hrp0086p1-p621 | Growth P1 | ESPE2016

Final Height after Growth Hormone Treatment in Children with Chronic Renal Failure

Lonero Antonella , Bizzarri Carla , Delvecchio Maurizio , Emma Francesco , Strologo Luca Dello , Cappa Marco

Background: Growth retardation is seen in about 30% of children with chronic renal failure (CRF). Under-nutrition, anaemia, secondary hyperparathyroidism, acidosis, corticosteroid therapy and abnormalities in the GH/insulin like growth factor system have been implicated. Recombinant GH (rGH) therapy is recommended in children showing failure to maintain a normal height velocity despite optimized primary treatments.Objective and hypotheses: It has been de...

hrp0084p1-87 | Growth Hormone | ESPE2015

A Novel OTX2 Gene Mutation in a Child with Growth Hormone Deficiency

Lonero Antonella , Delvecchio Maurizio , Primignani Paola , Caputo Roberto , De alma Fabrizia , Luce Vincenza , Faienza Maria Felicia , Cavallo Luciano

Background: OTX2 is expressed in the human brain and plays a key role in the eye development. OTX2 mutations are reported in patients with ano/microphtalmia, optic nerve or optic chiasm hypoplasia, ocular coloboma and retinal dystrophies, associated in some cases with brain or pituitary abnormalities.Objective and hypotheses: Most of OTX2 mutations are nonsense or frameshift, more rarely missense mutations occur.Method: We...

hrp0084p3-813 | Endocrine Oncology | ESPE2015

Metabolic Syndrome in Childhood Acute Lymphoblastic Leukaemia Survivors

Delvecchio Maurizio , Luce Vincenza , Monteduro Mariantonietta , Giordano Paola , Muggeo Paola , Santoro Nicola , Cavallo Luciano , Faienza Maria Felicia

Background: A significant number of long-term complications are reported in childhood acute lymphatic leukemia (ALL) survivors, and among them metabolic syndrome (MetS).Objective and hypotheses: To evaluate the prevalence of features of MetS. In addition, we evaluated the presence of steatohepatitis which is described in association with MetS in otherwise healthy subjects.Method: We assessed waist circumference, triglycerides level...

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...

hrp0082p2-d1-417 | Growth Hormone | ESPE2014

GH Stimulated Levels in Prader–Willi Syndrome During the Transition Period between Childhood and Adulthood

Grugni Graziano , Corrias Andrea , Di Candia Stefania , Fintini Danilo , Gargantini Luigi , Iughetti Lorenzo , Ragusa Letizia , Salvatoni Alessandro , Sartorio Alessandro , Bocchini Sarah , Delvecchio Maurizio , Chiumello Giuseppe , Crino Antonino

Introduction: Previous reports support the hypothesis of an age dependent derangement of the hypothalamus¬Ėpituitary axis occurring in PWS subjects. In this context, transition years represent an important phase of growth process when somatic development reaches its completion. In the general population, GH deficiency (GHD) during the transition phase is associated with deterioration of body composition, metabolic alterations and reduced bone mineral density. PWS subjects ...

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: ...

hrp0095fc7.3 | Growth and Syndromes | ESPE2022

Italian long-term data on efficacy and safety of rhGH therapy in children with SHOX deficiency: the final report of a national survey performed into ISPED Growth Study Group.

Bruzzi Patrizia , Vannelli Silvia , Scarano Emanuela , Elisabeth Maria , Parpagnoli Maria , Salerno Mariacarolina , Secco Andrea , Andrea Trettene Adolfo , Wasniewska Malgorzata , Pitea Marco , Faienza Mariafelicia , Delvecchio Maurizio , Corciulo Nicola , Tornese Gianluca , Filomena Madeo Simona , Iughetti Lorenzo

Background: Among children with idiopathic short stature, Italian data reported a prevalence of short stature homeobox-containing gene (SHOX) deficiency disorders (SHOX-D) near to 1/1.000-2.000 (1.1-15%) with a wide phenotypic spectrum. Current guidelines support recombinant human growth hormone (rhGH) therapy in SHOX-D children, but long-term data are still lacking. This national survey aims to evaluate long-term efficacy and safety of rhGH therapy in Italian...

hrp0092p2-171 | GH and IGFs | ESPE2019

Final Height in GH-deficient Paediatric Patients: A Nationwide Experience

Zucchini Stefano , Lonero Antonella , Bellone Simonetta , Bozzola Mauro , Cassio Alessandra , Felicia Faienza Maria , Giacomozzi Claudio , Grandone Anna , Guzzetti Chiara , Iughetti Lorenzo , Parpagnoli Maria , Salerno Mariacarolina , Elizabeth Street Maria , Tornese Gianluca , Wasniewska Malgorzata , Delvecchio Maurizio

Background: The primary goal of GH treatment in GHD children and adolescents is to normalize height, in order to attain an adult height within the target height (TH) range.Aim: to investigate height improvement in GHD paediatric patients treated with GH in our Country.Subjects: 737 patients with isolated GHD (39.5% females) from 13 tertiary Centres for Paediatric Endocrine Care...

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...