hrp0095p2-69 | Diabetes and Insulin | ESPE2022

Impact of β-cell function and glucose effectiveness on the development of type 2 diabetes mellitus in obese European children and adolescents

Ellen Kratzer Sophie , Vogt Josef , Kohlsdorf Katja , von Schnurbein Julia , Wabitsch Martin , Denzer Christian

Objectives: Despite high prevalence rates of childhood obesity, youth-onset T2DM occurs in a comparably lower incidence in Europe than in other world regions. Available data from cohorts of obese children and adolescents living in the US suggest a parallel decline of insulin sensitivity and β-cell function as key factors in the pathophysiology of early-onset T2DM. If these results can be applied to European children and adolescents is currently unknown.</...

hrp0095p2-98 | Fat, Metabolism and Obesity | ESPE2022

Effects of long-term metreleptin replacement therapy on weight and comorbidities in a patient with bioinactive leptin

Zorn Stefanie , von Schnurbein Julia , Kohlsdorf Katja , Brandt Stephanie , Wabitsch Martin

Introduction: Congenital leptin deficiency can be treated with the human leptin analogon metreleptin as first reported in 1999. To date, the long-term effects (> 1 year) of metreleptin treatment on anthropometry and comorbidities have only been described in 8 patients with leptin deficiency. Here, we present the long-term effects of metreleptin substitution on weight and comorbidities in a patient with bioinactive leptin.Metho...

hrp0092rfc7.2 | Diabetes and Insulin Session 2 | ESPE2019

β-Cell Function and Glucose Effectiveness in the Development of Impaired Fasting Glucose in Obese European Children and Adolescents

Denzer Christian , Kohlsdorf Katja , von Schnurbein Julia , Wabitsch Martin , Vogt Josef

Objectives: Impaired fasting glucose (IFG) is a risk factor for the development of type 2 diabetes in adults. In obese children and adolescents, IFG and impaired glucose tolerance constitute distinct prediabetic stages, which do not necessarily coexist. Pathophysiological mechanisms leading to IFG in children have not been fully elucidated. Available data from cohorts of obese adolescents living in the US suggest a concurrent worsening of insulin sensitivity a...

hrp0089fc12.3 | Diabetes and Insulin 2 | ESPE2018

Impact of Insulin Sensitivity and β-cell Function on the Development of Impaired Glucose Tolerance (IGT) in Obese European Children and Adolescents

Denzer Christian , Vogt Josef , Kohlsdorf Katja , von Schnurbein Julia , Wabitsch Martin

Objectives: Compared to the US, prevalence rates of T2DM in obese children are significantly lower in European countries. Data from cohorts of obese children living in the US suggest a concurrent worsening of insulin sensitivity and ß-cell function over the spectrum of glucose tolerance. If these results can be applied to European populations is currently unknown.Methods: A combination of our novel method for mathematical modelling ...

hrp0094p2-195 | Fat, metabolism and obesity | ESPE2021

Assessment of Hyperphagia in Patients with Monogenic Obesity

Zorn Stefanie , von Schnurbein Julia , Schirmer Melanie , Kohlsdorf Katja , Brandt Stephanie , Wabitsch Martin ,

Introduction: Besides an excessive, early-childhood weight gain, hyperphagia is the key symptom in patients with monogenic obesity. However, the assessment of hyperphagia is still challenging. Here, we applied the hyperphagia questionnaire developed for patients with Prader-Willi-Syndrome (PWS) to assess the severity of hyperphagia in patients with monogenic obesity.Methods: Pediatric patients with biallelic pathogenic l...

hrp0095p1-268 | Fat, Metabolism and Obesity | ESPE2022

Preliminary evidence for a strong effect of leptin on mood and behaviour in patients with congenital leptin deficiency

von Schnurbein Julia , Remy Miriam , Brandt Stephanie , Manzoor Jaida , Kohlsdorf Katja , Mahmood Saqib , Hebebrand Johannes , Wabitsch Martin

Purpose: States of starvation are characterized by reduced physical activity and social withdrawal. The reduction of leptin levels during starvation has been proposed to be a mediator of these changes, therefore we aimed to ascertain if leptin substitution in patients with congenital leptin deficiency (CLD) can increase physical activity and improve mood.Methods: Seven CLD patients were filmed prior to and after short te...

hrp0092rfc11.5 | Pituitary, Neuroendocrinology and Puberty Session 2 | ESPE2019

IGF-1 Serum Concentrations and Growth in Children with Congenital Leptin Deficiency (CLD) Before and After Replacement Therapy with Metreleptin

Beghini Marianna , von Schnurbein Julia , Körber Ingrid , Brandt Stephanie , Kohlsdorf Katja , Vollbach Heike , Lennerz Belinda , Denzer Christian , Wabitsch Martin

Background: Leptin, primarily secreted by adipocytes, is a pivotal signal of the body's energy status and exhibits pleiotropic effects. Homozygous mutations in the leptin gene which result in defective synthesis, release or bioactivity, cause intense hyperphagia and early-onset severe obesity, along with multiple metabolic, hormonal, and immunological abnormalities. In vitro and animal model studies suggest that leptin plays a role in linear growth. So far...