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...

hrp0092p3-279 | Late Breaking Abstracts | ESPE2019

Relation between Levels of Atymullerian Hormone and Inhibin B and Spontaneous Puberty in Patients with Turner Syndrome – Preliminary Results

Ruszała Anna , Wójcik Małgorzata , Starzyk Jerzy B.

Introduction: Primary hypogonadism is stated as one of major features in patients with Turner Syndrome (TS), however it is not a constant finding. Variable markers of ovaries failure in TS patients are still missing. The present study aimed to evaluate the usefulness of atymullerian hormone and inhibin B assessment in predicting spontaneous puberty in patients with TS.Methods: The study included 35 TS patients. Gonadal a...

hrp0092p3-317 | Late Breaking Abstracts | ESPE2019

Body Mass Index and Incident Type 1 Diabetes in Children from Lesser Poland Over an 11 Year Observation Period

Wasyl-Nawrot Barbara , Wójcik Małgorzata , Nazim Joanna , Skupień Jan , Starzyk Jerzy

Background: One of the speculated causes of the decreasing age of onset of type 1 diabetes is the increase in body weight in children. This so-called accelerator hypothesis is, however, controversial. The aim of the study was to test whether younger age of type 1 diabetes onset is associated with higher BMI-SDS at the time of diagnosis.Methods: Retrospective data analysis from medical records of all patients und...

hrp0089p1-p156 | GH & IGFs P1 | ESPE2018

The Effect of Growth Hormone Treatment in Children after Hematopoietic Stem Cell Transplantation

Hoekx CA , Bresters D , Le Cessie S , Scholte C , Oostdijk W , Hannema SE

Background: Hematopoietic stem cell transplantation (HSCT) has become more common in treating malignant and nonmalignant diseases in children. However, HSCT is associated with several late effects that can impair growth, like insufficient growth hormone (GH) secretion, hypogonadism and growth plate damage. Growth hormone treatment (GHRx) is offered but limited data are available on its effect on adult height.Objective: To evaluate the effectiveness of GH...

hrp0086p2-p181 | Bone & Mineral Metabolism P2 | ESPE2016

Pseudohypoparathyroidism Type IB Associated to Assisted Reproductive Technologies: Case Report

Fernandez Monica , Riquelme Joel , Castiglioni Claudia , Jueppner Harald W , Mericq Veronica

Background: Pseudohypoparathyroidism type Ib (PHP-Ib) is due to a maternal loss of GNAS exon A/B methylation and leads to decreased expression of the stimulatory G protein (Gsα) in specific tissues. Evidence suggests an increased incidence of imprinting disorders in children conceived by Assisted Reproductive Technologies (ART). Nevertheless, no associations between ART and PHP – Ib have been found to date.Clinical case: 7.4-years-old male with...

hrp0086p1-p489 | Fat Metabolism and Obesity P1 | ESPE2016

Impact of a Group-based Treatment Program on Adipocytokines, Oxidative status, Inflammatory Cytokines, and Pulse Wave Velocity in Obese Children and Adolescents

Santiprabhob Jeerunda , Limprayoon Kawewan , Aanpreung Prapun , Charoensakdi Ratiya , Kalpravidh Ruchaneekorn W. , Phonrat Benjaluck , Tungtrongchitr Rungsunn

Background: The link between obesity and dysregulation of adipocytokines, inflammatory cytokines, and oxidative status has been found to underline the pathogenesis of obesity-related complications, such as cardiovascular disease and type 2 diabetes.Objective and hypotheses: The objectives were i) to evaluate the effect of a group-based lifestyle modification program on adipocytokines, inflammatory cytokines, oxidative status, and brachial-ankle pulse wav...

hrp0086p1-p620 | Growth P1 | ESPE2016

Efficacy of Growth Hormone Treatment in Patients with Type 1 Diabetes Mellitus and Growth Hormone Deficiency

Bonfig Walter , Lindberg Anders , Cutfield Wayne , Dunger David , Camacho-Hubner Cecilia , Holl Reinhard W

Background: The combination of type 1 diabetes mellitus (T1DM) and GH deficiency is uncommon. In a previous study (1) we found that in children with T1DM and GHD with adequate adaptation of insulin dosage, the metabolic control of T1DM did not worsen during GH treatment. However, decreased catch-up growth was observed and no data on GH dose was available.Objective: To analyse first year treatment growth response and GH dosage in prepubertal patients with...

hrp0084p3-705 | Diabetes | ESPE2015

Treatment of Dyslipidemia in Children and Adolescents with Diabetes Mellitus Type 1

Borgerink M M H , van Albada M A , Venema G , Bakker-van Waarde W M

Background: Cardiovascular disease (CVD) is the major cause of mortality in diabetes mellitus type 1 (T1DM). Dyslipidemia will increase this risk. Several guidelines have been published, how to treat dyslipidemia in T1DM, yet some studies have shown that the number of patients who are treated according to these guidelines is low.Objective and hypotheses: To investigate the frequency of dyslipidemia in children and adolescents with T1DM and if they are tr...

hrp0097t6 | Section | ESPE2023

Granulosa cell tumors in girls: Preliminary results of a meta-analysis of new and published cases

Kiss Eszter , Wessmann Sandra , W Carlson Joseph , Lundberg Elena , Stenmarker Margaretha , Bobeck Johan , Lodefalk Maria

Background: Granulosa cell tumors (GCT) originate from sex cord/stromal tissue in the gonad. They are typically located in an ovary, but extra-gonadal localisation exists. These tumors are extremely rare in children and no systematic review has been published. The objective of this systematic review is to examine the following questions: What is the clinical picture of girls with a GCT? How are these patients treated and what is their prognosis?<p class="a...

hrp0084fc1.6 | Adrenal | ESPE2015

An Update on Noninvasive Prenatal Diagnosis of Congenital Adrenal Hyperplasia Using Cell-free Foetal DNA in Maternal Plasma

New Maria , Tong Yu , Jiang Peiyong , Pina Christian , Chan K C Allen , Khattab Ahmed , Liao Gary J W , Yau Mabel , Kim Se-Min , Chiu Rossa W K , Sun Li , Zaidi Mone , Lo Y M Dennis

Background: Congenital adrenal hyperplasia (CAH) arises from mutations in CYP21A2 gene, which encodes for the steroidogenic enzyme 21-hydroxylase. Currently employed chorionic villus sampling and amniocentesis provide genetic results at ~14 weeks of gestation at the earliest. At this time, the genitalia of the affected female foetuses have already become virilized. To prevent genital ambiguity, prenatal treatment with dexamethasone must begin on or before gestational ...