hrp0092p2-160 | GH and IGFs | ESPE2019

Metabolic Outcome in Adolescents with Growth Hormone Deficiency During Transition Phase

Improda Nicola , Moracas Cristina , Paolo Ciccarelli Gian , Capalbo Donatella , Salerno Mariacarolina

Background: It is well known that GH deficiency (GHD) in adulthood is associated with detrimental cardiovascular (CV) effects. Although data are controversial, adolescents with childhood-onset GHD (COGHD) and reconfirmed GHD may have increased metabolic risk after GH treatment withdrawal at final height (FH).Aim: Aim of our study is to compare growth response and metabolic profile in idiopathic COGHD adolescents with rec...

hrp0092p3-120 | Fat, Metabolism and Obesity | ESPE2019

Toward a Simple Marker of Hepato-Visceral Adiposity and Insulin Resistance:The Z-Score Change from Weight-at-Birth to BMI-in-Childhood

Malpique Rita , de Zegher Francis , Garcia-Beltran Cristina , Ibáñez Lourdes

Insulin resistance and hepato-visceral (central) fat excess are thought to contribute to an earlier timing of adrenarche/pubarche and puberty/menarche; this earlier timing in turn relates often to a mismatch between pre- and postnatal weight gain, which can be estimated by calculating the Z-score change from birth weight (BW) to body mass index (BMI) in childhood. We tested the hypothesis that this calculation may serve as a proxy of insulin resistance and hepato-visceral adip...

hrp0092p3-144 | GH and IGFs | ESPE2019

A Case of Paediatric GH-secreting Pituitary Adenoma Apoplexy

Partenope Cristina , Gallo Dario , Pitea Marco , Pajno Roberta , Weber Giovanna , Barera Graziano , Cinzia Pozzobon Gabriella

Background: Paediatric pituitary adenomas comprise rare but challenging pathologies in children and adolescents related to their endocrine and neurological characteristics.Objective and Hypotheses: We aimed to describe a case report of growth hormone (GH)-secreting pituitary adenoma apoplexy.Method: a 11-year-old girl presented with fever, sudden headache with vomiting. She abruptl...

hrp0092p3-205 | Pituitary, Neuroendocrinology and Puberty | ESPE2019

Childhood Craniopharyngioma: A Single Centre Experience

Gallo Dario , Partenope Cristina , Pajno Roberta , Pitea Marco , Weber Giovanna , Barera Graziano , Pozzobon Gabriella Cinzia

Backround: Craniopharingiomas are rare embryogenic malformations of the sellar and parasellar region with slow growth and high local recurrence rate. Due to their unfavorauble location, presurgical hypothalamic involvement and treatment-related hypothalamic damage is frequent and lead to hypothalamic syndrome.Objective: we compare weigh gain and growth pattern regarding to hypothalamic involvement of pediatric patients a...

hrp0089p1-p064 | Diabetes & Insulin P1 | ESPE2018

Efficacy of Mecasermin Treatment and Long-Term Survival in a Child with Leprechaunism

Maggio Maria Cristina , Barbetti Fabrizio , Vitaliti Marcello , Ragusa Saveria Sabrina , Vitaliti Giuliana , Corsello Giovanni

Homozygous mutation of Insulin receptor (INS-R) gene cause an extremely rare disease called Leprechaunism, and induce intrauterine growth restriction with poor postnatal growth, hyperinsulinemia, postprandial hyperglycaemia, pre-prandial hypoglycaemia, typical facies, lack of subcutaneous fat, thick skin, hypertrichosis, macrogenitosomia in males. The survival is severely compromised in these patients. Treatment with diazoxide could ameliorate glycaemic control, however these ...

hrp0089p3-p108 | Diabetes & Insulin P3 | ESPE2018

Psychosocial Risks, Comorbidities and Health Events During the Follow-up of Children and Adolescents with Type 1 Diabetes

Temboury Ma Carmen , Villamor Raquel , Hernandez Ma Belen , Lapena Sara , Lopez Cristina

Background: Psychosocial factors and health events are important for patients with chronic diseases such type 1 diabetes (TD1).Objective and hypotheses: The aim was to explore the psychosocial factors, health events and comorbidity found at diagnosis and during the follow up of children and adolescents with type 1 diabetes. Also to analyze the association between socioeconomic status and glycaemic control.Method: Medical charts of ...

hrp0089p2-p161 | Fat, Metabolism and Obesity P2 | ESPE2018

Metabolic Alterations and Weight Status in Children at 8 Years: A Prospective Cohort Study

Riano-Galan Isolina , Fernandez-Somoano Ana , Rodriguez-Dehli Cristina , Venta Obaya Rafael , Tardon Adonina

Background: Prevalence of childhood obesity (OB) represents a major public health concern, given the tracking of body weight from childhood to adult age and obesity-related morbidity.Objective: To describe prevalence of overweight (OW) and OB in children at 8 years and investigate relationship with metabolic alterations (lipid profile and insulin resistance).Methods: 485 pregnant mothers recruited between 2004-2007 and 409 children...

hrp0089p2-p259 | Growth & Syndromes P2 | ESPE2018

Unusual Case of Combination of Beckwith-Wiedemann Syndrome and SHOX Gene Deficiency

Cassano Gilda , Osimani Sara , Pajno Roberta , Pitea Marco , Partenope Cristina , Russo Silvia , Pozzobon Gabriella

Background: Beckwith-Wiedemann Syndrome (BWS) is an overgrowth disorder involving a predisposition to tumor development, etiologically connected with genetic/epigenetic dysregulation. The main features of BWS include omphalocele, macroglossia and macrosomia; however there is significant clinical heterogeneity. SHOX mutation is a frequent cause of short stature with high penetrance, but extremely variable clinical expression. The mean adult height is -2.2 SDS. The presence of m...

hrp0089p3-p347 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology P3 | ESPE2018

Genital Swelling and Ovarian Stimulation Syndrome in an Extremely Preterm Infant

Cristina Azcona Maria , Ochotorena Amaia , Prados Monica , Luis Leon Jose , Alzina Valentin

Introduction: Ovarian stimulation syndrome (OSS) in an infrequent disorder, in preterm infants under 30 weeks gestation. The are very few cases described in the literature. The immaturity, lack of gonadal axis feed-back or mutations in the FSH receptor genes, may explain its physiopathology. We present the case of a 26-week neonate with this syndrome.Case summary: 26-week newborn with extremely low weight (460g) born by emergency cesarean section due to ...

hrp0089p3-p383 | Thyroid P3 | ESPE2018

Corticosteroid Resistant Immune Thrombocytopenic Purpura, is it a Marker of Future Graves Disease?

Azcona Maria Cristina , Leon Jose Luis , Prados Monica , Ochotorena Amaia , Oliver Asier

Introduction: The Immune Thrombocytopenic Purpura (ITP) and Graves Disease (GD) have in common an autoinmune physiopathology. ITP is characterized by a platelet count less than 100 × 106/l in the absence of other cause. On one hand, ITP has been associated with thyroid dysfunction, without developing GD. On the other hand, GD might develop with moderate thrombocytopenia, generally more than 100×109/l. It is described that in most cases ITP might...