hrp0084p2-439 | Gonads | ESPE2015

Markers of Fertility and Quality of Life in Adolescents with Chronic Endocrine Diseases at the Time of Transition from Paediatric to Adult Care

Mittnacht Janna , Choukair Daniela , Klose Daniela , Inta Ioana , Bettendorf Markus

Background: 25–50% of paediatric patients with chronic endocrine diseases are lost for follow-up in adult care.Aims and objectives: A standardised medical and psychological work-up to identify disease specific morbidity and to comprehend quality of life in adolescents with chronic endocrine diseases at the time of transition from paediatric to adult care.Methods: The quality of life (DISABKIDS1 and KIDSCREEN2</...

hrp0092p1-365 | GH and IGFs (2) | ESPE2019

De Novo Formation of Neutralizing IGF-I Antibodies During rhIGF-1 Treatment in a Girl with IGFALS Deficiency as Distinct Adverse Event Interfering with Growth Promotion

Mittnacht Janna , Breil Thomas , Choukair Daniela , Duffert Christin , Hwa Vivian , Rosenfeld Ron , Bettendorf Markus

Background: IGFALS deficiency is a rare cause of GH insensitivity (GHI). We report a German girl with short stature who was born as 2nd child at 40 weeks of gestation. Her Caucasian parents were unrelated and healthy (target height 168 cm, SDS 0.2). She was born appropriately sized for gestational age (49 cm, SDS -1.2; 2950 g, SDS-1.2). Height (104.8 cm; SDS -2.94) and height velocity (5.1 cm / year; SDS -1.67) were reduced at the age of 6 years.</p...

hrp0092p2-269 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology | ESPE2019

Pseudo-Precocious Puberty in Children Triggered by Incidental Transdermal Contamination with Topical Sex Steroids Through Parents

Yakovenko Vira , Choukair Daniela , Duffert Christin , Mittnacht Jana , Klose Daniela , Bettendorf Markus

Introduction: Pseudo-precocious puberty may be triggered by either endogenous or exogenous sex steroids. Accidental ingestion of contraceptives, anabolic steroids or life style products containing sex steroids as well as transdermal transmission of topical sex steroids may cause early onset of secondary sexual development.Objective: To report the clinical course of incidental contamination with topical sex steroids in ch...

hrp0086p1-p722 | Pituitary and Neuroendocrinology P1 | ESPE2016

Role of the Metabotropic mGlu5 Glutamate Receptor in the Initiation of Puberty and Reproduction in Female Mice

Inta Ioana , De Angelis Roberto , Domonkos Emese , Lankisch Katja , Boehm Ulrich , Gass Peter , Bettendorf Markus

Background: The neuroendocrine mechanisms of the initiation of puberty are still incompletely deciphered. Accumulating data indicate a main role of the glutamate system in regulating hypothalamic GnRH. However, the underlying neurobiological mechanisms are yet poorly investigated. Mice lacking metabotropic mGlu5 receptors (mGluR5) show sever unexplained infertility.Objective and hypotheses: We aimed in the present study to analyze the specific role of mG...

hrp0082p1-d2-151 | Growth (1) | ESPE2014

Sequential Measurements of IGFI Serum Concentrations in Patients With Severe Primary IGFI Deficiency (SPIGFD) and Growth Failure Treated With Recombinant IGFI (Increlex®)

Bettendorf Markus , Kapelari Klaus , Kneppo Carolin , Muller Hermann L , Schnabel Dirk , Wolfle Joachim

Introduction: Increlex® was approved as an orphan drug for treatment of growth failure in children and adolescents with SPIGFD in 2007 with relatively little data available. Therefore sequential measurements of serum IGFI, glucose, insulin and potassium were performed in SPIGFD patients treated with Increlex® to evaluate their significance in safety and efficacy.Design: Blood samples were taken after meals before and 30, 6...

