hrp0095lb19 | Late Breaking | ESPE2022

Early adrenal activation in boys with Cystic Fibrosis may explain impaired final height

Tamer Gizem , GM Arets Hubertus , K van der Ent Cornelis , M van Santen Hanneke , J van der Kamp Hetty

Background: Rapid BMI increase in early childhood was found to be a contributing factor for impaired final height in boys with Cystic Fibrosis (CF). This may be explained by early adrenarche triggering an accelerated bone maturation resulting in a compromised growth spurt during puberty, comparable to children with SGA. In healthy Dutch boys, adrenarche starts from the age of 9 years with DHEAS ≥ 1 µmol/L. In Dutch girls, the onset of adrenarche is fr...

hrp0097t8 | Section | ESPE2023

Design and Objectives of the Acorn Study: A Non-Interventional Study Evaluating Long-term Safety in Achondroplasia Patients Treated with Vosoritide

M Pimenta Jeanne , Cohen Shelda , Mukherjee Swati , Fettes Fiona , Jayaram Kala , Lausch Ekkehart , Mallya Usha G. , Min Yang , Caroline Huber , Cala Mary Lynn , Ali Greatsinger , Jeremy Pomeroy , Haqq Andrea M.

Introduction: Achondroplasia is caused by a pathogenic mutation in the FGFR3 gene, leading to impaired endochondral bone growth and multiple medical complications. Vosoritide, a modified recombinant human C-type natriuretic peptide (rhCNP), was approved by the European Medicines Agency (EMA) in August 2021 for treating genetically confirmed achondroplasia in patients aged ≥2 years until closure of epiphyses. Acorn is the first treatment-based registry for a...

hrp0097p1-255 | Fat, Metabolism and Obesity | ESPE2023

Comparison of triglyceride-glucose index and HOMA-IR for predicting severity of nonalcoholic fatty liver disease in Korean children and adolescents

Young Kim Eun , Tae Hwang Il

Background: Triglyceride-glucose (TyG) index is known as a reliable insulin resistance surrogate marker to identify non-alcoholic fatty liver disease (NAFLD). Ultrasonographic fatty liver indicator (US-FLI) is proposed as a non-invasive, semi-quantitative method for predicting hepatitis in patients with NAFLD and accurately identifies histological severity. Few studies have compared the TyG index to homeostasis model assessment of insulin resistance (HOMA-IR) ...

hrp0098fc1.3 | Diabetes and Insulin | ESPE2024

A Non-Invasive Diagnostic Tool for Early Detection of Microvascular Alterations in Children with Type 1 Diabetes Mellitus: Nail-Fold Capillaroscopy

Akın Kağızmanlı Gözde , Aydın Tuncay , Yüksek Acinikli Kübra , İşgüder Rana , Kızıldağ Karabacak Zehra , Demir Korcan , Böber Ece , Erbil Ünsal Şevket , Abacı Ayhan

Background: Nail-fold capillaroscopy (NFC), a non-invasive tool that can detect microvascular changes, has recently gained popularity in the diabetes field as a warning sign before the development of diabetes-related complications.Objective: We aimed to assess capillary microarchitecture in children with type 1 diabetes mellitus (T1DM) by comparing them with a healthy control group.Subjects...

hrp0098fc2.4 | Bone, Growth Plate and Mineral Metabolism | ESPE2024

Analysis of baseline data from the SUNFLOWER longitudinal, observational cohort study of patients with XLH: relationship between various complications and QOL

Namba Noriyuki , Ito Nobuaki , Michigami Toshimi , Gyung Kang Hee , Kubota Takuo , Miyazaki Osamu , Shintani Ayumi , Kabata Daijiro , Nishida Yayoi , Fukumoto Seiji , Ozono Keiichi

