hrp0084p2-425 | GH & IGF | ESPE2015

Comparison of Baseline Parameters and Response to GH Treatment in 125 Children with Short Stature with Eight Different Diagnosis

Ioimo Irene , Mussa Alessandro , Vannelli Silvia , Feyles Francesca , Einaudi Silvia , Matarazzo Patrizia

Background: Response to rhGH treatment is variable among GH deficiency (GHD), GH insensitivity and several intermediate conditions.Aims and objectives: To compare baseline parameters and response to rhGH treatment in eight diagnostic categories of patients with short stature.Methods: We selected 125 prepubertal children presenting at least 2 years of rhGH treatment (mean 5.29 years, range 2–15.6 years), hormonal and clinic par...

hrp0084p3-951 | GH & IGF | ESPE2015

Predictors of Response to rhGH Treatment in 125 Children with Short Stature of Various Aetiologies

Ioimo Irene , Mussa Alessandro , Vannelli Silvia , Verna Francesca , Borraccino Alberto , Matarazzo Patrizia

Background: Response to rhGH treatment is extremely variable in pediatric growth disorders; predictors of the response are not yet clearly determined, due to disomogeneity of studied cohorts.Aims and objectives: To investigate the correlation between clinical parameters and height gain after the 1st year of rhGH treatment and at the last visit in eight different aetiologies of short stature, with the aim of identifying predictors of response to rhGH trea...

hrp0084p3-1105 | Pituitary | ESPE2015

Off-label Use of Vaptans in Children with Severe Symptomatic Hyponatremia due to SIADH

Tuli Gerdi , Tessaris Daniele , Di Taranto Serena , Giorgis Alberto , Einaudi Silvia , Matarazzo Patrizia

Background: Vaptans, vasopressin receptor 2 antagonist, are used in adults to treat hyponatremia associated with congestive heart failure, cirrhosis, and the syndrome of inappropriate antidiuretic hormone (SIADH). To date, in paediatric patients with SIADH there are few data about use of vaptans, still considered off-label.Case presentation 1: A 9-yo female with surgically treated suprasellar astrocitoma developed chronic hyponatremia (121–128 mmol/...

hrp0094p1-93 | Thyroid A | ESPE2021

"Lympocyte population in children and adolescents affected by Graves’ disease. Potential predictive tool of disease severity"

Tuli Gerdi , Munarin Jessica , Tessaris Daniele , Matarazzo Patrizia , De Sanctis Luisa ,

Introduction: Graves-Basedow disease (GD) is the most common cause of thyrotoxicosis in pediatric age. Multi-factorial pathogenesis stand on the basis of the autoimmune disorder activation, leading to lymphocyte imbalance that include increased CD4+ and CD8+ subtypes, increased CD4+/CD8+ ratio and B cells dysregulation. The aim of this study is to determine the potential prognostic value of lymphocyte population parameters in pediatric GD.<p class="abstext...

hrp0094p2-400 | Sex differentiation, gonads and gynaecology or sex endocrinology | ESPE2021

Peripheral Precocious Puberty in girls affected by McCune-Albright Syndrome: safety and efficacy retrospective study on letrozole treatment

Tessaris Daniele , Gazzin Andrea , Bonino Elisa , Tuli Gerdi , Matarazzo Patrizia , de Sanctis Luisa ,

Introduction: Precocious Peripheral Puberty (PPP) due to autonomous ovarian activation is the most frequent endocrinopathy in girls with McCune-Albright Syndrome (MAS). Main goals of treatment for PPP are to reduce signs of estrogen exposure, also improving final height, and mitigate secondary psychological influence of sexual hormones exposure. This retrospective study analyzes the long-term safety and efficacy of letrozole treatment in a cohort of girls with...

hrp0089p1-p189 | Multisystem Endocrine Disorders P1 | ESPE2018

Clinical Features and Assessment of the Pathway-Care Proposed by ISPED-Gsa Study Group in an Pediatric Italian Cohort with Pseudohypoparathyroidism

Tessaris Daniele , Bonino Elisa , Matarazzo Patrizia , Tuli Gerdi , Wasniewska Malgorzata , Loche Sandro , Weber Giovanna , de Sanctis Luisa

