hrp0095fc4.2 | Fat, Metabolism and Obesity | ESPE2022

Positive effects of a structured pre- and post-bariatric surgery program on follow-up rate and supplement intake in adolescents and youths- Results from the German YES cohort

Brandt Stephanie , Schirmer Melanie , Kleger Pauline , von Schnurbein Julia , Holle Rolf , Holl Reinhard W. , Hebebrand Johannes , Wiegand Susanna , Wabitsch Martin

Introduction: Until recently, bariatric surgery (BS) has been the only option for clinically meaningful weight reduction for adolescents and youth with extreme obesity. Low participation rate in follow-up examinations and low supplement intake after BS in adolescents has been described in literature (PMID: 24048144, 25078533). We developed a structured pre- and post-bariatric surgery program in order to improve follow-up rate and supplement intake in adolescen...

hrp0086rfc9.6 | Pathophysiology of Disorders of Insulin Secretion | ESPE2016

Emerging Pitfalls of Etiological Diagnosis of Diabetes in Children and Adolescents? Analysis of a French Cohort of 310 Recent-Onset Cases

Bismuth Elise , Plat Helene , Chevenne Didier , Chantelot Christine Bellane , Carel Jean-Claude , Tubiana-Rufi Nadia

Background: A diagnosis of diabetes in children used to mean type 1 diabetes (T1D) and lifelong insulin therapy. However, over the last decades the spectrum of diabetes has widened and even if auto immunity remains the most prevalent etiology, confounding factors and overlap with other causes of diabetes types sometimes make a revision of the initial diagnosis necessary.Objective and hypotheses: To classify diabetes etiologies in youth with diabetes usin...

hrp0086p2-p67 | Adrenal P2 | ESPE2016

Successful Medical Management of Severe Neonatal Cushing Syndrome with Metyrapone, Guided by Mass Spectrometry Monitoring

Poidvin Amelie , Storey Caroline , Martinerie Laetitia , Braun Karine , Lahlou Najiba , Leger Juliane , Carel Jean-Claude

Background: Neonatal Cushing syndrome is a rare and severe condition, mostly associated with the McCune-Albright (MCA) syndrome. Management options include medical treatment (with ketoconazole or metyrapone resulting in 11-beta-hydroxylase blockade) and radical treatment with bilateral adrenalectomy. Spontaneous regression in late infancy has been reported.Objective and hypotheses: To report on the outcome of a 14 month-old girl with severe neonatal Cush...

hrp0086p1-p563 | Perinatal Endocrinology P1 | ESPE2016

Hyponatremia in Infants Under 100 Days Old: Frequently Overlooked and Multifactorial

Storey Caroline , Dauger Stephane , Baud Olivier , Deschenes Georges , Carel Jean-Claude , Martinerie Laetitia

Background: Hyponatremia is one of the most common electrolyte disorders in hospitalized children and early diagnosis and management are crucial to prevent morbidity and mortality. Because of the physiological resistance to aldosterone under 3 months of age, the mechanisms leading to hyponatremia are often misunderstood.Objective and hypotheses: To assess the prevalence of hyponatremia in hospitalized infants younger than 100 days and evaluate the mechan...

hrp0086p1-p724 | Pituitary and Neuroendocrinology P1 | ESPE2016

Estimating the Psychosocial Impact of Idiopathic Central Precocious Puberty (ICPP) in Girls Aged 6 to 8 Years: A Qualitative Study

Vasse Camille , Teixeira Maria , Paulsen Anne , Malivoir Sabine , Samara-Boustani Dinane , Carel Jean-Claude , Simon Dominique

Background: Emotional and behavioural problems are often used in support of GnRH agonists therapy in girls with early pubertal timing. However, there is little evidence to show that CPP leads to psychological distress and whether treatment is associated with improved psychological outcome.Objective and hypotheses: The objective of this qualitative study was to explore the psychosocial impact of ICPP in recently diagnosed girls.Meth...

