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

Zone Wise Cell Separation Methods Comparison, Based on Relative Expression of Specific Growth Plate Markers in a Pig Model

Javanmardi Alireza , Raimann Adalbert , Egerbacher Monika , Sagmeister Susane , Gleiss Andreas , Haeusler Gabriele

Introduction: Longitudinal skeletal growth is achieved by enchondral ossification in epiphyseal growth plates (GP) of long bones and vertebrae. These highly organized cartilaginous tissues contain chondrocytes of all differentiational stages classified in three distinct zones named resting, proliferative and hypertrophic. Separated analysis of individual zones is essential in basic GP research thus efficiency of different zonal separation methods confers high ...

hrp0092rfc12.2 | Growth and Syndromes (to include Turner syndrome) | ESPE2019

Treatment with Growth Hormone Increases Klotho Concentration in Patients with Turner Syndrome

Wikiera Beata , Seifert Monika , Nocon-Bohusz Julita , Noczynska Anna , Daroszewski Jacek

Background: Short stature, increased adiposity and insulin resistance are conditions frequently observed in patients with Turner syndrome (TS). Many hormones are involved in the pathogenesis of the condition but therapeutic options we can offer to the patients are still scant. Each newly discovered peptide give us hope. Klotho play a very important role in the regulation of the human body metabolism and was not investigated in patients with TS so far.<p cl...

hrp0092p2-278 | Thyroid | ESPE2019

A 10- Year-Old Girl with Thyroid Hormone Resistance (βTHR)- Case Report

Bossowski Artur , Michalak Justyna , Sawicka Beata , Sanczyk Hanna Borysewicz- , Kolanowska Monika , Kubiak Anna , Jazdzewski Krystian

Introduction: Thyroid hormone resistance (THR) is a rare syndrome which is characterized by reduced response to thyroid hormones at tissue level. The disorder is caused by genetic mutation in the thyroid hormone receptor. The most common are a heterozygous thyroid hormone β (THRβ) gene mutations. Laboratory tests usually show normal or elevated level of thyroid- stimulating hormone (TSH) and high concentration of thyroid hormones (T3 and T4).<p c...

hrp0089fc10.4 | Late Breaking | ESPE2018

Hypothalamus Sparing Surgery Improves the Outcome of Patients with Severe Initial Hypothalamic Involvement of Childhood Craniopharyngioma: Results of the Prospective Multinational Trial KRANIOPHARYNGEOM 2007

Bogusz Agnieszka , Boekhoff Svenja , Warmuth-Metz Monika , Calaminus Gabriele , Eveslage Maria , Muller Hermann L

Context: Quality of survival (QoS) is frequently impaired in childhood-onset craniopharyngioma (CP) patients due to sequelae caused by the hypothalamic syndrome. The debate, whether primary hypothalamic involvement (HI) has apriori prognostic impact or surgical hypothalamic lesions (HL) determine outcome, is controversial. Accordingly, we analyzed, whether CP patients at high risk for hypothalamic obesity due to primary HI of anterior and posterior hypothalamic structures bene...

hrp0089fc11.2 | Bone, Growth Plate &amp; Mineral Metabolism 2 | ESPE2018

Elevated Phosphate Levels Inhibit Skeletal Muscle Cell Differentiation In vitro

Raimann Adalbert , Egerbacher Monika , Greber-Platzer Susanne , Dangl Alexander , Pietschmann Peter , Haeusler Gabriele

Background: Hyperphosphatemic conditions such as chronic kidney disease are associated with muscle wasting and impaired life quality. While muscle regeneration relies on myogenic progenitor recruitment, the effects of high phosphate loads on this process has not been investigated in detail. This study aims to clarify the direct effectsof hyperphosphatemic conditions on skeletal myoblast differentiation in an murine cell model system.Material and methods:...

hrp0089p1-p148 | GH &amp; IGFs P1 | ESPE2018

GH Treatment Causes an Increase in Klotho Concentration in Children with Growth Hormone Deficiency

Wikiera Beata , Seifert Monika , Nocon-Bohusz Julita , Noczynska Anna , Daroszewski Jacek

Introduction: Klotho is a protein which may serve as a regulator of GH secretion. Growth hormone deficiency is diagnosed in children with growth restriction when GH secretion in two GH stimulation tests do not exceed the level of 10 ng/ml.Aim: The objective of the study was to investigate Klotho and FGF23 in children with growth hormone deficiency (GHD) and their responce to the treatment with recombinant human growth hormone (rHGH).<p class="abstext...

hrp0089p2-p222 | GH &amp; IGFs P2 | ESPE2018

Height Perception of Children with GH Deficiency: Influencing Factors and Links to Psychosocial Functioning

Drosatou Chrysoula , Vlachopapadopoulou Elpis-Athina , Bullinger Monika , Quitmann Julia , Silva Neuza , Michalacos Stefanos , Tsoumakas Konstantinos

Objectives: The aims of this study were: (1) to evaluate the perception of children with GH Deficiency (GHD) and their parents, regarding their current and future predicted height, as well as the modulating factors; (2) to investigate the relation between perceptions of height and psychosocial functioning in children with GHD.Methods: The study group consists of 322 children/adolescents (219 boys) diagnosed with (isolated) GHD, treated with GH. The mean ...

hrp0089p2-p224 | GH &amp; IGFs P2 | ESPE2018

Factors Influencing Health Related Quality of Life in Children/Adolescents with GH Deficiency

Drosatou Chrysoula , Vlachopapadopoulou Elpis-Athina , Quitmann Julia , Bullinger Monika , Silva Neuza , Michalacos Stefanos , Tsoumakas Konstantinos

Objectives: To describe the Health Related Quality of life (HrQoL) of children/adolescents with GH Deficiency (GHD) and to examine the effects of sociodemographic (i.e., patients’ age, sex and family socioeconomic status) and clinical characteristics (i.e. degree of short stature and duration of treatment) on HrQoL from patients’ and their parents’ perspectives. Associations between QoLISSY questionnaire and demographics were evaluated using Mann-Whitney or Krus...

hrp0086p1-p144 | Bone &amp; Mineral Metabolism P1 | ESPE2016

Effects of Phylloquinone and Magnesium on ATDC5 Prechondrocytes

Raimann Adalbert , Javanmardi Alireza , Sagmeister Susanne , Ertl Diana-Alexandra , Claudia Hochsmann , Monika Egerbacher , Gabriele Haeusler

Background: Cell-mediated initiation of enchondral ossification is essential for growth plate maturation. The matrix mineralization inhibitors matrix Gla protein (MGP) and osteocalcin (OC) represent key regulators of matrix mineralization and are highly expressed in growth plate chondrocytes. Pharmacological or nutritional phylloquinone (K1) depletion is known to affect skeletal mineralization by reduced gamma-carboxilisation of MGP and OC. Constituents of mineral matrix such ...

hrp0082p1-d2-121 | Fat Metabolism &amp; Obesity (1) | ESPE2014

Sex-, Age- and Height-Specific Reference Curves for the 6-min Walk Test in Healthy Children and Adolescents

Saraff Vrinda , Schneider Johanna , Colleselli Valeria , Ruepp Monika , Neururer Sabrina , Rauchenzauner Markus , Geiger Ralf , Hogler Wolfgang

Background: The 6-min walk test (6MWT) is a simple, accurate and safe method to measure functional exercise capacity. The 6MWT is increasingly used in children to predict morbidity and mortality from cardiopulmonary disease, to assess functional capacity and measure disease progression in chronic childhood conditions such as muscular dystrophy.Objective and hypotheses: To provide smooth reference curves for the 6-min walk distance (6MWD) in healthy child...