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...

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...

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...

hrp0082p1-d1-184 | Perinatal and Neonatal Endocrinology | ESPE2014

Very Low Birth Weight <1500 g is Associated with Reduced Sex-Typical Behaviour in Childhood

Sankilampi Ulla , Hines Melissa , Lamminmaki Annamarja

Objective: Low birth weight and prematurity are linked to various behavioural outcomes. In addition, preterm (PT) infants show altered maturation of pituitary–gonadal axis, as demonstrated by its pronounced transient activation during the first postnatal months. Given that gonadal steroid hormones shape the basic processes of neural and behavioural sexual differentiation these elevated sex steroids in premature infants might affect the developing brain and alter subsequen...

hrp0086p2-p759 | Pituitary and Neuroendocrinology P2 | ESPE2016

A Case of Central Precocious Puberty in a Patient with Prader-Willi Syndrome

Pellegrin Maria Chiara , Tornese Gianluca , Faleschini Elena , Ventura Alessandro

Background: Hypogonadism is one of the major diagnostic criteria of Prader-Willi syndrome (PWS). A hypogonadotropic hypogonadism is often present as a result of hypothalamic dysfunction (together with other hormonal disorders, such as growth hormone deficiency and hypothyroidism).Presentation: A 8.5-year-old boy with genetically-confirmed PWS (maternal uniparental disomy) presented in our Endocrinology Unit for routinely follow-up. Therapy with rhGH was ...

hrp0084p3-1020 | Growth | ESPE2015

GH Deficiency and Glucose 1 Transporter Deficiency Syndrome

Patti Giuseppa , Tornese Gianluca , Costa Paola , Faleschini Elena , Ventura Alessandro

Background: GLUT1 deficiency syndrome (GLUT1DS) is a treatable epileptic encephalopathy resulting from impaired glucose transport into the brain. Clinical features comprise motor and mental developmental delay, seizures with infantile onset, deceleration of head growth often resulting in acquired microcephaly, and a movement disorder with ataxia, dystonia, and spasticity. While it is known that ketogenic diet may cause growth retardation, GH deficiency (GHD) may represent anot...

hrp0092p1-282 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology (1) | ESPE2019

Characteristics of 311 Children with Early Onset Pubertal Signs. Descriptive Study

Wichner Paula Sol Ventura , Herrero Xavier , Llorca Maria Laura , Bosch Zelmira , Lacruz Marisa Torres

Aim of our study was to assess clinical characteristics and complementary studies, in patients who consulted the Endocrinology Department of our pediatric hospital, referred by pediatricians to discard precocious puberty.Methodology: it is a descriptive study based on review of medical records, with first consultation between 2010 and 2018. Criteria were developed to assign patients to one of six diagnostic categories based on age, growt...

hrp0086p1-p824 | Syndromes: Mechanisms and Management P1 | ESPE2016

Growth Hormone Deficiency in Noonan Syndrome: Does it Influence Clinical Response to GH Therapy?

Pellegrin Maria Chiara , Tornese Gianluca , Faleschini Elena , Masera Nicoletta , Pozzobon Gabriella , Selicorni Angelo , Ventura Alessandro

Background: Short stature is a main feature of Noonan syndrome (NS). Although rhGH is commonly used in NS patients, it is not known whether a defect in the secretion of GH influences the response to rhGH therapy.Objective and hypotheses: The aim of this study was to evaluate the efficacy and safety of rhGH treatment in NS patients, according to the presence of GH deficiency at the baseline.Method: We retrospectively collected data ...

hrp0082p2-d3-392 | Fat Metabolism &amp; Obesity (2) | ESPE2014

HbA1c and Metabolic Parameters in a Pediatric Overweight/Obese Population

Pellegrin Maria Chiara , Grillo Andrea , Faleschini Elena , Tornese Gianluca , Ventura Alessandro

Background: HbA1c was recommended as diagnostic tool in adults at risk for diabetes. In obese patients, HbA1c shows an association even with other features of metabolic syndrome. However, its value in pediatric population for this purpose has yet to be established.Material and methods: We determined HbA1c (IFCC method) in 307 overweight/obese children and adolescents (age 11.4±3.2; range 3.0–17.9 and BMI 27.9±4.7; range 20.4–47.6) val...