hrp0086p1-p745 | Pituitary and Neuroendocrinology P1 | ESPE2016

Constitutional Delay of Puberty or Hypogonadotropic Hypogonadism: Diagnostic Value of Inhibin B and AMH Measurements

Istanbullu Sibel , Lahlou Najiba , Chevenne Didier , Leger Juliane , Carel Jean-Claude , Martinerie Laetitia

Background: Boys with delayed puberty represent one of the main cause for pediatric endocrine referrals. Although the majority of them have constitutional delay of growth and puberty (CDGP), it is important to identify isolated hypogonadotropic hypogonadism (IHH) for optimal management.Objective and hypotheses: The aim of the study was to evaluate the usefulness of inhibin B and AMH as biological markers for distinguishing between CDGP and IHH.<p cla...

hrp0086p2-p772 | Pituitary and Neuroendocrinology P2 | ESPE2016

Brain MRI in Evaluation of Endocrine Diseases of Childhood: Causal and Incidental Lesions

Selbonne Emilie , Martinerie Laetitia , Leger Juliane , Elmaleh Monique , Sebag Guy , Carel Jean-Claude

Background: Brain MRI is an essential tool in the diagnosis of neuroendocrine disorders and aims at detecting anatomical abnormalities and tumors. However, it may lead to the identification of unrelated or questionably related abnormalities in the hypothalamic-pituitary region and/or the rest of the brain parenchyma.Objective and hypotheses: To establish the prevalence of causal lesions on brain MRI of children evaluated for hypopituitarism, assess the f...

hrp0086p2-p841 | Syndromes: Mechanisms and Management P2 | ESPE2016

Safety and Efficacy of Growth Hormone (GH) in Combination with the Gonadotrophin Releasing Hormone Agonist Leuprorelin in Pubertal Children with Idiopathic Short Stature

Benabbad Imane , Tauber Maite , Rosilio Myriam , Paris Emmanuel , Berggren Lovisa , Patel Hiren , Carel Jean-Claude

Background: Due to delays in diagnosis of Idiopathic Short Stature (ISS), puberty has often started when Growth Hormone (GH) is initiated.Objective and hypotheses: To compare GH+leuprorelin (GH+L) with GH alone in patients with ISS and at Tanner puberty stage 2 or 3. We hypothesised that co-administration of GH+L for 2–3 years would delay puberty, prolong GH efficacy and increase adult height.Method: A phase 3, randomised, ope...

hrp0092p2-251 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology | ESPE2019

3β-HSD2 Deficiency Due to Compound Heterozygosity of a Missense Mutation (p.Thr259Met) and a Frameshift Deletion (p.Lys273ArgFs*7) in an Under-Virilized Infant Male with Salt Wasting

Leka-Emiri Sofia , Taibi Ludmia , Fotinou Aspasia , Vlachopapadopoulou Elpis , Michalacos Stefanos , de Roux Nicolas

Objectives: To describe clinical, hormonal and genetic findings of an under-virilized infant male, 13 month old, of afghani origin, presented at the emergency department with salt-wasting adrenal crisis.Case report: The patient, a 13 month old male infant, presented at the emergency department with complaints of vomiting and fever. Physical examination was significant for severe dehydration along with perineal hypospadia...

hrp0089fc13.4 | Pituitary, Neuroendocrinology and Puberty 2 | ESPE2018

The Kallman Syndrome Gene Product is Specifically Expressed in ACTH-Expressing Cells and Dysplays Sexual Dimorphism Expression in Human Fetal Pituitary

Guimiot Fabien , Bonnard Adeline , Soussi-Yaniscostas Nadia , Schnoll Carol , de Roux Nicolas

Kallman syndrome is defined by the association of anosmia due to an agenesis of the olfactory bulbs and hypogonadotropic hypogonadism due to a GnRH deficiency which is currently explained by a deficit of GnRH neuron migration from the olfactory placode toward the hypothalamus. In fact, the X-linked form of KS is due to loss of function mutations in ANOS1 which encodes an extracellular protein (ANOSMIN) interacting with cell membrane heparin sulfate proteoglycans but a...