hrp0089p1-p161 | Growth & Syndromes P1 | ESPE2018

Analysis of Osteoblats Precursors in Girls with Turner Syndrome

Brunetti Giacomina , Chiarito Mariangela , Piacente Laura , Aceto Gabriella , Colucci Silvia , Colaianni Graziana , Grano Maria , D'Amato Gabriele , Felicia Faienza Maria

Background and aim: Subjects with Turner Syndrome (TS) show low cortical bone mineral density (BMD), osteoporosis and risk of fractures. Previously, we demonstrated the enhanced spontaneous osteoclastogenesis in girls and young women with TS before and after pubertal induction with hormonal replacement therapy (HRT). The bone resorption observed in girls before puberty induction seems to be supported by the high FSH serum levels observed at prepubertal stage, while in young wo...

hrp0086fc4.2 | Pathophysiology of Obesity | ESPE2016

Immune-Fat-Bone Axis in Obese Children: The Role of LIGHT

Brunetti Giacomina , Piacente Laura , Ventura Annamaria , Aceto Gabriella , Colucci Silvia , Cavallo Luciano , Grano Maria , Faienza Maria Felicia

Background: Obesity during childhood has been demonstrated to exert profound and lasting effects on bone strength and fracture risk. Furthermore, obesity is characterized by chronic inflammation and oxidative stress, with an increase in the mediators of innate immunity. It has been found that skeletal homeostasis is influenced by immune cells. LIGHT (lymphotoxin-like inducible protein that competes with glycoprotein D for herpesvirus entry on T cells) is emerging among cytokin...

hrp0092rfc6.2 | Bone, Growth Plate and Mineral Metabolism Session 2 | ESPE2019

High Levels of LIGHT/TNFSF14 in Prader-Willi Syndrome

Brunetti Giacomina , Fintini Danilo , Crinò Antonino , Piacente Laura , Convertino Alessio , Concetta Colucci Silvia , Grano Maria , Grugni Graziano , Faienza Maria Felicia

Background: Low bone mineral density (BMD) has been found in up to 50% of adolescents and adults with Prader-Willi syndrome (PWS). High fracture risk has been described in adult PWS patients. However, the mechanism/s of low BMD in PWS have not been clarified. These patients also display high BMI-SDS that prompted us to evaluate the levels of LIGHTTNFSF14, a cytokine involved in pathological bone remodeling and obesity.Objecti...

hrp0089rfc6.6 | Fat, Metabolism and Obesity | ESPE2018

Effects of Cherry’s Extract on Increased Osteclastogenesis in Obese Children

Faienza Maria Felicia , Corbo Filomena , Carocci Alessia , Catalano Alessia , Piacente Laura , Clodoveo Maria Lisa , Bortolotti Sara , Storlino Giuseppina , Colucci Silvia , Grano Maria , D'Amato Gabriele , Brunetti Giacomina

The accumulation of adipose tissue, especially visceral fat, represents a risk factor for reduced bone mineral density and spontaneous fractures. Several mechanisms have been proposed to explain the complex relationship between adipose tissue and bone. Obesity is associated with the production of inflammatory cytokines both by adipocytes and immune cells which may stimulate bone resorption and reduce bone strength. Studies in vitro and in vivo have reported t...

hrp0086fc5.6 | Management of Disorders of Insulin Secretion | ESPE2016

Impact of Continuous Subcutaneous Insulin Infusion versus Multiple Daily Injections on Bone Health in Children and Adolescents with Type 1 Diabetes

Faienza Maria Felicia , Delvecchio Maurizio , Ventura Annamaria , Aceto Gabriella , Piacente Laura , Zecchino Clara , Colucci Silvia , Grano Maria , Cavallo Luciano , Brunetti Giacomina

Background: Type 1 diabetes (T1DM) is associated with low bone mineral density (BMD) and bone alterations, probably due to lack of insulin and chronic hyperglycemia. Sclerostin and Dickkopfs-1 (DKK-1) are Wnt signaling inhibitors involved in bone remodelling.Objective and hypotheses: To assess serum levels of DKK-1 and sclerostin in T1DM children and adolescents on continuous subcutaneous insulin infusion (CSII) or multiple daily therapy (MDI). We hypoth...

