hrp0097p1-445 | Fat, Metabolism and Obesity | ESPE2023

Impact of early-life overweight/obesity trajectory on insulin resistance at age 8: a prospective cohort study

Jin Lee Hye , Jeong Lee Yun , Lim Youn-Hee , Hong Yun-Chul , Ho Shin Choong , Ah Lee Young

Objective: To investigate whether the early-life overweight/obesity trajectory from ages 2, 4, to 8 affects insulin resistance (IR) in 8-year-old prepubertal children.Methods: From the Environment and Development of Children (EDC) cohort, 262 prepubertal children (147 boys and 115 girls) who visited Seoul National University Children’s Hospital for anthropometric measurements at ages 2, 4 and 8 were included. At ag...

hrp0097p1-464 | Fat, Metabolism and Obesity | ESPE2023

Occurrence of cardiovascular risk factors in Polish children and adolescents with severe obesity. Preliminary results of the Polish-German study project on severe early-onset obesity

Stępniewska Anna , Szczudlik Ewa , Bik-Multanowski Mirosław , Brandt Stephanie , Flehmig Bertram , Małecka-Tendera Ewa , Mazur Artur , Petriczko Elżbieta , B. Ranke Michael , Zachurzok Agnieszka , Wójcik Małgorzata

It is estimated that 1-5% of children and adolescents in Europe suffer from severe obesity (corresponding to an adult BMI > 40 kg/m2). However, in risk stratification, the occurrence of metabolic complications is more important than BMI itself. The study aimed to assess the occurrence of cardiovascular risk factors in a cohort of children and adolescents with severe obesity.Patients and Methods: The analysis included 140 patients (75 ...

hrp0097p1-287 | Fetal, Neonatal Endocrinology and Metabolism | ESPE2023

Mutations in exon 28 of ABCC8 gene in Egyptian patients with congenital hyperinsulinism

Abdelghaffar Shereen , Madani Hanan , Ashour Mohammed , Ahmed Yomna , Abdou Maryz

Background: Congenital hyperinsulinism in infancy (CHI) is the most frequent cause of persistent hypoglycemia in infants. The most common and severe form of monogenic CHI is caused by inactivating mutations in ABCC8 and KCNJ11 genes located on chromosome 11p15.1. On the ABCC8 gene; previous studies have shown that mutations were reported to be mostly localized in exon 28. There is no sufficient research in Egyptian population about different mutations in conge...

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...

hrp0097p1-295 | GH and IGFs | ESPE2023

Sex steroid priming decreases the frequency of divergent results between spontaneous and stimulated GH tests

Lennartsson Otto , Nilsson Ola , Lodefalk Maria

Introduction: The diagnosis of growth hormone (GH) deficiency (GHD) is complicated by the low specificity of GH testing, especially in children before and during early pubertal stages. Sex steroid priming reduces false positive results in pre- and early pubertal children. However, only a small number of studies have assessed its efficacy in improving the diagnostic accuracy of GHD investigations.Aim: To evaluate the effe...

hrp0097p1-302 | GH and IGFs | ESPE2023

Influencing Factors on Selection of Initial Treatment Dose in Children Diagnosed with Isolated Growth Hormone Deficiency

Mert Erbaş İbrahim , Uyar Nilüfer , Nalbantoğlu Özlem , Özkan Behzat

Background: Although it has been recommended to start treatment in the range of 22-35 µg/kg/day in growth hormone (GH) deficiency, individualized dose selection is more preferred for those patients instead of a fixed dose. However, there is no consensus as to which dose should be started for which patient. In this study, we aimed to reveal the factors that affect the clinicians' preferences in the selection of the starting dose of GH treatment.<...

hrp0097p1-491 | GH and IGFs | ESPE2023

Priming with sex steroids increases the specificity of GH tests in the diagnosis of “True” isolated growth hormone deficiency

Vuralli Dogus , Ozon Alev , Gonc Nazli

Background and Aim: Priming with sex steroids before growth hormone (GH) stimulation tests is considered to increase the specificity of the GH stimulation tests, however, its use in the diagnosis of growth hormone deficiency (GHD) is still controversial. Purpose of this study is to analyze efficacy of sex steroid priming in the diagnosis of GHD.Methods: The study comprised 115 peripubertal boys who were diagnosed with is...

hrp0097p1-113 | Growth and Syndromes | ESPE2023

Etiology of extreme tall stature and auxological cues at presentation

Toivakka Essi-Maria , Kärkinen Juho , J. Miettinen Päivi , Raivio Taneli , Hero Matti

Background: Tall stature is a frequent concern in pediatric endocrine clinics. However, no representative reports addressing etiology and auxological diagnostic cues at specialized healthcare presentation currently exist. We report the etiology of extreme tall stature and describe auxological cues indicative of a growth disorder.Methods: We identified 145 subjects (girls/boys: 78/67) with extreme tall stature from our in...

hrp0097p1-127 | Growth and Syndromes | ESPE2023

Effects and safety of growth hormone (GH) treatment in 6 children with pycnodysostosis

Renes Judith , Sas Theo , Clement-de Boer Agnes , Zwaveling-Soonawala Nitash , van der Kaay Danielle , Hokken-Koelega Anita

Background: Pycnodysostosis is a very rare autosomal recessive skeletal dysplasia caused by cathepsin K deficiency. It is characterized by extreme short stature resulting in an adult height in males typically <150 cm and in females <134 cm. Bone-fragility and frequent fractures are present. There are few case-reports on the effects of GH treatment.Objective: To evaluate the effect and safety of GH in 6 patients wit...

hrp0097p1-319 | Growth and Syndromes | ESPE2023

Progressively impaired prepubertal growth in children with APECED

Saari Viivi , Alanko Venla , Holopainen Elina , Mäkitie Outi , Laakso Saila

Background: Autoimmune-polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED; APS-1) is a disorder arising from mutations in the autoimmune regulator (AIRE) gene, that lead to the development of circulating autoreactive T-cells and deficiency of regulatory T-cells. Multiple manifestations, such as primary adrenocortical insufficiency, and their treatments may disturb growth in children with APECED. However, no previous data on prepubertal growth in child...