hrp0092fc2.6 | Bone, Growth Plate and Mineral Metabolism Session 1 | ESPE2019

Validation of a New Version of BoneXpert Bone Age in Children with Congenital Adrenal Hyperplasia (CAH), Precocious Puberty (PP), Growth Hormone Deficiency (GHD), Turner Syndrome (TS), and other Short Stature Diagnoses

Thodberg Hans Henrik , Martin David D

Background: The BoneXpert method for automated determination of bone age from hand X-rays is based on machine learning, so it lends itself naturally to be improved by adding more training data and using better learning algorithms. Currently, version 2 is running in 145 hospitals across Europe, and a new version 3 is rolled out in 2019.Objective and Hypotheses: The aim was to validate version 3 against manual ratings in r...

hrp0092rfc5.2 | Thyroid | ESPE2019

Ultrasound Features of Multinodular Goiter in DICER1 Syndrome

Niedziela Marek , Muchantef Karl , Foulkes William D

Background: DICER1 syndrome is caused by germline mutations in the DICER1 gene. It is associated with a wide spectrum of benign and malignant neoplasms, which are accompanied by specific somatic mutations in DICER1. Multinodular goiter (MNG) is a common clinical feature of DICER1 syndrome in children and adults; the thyroid ultrasound (US) features of MNG in the setting of DICER1 syndrome have not been widely reported.<...

hrp0092p2-138 | Fat, Metabolism and Obesity | ESPE2019

A Rapid Instrument for Diagnosis and Screening of Pediatric Obesity and its Complications: The Neck Circumference

Guercio Nuzio Salvatore , D'Isanto Livio

Objectives: Pediatric obesity (PO) leads in adulthood to chronic high-risk pathologies, if not adequately identified and addressed. The anthropometric methods for evaluating PO have well-described limits. One of the most recently proposed indexes to better locate and evaluate PO is the neck circumference (NC). We have verified the relationship between NC, body mass index (BMI), waist circumference (WC) and some laboratory parameters, with the aim of defining t...

hrp0086p2-p864 | Syndromes: Mechanisms and Management P2 | ESPE2016

Secretion of Somatostatin and Growth Hormone (GH) in Various Forms of Hereditary Pathology

Muhamedov R.S. , Ibragimova N. Sh. , Dalimova D.

Background: Patients with HP have stunting of various degree of expression but the most pronounced stunting is found in patients with Russell-Silver syndrome, Sekkel syndrome and Cornelius de Lange syndrome which is associated with disorders in the hypothalamus ┬ľ hypophyseal system (somatostatin - GH).Objective and hypotheses: To study secretion of somatostatin and insulin-like growth factor (IGF-1, IGFBP-3) in various forms of hereditary pathologie...

hrp0092p1-370 | GH and IGFs (2) | ESPE2019

Challenges Experienced in Delivering Growth Hormone Therapy in Children's with Prader Willi Syndrome in Birmingham Children's Hospital.

Kollurage D Udeni Anuruddhika , Barrett Tim , Jayamanne B D W , Krone Ruth

Prader Willi Syndrome (PWS) is a rare neuro-genetic disorder inherited as a result of lack of expression in 15q11-13 gene and 70% are paternally inherited. Characteristic features are dysmorphism, behavioural problems, infantile hypotonia, short stature, hypothalamic dysfunction, hyperphagia and morbid obesity. The long-term morbidity and mortality depend on hypothalamic dysfunction and obesity. While multidisciplinary care is essential, growth hormone (GH) is a recognized...

hrp0092p3-109 | Fat, Metabolism and Obesity | ESPE2019

Follow-Up Evaluation of Clinical Markers and Inflammatory, Biochemical and Hormonal Profiles in Children with Bodyweight Problems

Ṭaranu Ioana , Bolboacă Sorana D. , Creţ Victoria

Introduction: In the last years, the relationship between clinical markers and blood parameters has been evaluated closely as part of transversal studies, but a longitudinal approach might be more useful in explaining its complexity.Aim: The study aims to evaluate the relationship between changes in the values of clinical markers and of the inflammatory, biochemical and hormonal profiles of obese and overweight children ...

hrp0082p3-d2-686 | Bone (1) | ESPE2014

I.V. Zolendronic Acid: Experience of Treatment of Children with Osteogenesis Imperfecta in Indonesia

Pulungan A , Zacharin M , Armstrong K , Soesanti F , Pramesti D L

Background: The incidence of osteogenesis imperfecta (OI) worldwide is unknown. In the USA, the incidence is ~1/20 000 live births: for Indonesia (population 240 million) this should extrapolate to 12 000 OI patients rather than the 35 patients currently registered with the Indonesian Pediatrics Society (IPS), Faculty of Medicine, University of Indonesia-Cipto Mangunkusumo Hospital (RSCM). This enormous disconnect signifies many missed diagnoses, mortality or both.<p class...

hrp0084p2-343 | Fat | ESPE2015

Chronodisruption in Obese Children

Saner Christoph , Mullis Primus , Simonetti Giacomo D , Janner Marco

Background: Altered circadian and ultradian blood pressure (BP) and heart rate (HR) rhythmicity has been described in many diseases with increased cardiovascular risk.Objective and hypotheses: We tested the hypothesis that rhythmicity in obese children is changed, compared to healthy subjects.Method: Circadian and ultradian BP and HR rhythmicity was assessed with Fourier analysis from 24-h ambulatory BP measurement (ABPM) in 75 obe...

hrp0084p3-1129 | Pituitary | ESPE2015

Thickened Pituitary Stalk with Central Diabetes Insipidus: What Diagnosis?

Merazka Amel , Achir S , Baz O , Foudil D , Mimouni S

Background: Central diabetes insipidus (DIC) is usually the final result of lesions affecting the hypothalamic┬ľneurohypophysal system, for the children, Germinoma is the main reason. The MRI aspect is often limited to thickness pituitary stalk with loss of hyperintensity of the neurohypophysis.Objective and hypotheses: Thickening of pituitary stalk is suggestive of germinoma, the clinical picture is dominated by a DIC (90%), associated to hypopituit...

hrp0092p1-42 | Fat, Metabolism and Obesity | ESPE2019

De-novo and Depot-Specific Androgen Production in Human Adipose Tissue - a Source of Hyperandrogenism in Obese Females

Wagner Isabel Viola , Sahlin Lena , Kulle Alexandra , Klöting Nora , Döbeln Viola , Savchuk Iuliia , Dötsch Jörg , Söder Olle

Background: Obesity in females is often associated with metabolic complications and hyperandrogenism but the role of adipose tissue (AT) in androgen synthesis remains unclear.Aims/Objectives: Employing human subcutaneous and visceral AT and cultured adipocytes, we studied whether AT could be a source of androgens promoting hyperandrogenism in lean and especially in obese females.Methods</st...