hrp0084p1-34 | Diabetes | ESPE2015

Low Bone Mineral Density is Associated to Poor Glycemic Control and Increased Dickkopf-1 (DKK-1) Serum Levels in Children and Adolescents with Type 1 Diabetes

Faienza Maria Felicia , Delvecchio Maurizio , Fusillo Anna , Lonero Antonella , Zecchino Clara , Acquafredda Angelo , Piacente Laura , Grano Maria , Cavallo Luciano , Brunetti Giacomina

Background: Decreased bone mineral density (BMD) and increased fracture risk have consistently been observed in type 1 diabetes mellitus (T1DM). The influence of T1DM on BMD seems to depend on gender or patient’s age and to occur early after T1DM diagnosis. The mechanisms of decreased BMD in T1DM patients are still unknown.Objective and hypotheses: To investigate the serum levels of dickkopf-1 (DKK-1), a Wnt signaling inhibitor which decreases bone ...

hrp0084p2-205 | Bone | ESPE2015

Mechanism of Bone Disease in Prader-Willi Syndrome

Faienza Maria Felicia , Brunetti Giacomina , Grugni Graziano , Crino Antonino , Bocchini Sarah , Oranger Angela , Gigante Isabella , Piacente Laura , Ventura Annamaria , Colucci Silvia , Grano Maria , Cavallo Luciano , Delvecchio Maurizio

Background: Low bone mineral density (BMD) is found in up to 50% of adolescents and adults with Prader-Willi syndrome (PWS). High fracture risk has been described in adult PWS patients. This bone fragility could be due to inadequate gonadal hormones levels during pubertal development, and to relative growth hormone insufficiency during childhood and adolescence. However, the mechanism/s of low BMD in PWS have not been clarified.Objective and hypotheses: ...

hrp0082p1-d2-120 | Fat Metabolism & Obesity (1) | ESPE2014

RANKL and Osteoprotegerin Serum Levels in Obese Children and Adolescents

Faienza Maria Felicia , Vulpi Maria Rosaria , Piacente Laura , Ventura Annamaria , Lonero Antonella , Luce Vincenza , De Palma Fabrizia , Acquafredda Angelo , Zecchino Clara , Minenna Antonio , Delvecchio Maurizio , Grano Maria , Cavallo Luciano , Brunetti Giacomina

Background: There is growing evidence of a correlation between fat and bone metabolism at both the clinical and molecular levels, although the systemic regulators have not been clearly identified. The receptor activator of nuclear factor kB ligand (RANKL) and its soluble decoy receptor, osteoprotegerin (OPG), are involved in bone resorption and vascular calcification. OPG levels has been related with insulin resistance in adult obese subjects.Objective a...

hrp0092p2-83 | Diabetes and Insulin | ESPE2019

17q12 Deletion and a Family History of Diabetes

Kasongo Laura , Nicolescu Ramona

Introduction: 17q12 deletion syndrome is associated with an enlarging phenotype, the most frequent clinical findings being renal and genitourinary malformations, diabetes mellitus (β-cell developmental defect) and exocrine pancreas deficiency, variable cognitive impairment with dysmorphic features.Diabetes, known as MODY 5 (maturity-onset diabetes of the young), is an autosomal dominant monogenic type and the most commonly identi...

hrp0092p3-257 | Thyroid | ESPE2019

Bilateral Hip Pain as First Symptomatic Expression of Severe Primary Hypothyroidism

Kasongo Laura , Nicolescu Ramona

Introduction: Legg-Calvé-Perthes disease is an idiopathic osteonecrosis of the femoral head with uncoupling of bone resorption and formation, presenting as unilateral involvement in most children. Symmetric involvement suggests other conditions: skeletal dysplasia, osteonecrotic entities including hypothyroidism, Gaucher's disease, glycogen storage defects, sickle cell anemia.Case presentation: We present a case...