Background and objective: Pseudohypoparathyroidism (PHP) refers to a heterogeneous group of rare endocrine disorders caused by genetic or epigenetic abnormalities affecting the GNAS locus. It is mainly characterized by resistance to PTH and TSH and a complete or partial Albright Hereditary Osteodistrophy (AHO) phenotype. Few data so far exist on LH/FSH, calcitonin and glucose-lipid metabolism involvement, as well as on neurocognitive aspects. The aim of this multicentre study ...

hrp0082p3-d3-786 | Fat Metabolism &amp; Obesity (2) | ESPE2014

Hypothalamic Obesity in Children and Adolescents: a Multi-Disciplinary Approach and Novel Therapeutic Tools

Tessaris Daniele , Tuscano Antonella , Rabbone Ivana , Lezo Antonella , Fenocchio Giorgia , Broglio Fabio , Spinardi Alessandra , Lala Roberto , Matarazzo Patrizia

Background: Hypothalamic obesity (HO), due to midline congenital malformations, genetic diseases or hypothalamo-hypophysis tumours, is severe and difficult to treat. Patients are scarcely compliant to diet and physical activity, for disabilities often affecting them. Drugs have been rarely employed.Objective and hypotheses: Aim of the study was to treat hypopituitaric children and adolescents affected by severe HO with a multidisciplinary approach includ...

hrp0097p2-4 | Adrenals and HPA Axis | ESPE2023

Newborn screening for 21 OH Congenital adrenal hyperplasia in Italy: a 14 years population study.

Baronio Federico , Abrigo Enrica , Azzolini Sara , Cavarzere Paolo , Matarazzo Patrizia , L.C. Meroni Silvia , Russo Gianni , Balsamo Antonio , Cassio Alessandra

Introduction: Early identification of classic 21OH-Congenital Adrenal Hyperplasia (21OH-CAH) through newborn screening (NBS) is crucial to prevent adrenal crises, especially in males. Today 21 OH-CAH NBS is performed in 5/21 regions of Italy. This study aims to report the results of 21OH-CAH NBS in Italy from 2006 to 2019.Methods: All patients underwent a dried blood spot (DBS) test for 17OH-progesterone (17OHP) within t...

hrp0092p1-106 | Pituitary, Neuroendocrinology and Puberty | ESPE2019

Management and Treatment Outcome of Craniopharyngiomas in Young Children Before 4 Years of Age in Italy: Multicentre Collection of 16 Cases

Baronio Federico , Zucchelli Mino , Aversa Tommaso , Driul Daniela , Guzzetti Chiara , Iughetti Lorenzo , Matarazzo Patrizia , Parpagnoli Maria , Pedicelli Stefania , Pozzobon Gabriella , Salerno Mariacarolina , Zucchini Stefano

Introduction: Craniopharyngiomas (CP) are rare pediatric intracranial tumors (1.2-4%) with a peak of incidence between 5-14 years . We retrospectively reviewed data of 16 cases (M/F 4/12) diagnosed before 4 years of age (median follow up 7.2 years) from a cohort of 117 patients (pts) (M/F 56/41) diagnosed after 01/01/2000, followed-up in 14 Italian centres of pediatric endocrinology belonging to the Italian Society for Pediatric Endocrinology and Diabetolo...

hrp0086p2-p950 | Thyroid P2 | ESPE2016

The Evolution of Thyroid Function after Hashimoto’s Thyroiditis Presentation is Different in Initially Euthyroid Girls with or without Turner Syndrome

Wasniewska Malgorzata , Salerno Mariacarolina , Corrias Andrea , Mazzanti Laura , Matarazzo Patrizia , Corica Domenico , Aversa Tommaso , Messina Maria Francesca , De Luca Filippo , Valenzise Mariella

Background: Hashimoto’s thyroiditis (HT) is the commonest autoimmune disorder in Turner syndrome (TS). Although there are in the pediatric literature many studies on the relationships between TS and HT, only few of them have specifically investigated whether the association with TS might be able to significantly affect the evolution over time of thyroid function in children and adolescents with HT, by conditioning a different thyroid status prognosis.<p class="abstext...