X-linked hypophosphatemia (XLH) is characterized by excess FGF 23, hypophosphatemia, skeletal deformities, and growth impairment. Due to the lack of large-scale and long-term observational studies, not enough evidence has been accumulated to gain consensus on optimal care. A longitudinal, observational cohort study (SUNFLOWER) was initiated in Japan and South Korea to clarify the course of XLH; delineate its physical, mental, and financial burdens; and collect information on t...

hrp0098p3-240 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2024

Delayed puberty: etiological and gender characteristics

Latyshev Oleg , Velmakina Nadejda , Kiseleva Elena , Okminyan Goar , Romaikina Daria , Samsonova Lubov

Delayed puberty is a common condition defined as the lack of sexual maturation by an age ≥ 2 sd above the population mean.Materials and Methods: to study the structure of the delayed puberty (DP), 96 medical records of patients who were examined in the endocrinology department between 2020 and 2024 were selected: 77 boys (14.8 ± 1.23 years), 19 girls (14.5 ± 2.03 years) by querying the electronic information system of the medica...

hrp0095p1-250 | Diabetes and Insulin | ESPE2022

The Metabolic Syndrome is frequent in Children and Adolescents with Type 1 Diabetes Compared to Healthy Controls

Barrett Mørk Freja , Otto Broby Madsen Jens , Ascanius Pilgaard Kasper , Kryger Jensen Andreas , Klakk Heidi , Tarp Jakob , Bugge Anna , Heidemann Malene , Van Hall Gerrit , Pociot Flemming , Wedderkopp Niels , Johannesen Jesper

Background: There is a rise in overweight and obesity among children and adolescents with type 1 diabetes (T1D) in parallel with a rise in the metabolic syndrome (MS) among children and adolescents in general.Objective: The aim of the study was to describe the prevalence and characteristics of the MS in children and adolescents with T1D compared to their healthy counterparts.Design and Sett...

hrp0095p1-233 | Diabetes and Insulin | ESPE2022

Effects of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-Modulating Therapy on Glucose Metabolism in Cystic Fibrosis Patients

Yesquen Pamela , Campos Ariadna , Mogas Eduard , Yeste Diego , Gartner Silvia , Clemente María

Introduction: CFTR-modulating therapy aims to restore CFTR function. It improves lung function and quality of life in CF patients however, its effects on glucose metabolism are not yet well defined.Methodology: Retrospective and longitudinal study.Inclusion criteria: patients with CF genetic diagnosis ≥10 years old on modulator therapy (ivacaftor + lumacaftor or ivacaftor + teza...

hrp0097fc14.4 | Late Breaking | ESPE2023

Improvement in insulin sensitivity and glucose metabolism in adolescents with obesity treated with once-weekly semaglutide 2.4 mg: a secondary analysis of the STEP TEENS trial

Arslanian Silva , Goldman Bryan , Gies Inge , M. Harder-Lauridsen Nina , Karlsson Tobias , Kelly Aaron , Wabitsch Martin , Weghuber Daniel

Obesity in adolescents is associated with increased risk of prediabetes and type 2 diabetes (T2D) and long-term complications in adulthood. Data regarding the effects of anti-obesity medications on glycaemic outcomes in adolescents are sparse. STEP TEENS (NCT04102189), a phase 3a, double-blind, placebo-controlled randomised trial in adolescents 12 to <18 years of age with obesity demonstrated that once-weekly subcutaneous semaglutide 2.4 mg provided a greater percentage red...

hrp0097p1-433 | Diabetes and Insulin | ESPE2023

Effects of triple cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy on glucose metabolism in cystic fibrosis patients

Yesquen Pamela , Campos Ariadna , Mogas Eduard , Yeste Diego , Gartnet Silvia , Clemente María

Introduction: Triple CFTR modulator therapy has been shown to improve lung function and quality of life in patients with at least one F508del mutation in the CFTR gen. However, effects on glucose metabolism are not yet well defined. This paper aims to describe the effects of this treatment on glucose metabolism in CF patients.Methodology: Ambispective study.Inclusion criteria: pati...