hrp0092fc6.5 | Bone, Growth Plate and Mineral Metabolism Session 2 | ESPE2019

No Change in Bone Density During 6 Months Off GH in Adolescents with Severe GHD at Near-Adult Height

Schweizer Roland , Donner Julia , Becker Bettina , Bauer Jana Leonie , Binder Gerhard

Objective: Re-testing of childhood-onset GHD needs discontinuation of GH treatment at near-adult height. We recently reported significant changes of body composition as a consequence of severe GHD during this time period.Aim: Does a 6 month interruption of GH treatment decrease bone quality significantly in patients with severe GHD of adolescence?Patients and Methods: In 90 patient...

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...

hrp0092lb-3 | Late Breaking Posters | ESPE2019

Insights into the Regulation of Androgen Biosynthesis from Males with Congenital Hypogonadotropic Hypogonadism: Quantification of Bioactive Steroid Hormones Reveals Differences Between Gonadotropin Replacement and Testosterone Replacement

Rohayem Julia , Holterhus Paul-Martin , Kliesch Sabine , Nieschlag Eberhard , Zitzmann Michael , Kulle Alexandra

Background: In males with congenital hypogonadotropic hypogonadism (CHH), LH/FSH stimulation of gonads is deficient. In clinical practice, two hormone replacement strategies are employed to induce and maintain virilisation: Treatment with testosterone and gonadotropin replacement with hCG/rFSH.Objective: To delineate the role of gonadotropins in pathways of male androgen biosynthesis and to thereby better define the gona...

hrp0092p2-110 | Fat, Metabolism and Obesity | ESPE2019

Pubertal Milestones and Related Hormonal Changes Among Children with Obesity

Stein Robert , Kempf Elena , Gesing Julia , Stanik Juraj , Kiess Wieland , Körner Antje

Background and Objective: Obesity is known to affect pubertal timing. However, existing data are still controversial, observing either delayed or accelerated pubertal onset, especially among boys. Herein, we evaluated pubertal milestones and underlying hormonal changes between lean and obese children.Material and Methods: We examined 13,484 events from 4,855 lean (BMI SDS <1.28) and 1,983 obese (BMI SDS > 1.88) c...

hrp0092p3-269 | Late Breaking Abstracts | ESPE2019

IGSF1 Mutation: Treatment in the Absence of Symptoms?

Castets Sarah , Vergier Julia , Godefroy Alice , Saveanu Alexandru , Collignon Patrick , Brue Thierry , Reynaud Rachel

Introduction: Congenital central hypothyroidism is a rare pathology, whose molecular origin has been identified more frequently since discovery of the role of IGSF1. The natural evolution of central hypothyroidism in patients with mutations is not well known however.Case report: A male infant born at term with a normal birth weight received thyroid function tests in the neonatal period because of symptoms of bra...

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 ...

hrp0089p1-p008 | Adrenals and HPA Axis P1 | ESPE2018

Impact of Puberty on Final Height in Children and Adolescents with Congenital Adrenal Hyperplasia (CAH)

Rohayem Julia , Schreiner Felix , Riedl Stefan , Voss Egbert , Wolf Johannes , Grasemann Corinna , Fink Katharina , Mohnicke Klaus

Introduction: An optimized replacement regimen with glucocorticoids and mineralocorticoids in subjects with congenital adrenal hyperplasia (CAH) aims at preventing life-threatening salt wasting and adrenal crises, virilization and pubertal precocity, and at enabling normal linear growth.Aims: We investigated puberty and its impact on final height in children and adolescents with CAH.Patients and Methods: In a cohort of post-pubesce...

hrp0089p3-p025 | Adrenals and HPA Axis P3 | ESPE2018

Congenital Adrenal Hyperplasia due to a Rare Homozygous Mutation R483P in the CYP21A2 Gene and Coexisting Growth Hormone Deficiency

Akulevich Natallia , Makarava Yulia , Boiko Julia , Mirabelli Silvestro , Wasniewska Malgorzata , DeLuca Filippo

In CAH due to 21-OH deficiency, GH treatment combined to GC and MC replacement is still considered to be experimental. We present a patient who has benefited from such treatment. A baby girl was born in term with clitoromegaly and manifested with salt loose at neonatal period. Low serum morning cortisol and sodium with high potassium and 17-OH levels were found resulted in the clinical diagnosis of CAH. The karyotype was 46,XX. Prednisolone and DOXA, the only available hormone...

hrp0089p1-p037 | Bone, Growth Plate &amp; Mineral Metabolism P1 | ESPE2018

Hypercalcaemia after Treatment with Denosumab in Children: Bisphosphonates as an Option for Therapy and/or Prevention?

Sydlik Carmen , Weissenbacher Claudia , Roeb Julia , Roland Durr Hans , Bechtold-Dalla Pozza Susanne , Schmidt Heinrich

Background: Pharmacologic options for treatment of osteolytic diseases especially in children are limited. Although not licensed for use, denosumab, a fully humanized antibody to RANKL, is used in children and shows good effects. Among others, one indication are giant cell tumors of the bone. Yet, there are reports of severe hypercalcemia after stop of denosumab, an adverse effect which is rarely seen in adults.Case reports: Four patients, aged 6, 13 and...

hrp0089p1-p046 | Diabetes &amp; Insulin P1 | ESPE2018

Phenotypes of Diabetes and Determinants of Glycemic Control and Diabetes Complications in Haitian Youth Living in Haiti

Dumas Marie-Pier , Sainvil Michele , Altenor Kelty , von Oettingen Julia Elisabeth

Background: In non-Caucasian youth residing in low-income settings, risk of mortality and rates of diabetes complications are substantially higher and clinical phenotypes may be distinct.Objectives: To assess the clinical presentation, glycemic control, and chronic complications of diabetes in Haitian youth residing in Haiti.Methods: Retrospective review between 01/2013–03/2018 of youth 0-25 years with diabetes followed at a c...