hrp0092rfc3.5 | Multi-system Endocrine Disorders | ESPE2019

Evaluation of Endothelial Function in Childhood Standard Risk Acute Lymphoblastic Leukemia Survivors: Role of Subclinical Markers and Identification of Preventable Factors

Bruzzi Patrizia , Bigi Elena , Felici Francesca , Righi Beatrice , Cano Carmen , Cellini Monica , Predieri Barbara , Iughetti Lorenzo

Background: Adult survivors from childhood malignancy are prone to accelerated atherogenesis and cardiovascular (CV) complications. In this population reliable tools are needed to detect preclinical onset of CV disease.Aim: To assess subclinical markers of inflammation and endothelial dysfunction in young survivors from acute lymphoblastic leukemia (ALL) treated with chemotherapy without cranial irradiation (AIEOP 2000 a...

hrp0092p1-202 | Fat, Metabolism and Obesity (1) | ESPE2019

Visceral Adiposity Index as a Marker of Metabolic Risk in Survivors of Paediatric Hematopoietic Stem Cell Transplantation After Chemotherapy-Only Conditioning

Cima Luminita-Nicoleta , Nedelea Lavinia , Soare Iulia , Barbu Carmen Gabriela , Zaharia Cristina , Colita Anca , Fica Simona

Background: Hematopoietic stem cell transplantation (HSCT) recipients exhibit excess adiposity that may result in an increased metabolic risk. Studies have shown that BMI is a poor predictor of body fatness in pediatric HSCT survivors population where diminished lean mass has been documented. The visceral adiposity index (VAI) has recently been proposed as a predictor of cardio-metabolic risk in both adults and children. However, the predictive value of this i...

hrp0092p2-228 | Pituitary, Neuroendocrinology and Puberty | ESPE2019

Near Adult Height According to Genetic Target and Absence Of Craniofacial Bone Fibrous Dysplasia in a Girl With Mc Cune Albright Syndrome and Growth Hormone Excess: 12.6 Years Follow-up

Fernández Mentaberry Verónica , Riu Carmen , Oneto Adriana , Stivel Mirta

Background: Mc Cune Albright (MAS), is a rare genetic disease clinically defined by bone fibrous dysplasia (BFD), café au lait skin spots and at least one hyperfunction endocrinopathy. Growth Hormone (GH) excess has been described in 20% of patients usually accompanied by hyperprolactinemia (80%). As reported in literature GH hypersecretion is allways associated with craniofacial BFD, macrocephaly and is also accompanied by higher risk of systemic...

hrp0089p1-p037 | Bone, Growth Plate & Mineral Metabolism P1 | ESPE2018

Hypercalcaemia after Treatment with Denosumab in Children: Bisphosphonates as an Option for Therapy and/or Prevention?

Sydlik Carmen , Weissenbacher Claudia , Roeb Julia , Roland Durr Hans , Bechtold-Dalla Pozza Susanne , Schmidt Heinrich

Background: Pharmacologic options for treatment of osteolytic diseases especially in children are limited. Although not licensed for use, denosumab, a fully humanized antibody to RANKL, is used in children and shows good effects. Among others, one indication are giant cell tumors of the bone. Yet, there are reports of severe hypercalcemia after stop of denosumab, an adverse effect which is rarely seen in adults.Case reports: Four patients, aged 6, 13 and...

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 ...

hrp0089lb-p11 | Late Breaking P1 | ESPE2018

Metabolic Profile in Survivors of Pediatric Hematopoietic Stem Cells Transplantation after Chemotherapy-Only Conditioning

Cima Luminita-Nicoleta , Nedelea Lavinia , Zaharia Cristina , Colita Anca , Barbu Carmen Gabriela , Fica Simona

Background: Metabolic syndrome (MS) is a long-term complication of pediatric haematopoietic stem cell transplantation (HSCT) and it was described more often in patients who were exposed to total body irradiation (TBI). Since previous studies reported discrepancy in the presence of metabolic complications in HSCT survivors who underwent chemotherapy-only conditioning, we investigated the frequency of MS in our HSCT-treated children for various disorders without being exposed to...

hrp0086p1-p141 | Bone & Mineral Metabolism P1 | ESPE2016

Off-Label Use of the Aromatase Inhibitor Letrozole in Pubertal Boys to Improve Final Height: Laboratory, Auxological and Bone Age Data

Schmidt Heinrich , Weissenbacher Claudia , Bechtold-Dalla Pozza Susanne , Roeb Julia , Sydlik Carmen

Background and aims: The aromatase inhibitor letrozole is used to improve final height in boys with predicted short stature by delaying bone age (BA) maturation due to suppression of estradiol levels. There are few data regarding its effects, especially when used as single medication.Methods: Ten pubertal boys with predicted low final height treated with letrozole 2.5 mg/d p.o. (no other medication) for up to 24 months were analysed for auxological, labo...

hrp0086p2-p182 | Bone & Mineral Metabolism P2 | ESPE2016

Primary Hyperparathyroidism- A Cause of Metabolic Syndrome in Children?

Ioana Arhire Amalia , Pavel Madalina , Florea Suzana , Miron Adrian , Gabriela Barbu Carmen

Introduction: We report a case of a 15 year old male with metabolic syndrome due to primary hyperparathyroidism.Case report: A 15 year old male was admitted in our department for the evaluation and management of obesity. His medical history revealed a progressive weight gain in the last 3 years in an emotional familial context. Clinical features: Obesity: Z-score of +2.32 DS, a height of 1.80 m and a weight of 108 kg, with a BMI of 33.3...

hrp0086p1-p251 | Diabetes P1 | ESPE2016

Metabolic Impairments among Adult Survivors of Paediatric Abdominal and Pelvic Tumours in the St Jude Lifetime Cohort Study

Wilson Carmen , Chemaitilly Wassim , Liu Wei , Srivastava Deokumar , Hudson Melissa , Robison Leslie , Ness Kirsten

Background: Adverse changes in metabolic parameters and body composition are frequently reported among childhood cancer survivors treated with cranial or total body irradiation. Data regarding the occurrence of metabolic impairments among survivors following abdominal and pelvic radiation are lacking.Objective and hypotheses: To define the prevalence of metabolic impairments among survivors of paediatric abdominal and pelvic solid tumours and to assess t...

hrp0086p2-p668 | Growth P2 | ESPE2016

A Rare Cause of Growth Delay: Jacobsen Syndrome

Constantinescu Georgiana , Belceanu Alina , Bursuc Anamaria , Armasu Ioana , Braha Elena , Rusu Cristina , Mogos Voichita , Vulpoi Carmen

Background: Jacobsen syndrome is a rare genetic condition caused by partial deletion of the long arm of chromosome 11 associated with delayed development, distinctive facial features, bleeding disorder, skeletal abnormalities and endocrine disorders.Case report: We report a rare case of Jacobsen syndrome in 4 year old boy addressed for short stature. Born at term (36 W) with low birth weight (1780 g) and delayed development, his height at presentation wa...