hrp0092fc6.1 | Bone, Growth Plate and Mineral Metabolism Session 2 | ESPE2019

Bone Tissue Characterization of a Mouse Model of Atypical Type VI Osteogenesis Imperfecta Reveals Hypermineralization of the Bone Matrix, Elevated Osteocyte Lacunardensity and Altered Vascularity

Hedjazi Ghazal , Guterman-Ram Gali , Blouin Stéphane , Roschger Paul , Klaushofer Klaus , Fratzl-Zelman Nadja , Marini Joan C

Objectives: Osteogenesis imperfecta (OI) is an extremely heterogeneous connective tissue disorder characterized by low bone mass, which together with altered bone matrix properties leads to skeletal fragility. Due to the wide range of symptoms, the pathophysiology of the OI is not fully understood. Null mutations in SERPINF1, encoding the potent antiangiogenic factor PEDF, cause type VI OI with excessive osteoid formation, abnormal osteoblast-osteocyt...

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 ...

hrp0092fc6.3 | Bone, Growth Plate and Mineral Metabolism Session 2 | ESPE2019

Decreased Trabecular Bone Mineral Density and Muscle Area at the Forearm Despite Improvement in Glycaemic Control Over 3 Years After Simultaneous Pancreas Kidney Transplantation

Soucek Ondrej , Maratova Klara , Sumnik Zdenek , Brunova Jana , Kratochvilova Simona

Background: Simultaneous pancreas kidney transplantation (SPKT) is a standard treatment option for young adults with type I diabetes (T1D) and concurrent renal failure. Despite the long-term immunosuppressive therapy the patients have better glycemic control, normalized renal function and an improved quality of life. Whether this is also reflected in the skeleton is not that clear yet.Methods: Patients were prospectively...

hrp0092fc6.4 | Bone, Growth Plate and Mineral Metabolism Session 2 | ESPE2019

Metabolically Unhealthy Obese Children and Adolescents Have Higher Bone Mineral Density Than Normal Weighted Controls but Lower than Metabolically Healthy Obeses: No Effect of FGF21 Levels

Akduman Filiz , Siklar Zeynep , Ozsu Elif , Doğan Ozlem , Kir Metin , Berberoglu Merih

Introduction: The harmfull or benefical effect of obesity on bone mineral density (BMD) is remain controversial in children and adolescence. Either increase or decrease of BMD have been reported. Several factors such as insülin resistance, prediabetes, high proportion of fat mass, sedentary lifestyle were suggested to cause the differences of BMD in obesity. FGF-21 is a metabolic factor that plays a specific role in the regulation of carbohydrate and lipi...

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...

hrp0092fc6.6 | Bone, Growth Plate and Mineral Metabolism Session 2 | ESPE2019

Craniosynostosis in Inactivating PTH/PTHrP Signaling Disorder 2: A Non-Classical Feature to Consider

Riaño-Galan Isolina , Rothenbuhler Anya , Debza Yahya , Barosi Anna , Mantovani Giovanna , Perez de Nanclares Guiomar , Linglart Agnès

Classic features of inactivating PTH/PTHrP Signaling Disorder 2 or 3 (iPPSD2, iPPSD3), i.e. former pseudohypoparathyroidism include multi-hormone resistance, short stature, subcutaneous ossifications, brachydactyly, and early-onset obesity and a molecular defect at the GNAS region. In addition, patients may present with less-known features including craniosynostosis (CSO).Objective: To describe the prevalence of CSO in a cohort ...

hrp0092fc7.1 | Diabetes and Insulin Session 2 | ESPE2019

Deployment of a Predictive Model Based on CpG Methylation Haplotypes Analysis on the Insulin Gene Promoter, in a Cohort of Children and Adolescents with Type 1 Diabetes

Kotanidou Eleni P , Mouzaki Konstantina , Chouvarda Ioanna , Koutsiana Elisavet , Kosvyra Alexandra , Giza Styliani , Galli-Tsinopoulou Assimina

Background: Cytosine-guanine(CpGs) sites in molecules identified as methylated or unmethylated; the combination of them in the genetic sequence of an individual includes a methylation haplotype (methyl-haplotype) for a specific locus. The insulin gene promoter(IGP) is highly regulated by methylation mechanisms, which lead to alteration of gene expression.Aim: To identify IGPmethyl-haplotypes among children/adolescents wi...

hrp0092fc7.2 | Diabetes and Insulin Session 2 | ESPE2019

Copeptin Kinetics and its Relationship to Osmolality During Rehydration for Diabetic Ketoacidosis in Children: An Observational Study

Burckhardt Marie-Anne , Beglinger Svetlana , Gotta Verena , Renggli Luzia , Bachmann Sara , Hess Melanie , Rentsch Katharina , Koch Gilbert , Zumsteg Urs , Jones Timothy , Pfister Marc , Szinnai Gabor

Background: Copeptin is a surrogate marker for arginine vasopressin (AVP) release in response to hyperosmolal stimuli such as diabetic ketoacidosis (DKA). We aimed to characterize the temporal course (kinetics) of serum osmolality and copeptin during rehydration and insulin therapy in children with type 1 diabetes (T1D) and DKA, and the relationship between both (dynamics).Methods: An observational multi-center study was...

hrp0092fc7.3 | Diabetes and Insulin Session 2 | ESPE2019

MicroRNA Circulating Levels in Children at Diagnosis of Type 1 Diabetes

Inzaghi Elena , Rapini Novella , Deodati Annalisa , Schiaffini Riccardo , Patera Patrizia , Matteoli Maria Cristina , Ciampalini Paolo , Cianfarani Stefano

Background: Type 1 diabetes (T1D) is a chronic disease characterized by autoimmune destruction of pancreatic beta-cells. Dysregulated miRNA levels have been described in T1D patients, though results are inconclusive.Objective and Hypotheses: The aim of this study was to assess the circulating profile of different miRNAs in children at diagnosis of T1D.Method: 27 children with T1D o...

hrp0092fc7.4 | Diabetes and Insulin Session 2 | ESPE2019

Metabolic Syndrome Features in Pre-Pubertal Children Born After Maternal Pre-Eclampsia

Goffin Sarah , Derraik José , Chiavaroli Valentina , Biggs Janene , Hofman Paul , Groom Katie , Cutfield Wayne

Background: Pre-eclampsia is associated with important complications for both mother and baby in the short term, but there are limited data about its long-term effects on offspring metabolism. Thus, we aimed to assess whether maternal pre-eclampsia was associated with adverse effects on metabolism and body composition in the offspring in childhood.Methods: We studied healthy pre-pubertal children (aged 4–10 years) b...