hrp0082p2-d1-269 | Adrenals &amp; HP Axis | ESPE2014

Congenital Adrenal Hyperplasia Caused by 11β-hydroxylase-Deficiency as a Rare Differential Diagnosis of Precocious Pseudo-Puberty and Hypertension

Yakovenko Vira , Simon Anke , Schulze Egbert , Grulich-Henn Jurgen , Bettendorf Markus

Background: 11β-hydroxylase deficiency (HD) represents a rare cause of congenital adrenal hyperplasia (CAH) characterized by glucocorticoid deficiency and excess of mineralcorticoids and androgens. The CYP11B1 gene on chromosome 8q22 encodes the 11β-hydroxylase.Objective: To report clinical, biochemical and molecular features of patients with 11β-HD.Results: Four male patients of Turkish descent with 11β-HD incl...

hrp0084p3-865 | Fat | ESPE2015

Waist Circumference to Body Height is a Suitable Measure of Cardiovascular Risk in Overweight and Obese Children

Yakovenko Vira , Teufel Ulrike , Henn Laura , Bettendorf Markus , Hoffmann Georg F , Grulich-Henn Juergen

Background: Several methods have been used to evaluate the risk of cardiovascular diseases in obese children. Both BMI and waist-to-hip ratio were suggested as risk factors. However, they did not prove to estimate the risk for cardiovascular events in adulthood. Recent studies suggest that the ratio of waist circumference to body height (WHtR) is a more reliable predictor for cardiovascular risk in 6-10-year old children (Kuba et al. 2013).Objec...

hrp0095p2-28 | Bone, Growth Plate and Mineral Metabolism | ESPE2022

Longitudinal assessment of bone health index (BHI) as a measure of bone health in short-statured children before and during treatment with recombinant growth hormone

Holzapfel Lukas , Bettendorf Markus , Rabe Michael , Mittnacht Janna , Choukair Daniela , Schenk Jens-Peter

Introduction: Bone Health Index (BHI) determined by measurement of cortical thickness of metacarpalia II-IV in x-rays of the left hand represents a method to estimate bone health in children. Aim of this study was to investigate changes of BHI SDS in the course of growth hormone (GH) treatment.Method: 256 consecutive children with short stature (isolated GH deficiency (IGHD) n = 121, multiple pituitary hormone deficiency...

hrp0089p2-p326 | Pituitary, Neuroendocrinology and Puberty P2 | ESPE2018

Pituitary Adenomas in Children and Adolescents: A Retrospective Single-Centre Analysis

Breil Thomas , Lorz Catherine , Choukair Daniela , Mittnacht Janna , Inta Ioana , Klose Daniela , Jesser Jessica , Schulze Egbert , Bettendorf Markus

Background: Paediatric pituitary adenomas are rare and mostly benign disorders which may secrete pituitary hormones. Prolactinomas account for half of all pituitary adenomas (PROLA), followed by non-secreting adenomas (20–40%; incidentalomas), adrenocorticotropic-hormone secreting adenomas (10–30%; ACTHA) and growth-hormone-secreting adenomas (5–15%; GHA).Methods: In this single-centre retrospective analysis clinical, biochemical and radio...

hrp0086p1-p99 | Bone &amp; Mineral Metabolism P1 | ESPE2016

Automated Greulich-Pyle Bone Age Determinations in Children with Chronic Endocrine Diseases

Choukair Daniela , Huckmann Annette , Mittnacht Janna , Inta Ioana , Klose Daniela , Schenk Jens Peter , Bettendorf Markus , Thodberg Hans Henrik

Background: Prediction of adult height is a standard procedure in pediatric endocrinology, but it is associated with considerable interrater variability.Objective and hypotheses: To compare the new adult height prediction (PAH) method by automated bone age determination (BoneXpert™) with the conventional PAH method by Bayley Pinneau (BP) based on bone age determination according to Greulich & Pyle. Furthermore, to assess measures of bone health ...