demonstrate the beneficial effect of SU on neuropsychological functio...

hrp0082p1-d2-113 | Fat Metabolism & Obesity (1) | ESPE2014

Prenatal Maternal Weight and Weight Status and Lipid Profile of the Offspring

Riano-Galan Isolina , Rodriguez-Dehli Cristina , Velazquez Ines Olaya , Fernandez-Somoano Ana , Valvi Damaskini , Rebagliato Marisa , Ibarluzea Jesus , Tardon Adonina

Background: Childhood obesity is a serious public health concern. To identify risk for obesity in early childhood is important.Objective: To analyse association of pre-pregnancy maternal weight with BMI and metabolic profile at 4 years.Methods: 2604 pregnant mothers and 1960 children from the Spanish population-based cohort study Environment and Childhood (INfancia y Medio Ambiente) Project (INMA). Research protocol was approved by...

hrp0082p1-d1-142 | Growth | ESPE2014

Parental Gonadal Mosaicism for a BRAF Mutation in Cardiofaciocutaneous Syndrome

Geoghegan Sarah , Morrissey Rose , Shorto Jeniffer , Ramsden Simon , O'Riordan Stephen , Green Andrew , O'Connell Susan

Background: Cardiofaciocutaneous syndrome (CFCS) is a rare autosomal dominant (AD) condition characterized by cardiac abnormalities, a distinctive craniofacial appearance and short stature. Endocrine manifestations include GH deficiency and precocious puberty. CFCS is part of the RASopathy group including Noonan, LEOPARD, and Costello syndromes. The four associated genes are BRAF (~75%), MAP2K1 and MAP2K2 (~25%), and KRAS (<2%). Most individuals represent new sporadic muta...

hrp0082p2-d3-447 | Growth Hormone (2) | ESPE2014

Efficacy, Adherence, and Cost Study According to Pathology and Treatment Devices in Children Treated with GHRH

Diez-Lopez Ignacio , Sarasua-Miranda Ainhoa , Elizalde Miriam Miriam , Lorente-Blazquez Isabel , Minguez Cristina , Martinez-Martinez Carlos

Background: Currently there are three distinct groups of GH devices: single dose (JM), preloaded pen/vial (VM) systems and electronic devices (DE) autoinjector systems. The choice could determine a greater or lesser adherence and thus influence the final treatment efficacy.Objective: Comparison of the therapeutic efficacy as measured by growth rate (VC), IGF1 as a function of various clinical variables, indicating GH and device used.<p class="abstext...

hrp0082p2-d1-589 | Thyroid | ESPE2014

No Difference in Cognitive Development of Young Adults and Adolescents Affected by Congenital Hypothyroidism Compared to Their Sibling Controls Despite High Dose L-Thyroxin Treatment

Aleksander Paulina , Blankenstein Oliver , Gruters Annette , Krude Heiko

Background: An early diagnosis and treatment based on neonatal screening offers a normal cognitive development in patients affected with congenital hypothyroidism (CH). However, several studies within cohorts of young adults have shown a still existing difference compared to control groups of up to eight IQ points. Moreover it has been claimed recently that a high L-T4 dose with subsequent episodes of overtreatment results in less favourable IQ outcom...