hrp0084p3-773 | Diabetes | ESPE2015

Mauriac Syndrome, a Rare Complication of Type 1 Diabetes Mellitus

Rivero-Martin Maria Jose , Perez-Segura M Pilar , Alcazar-Villar Maria Jose , Montes-Bentura David , Milian M Eugenia Oros

Background: Mauriac syndrome (MS) classically involves hepatomegaly (hepatic glycogenosis), growth impairment and cushingoid features in a patient with poorly controlled type 1 diabetes mellitus (T1DM). The typical age of presentation is adolescence. With the advent of improved insulin regimens, MS has seen less frequently. However, new cases appear each year in medical literature.Clinical case: We report the case of a 9 years and 9 months of age male, w...

hrp0084p3-964 | GH & IGF | ESPE2015

Evaluation of the Facility of Use of a New GH Administration Device – Study DAGH2014

Rivero-Martin Maria Jose , Ontanon-Nasarre Ana , Alcazar-Villar Maria Jose , Carrasco-Torrents America , Andres-Rosado Ana , Montes-Bentura David

Background: One of the limiting factors in adherence to GH therapy, is satisfaction with the administration device used.Objective and hypotheses: With the emergence of a new biosmiliar GH (BGH) administration device, we will assess the simplicity, ease of use, management and pain perceived by the patients.Method: Prospective through survey caregivers and children treated with BGH at least for 6 months. Signed informed consent was r...

hrp0094fc5.1 | Sex Development and Gender Incongruence | ESPE2021

Growth and growth reduction in transgirls

Boogers Lidewij S , Wiepjes Chantal M , Hellinga Ilse , Klink Daniel T , den Heijer Martin , Hannema Sabine E ,

Introduction: Little is known about the effects of puberty suppression (PS) and hormone therapy (HT) on growth and adult height in transgender adolescents. These are topics of interest since height differs between sexes and some transgirls wish to limit their growth. In this longitudinal cohort study, we investigated the influence of PS and HT on growth and the efficacy of growth reduction therapy in transgender girls.Methods:</s...

hrp0094fc7.4 | Fetal Endocrinology and Multisystem Disorders | ESPE2021

Endocrine sequelae in 157 paediatric survivors of haematopoietic stem cell transplantation

Guemes Maria , Martin-Rivada Alvaro , Arribas Marta Bascuas , Andres Esteban Eva Maria , Angulo Blanca Molina , Argente Jesus ,

Introduction: As successful rates of haematopoietic stem cell transplantation (HSCT) are on the rise, late endocrine and metabolic effects of these survivors have become more prevalent.Aims: We aimed to characterise the endocrine abnormalities observed following HSCT.Subjects and methods: A retrospective descriptive study in paediatric post-HSCT patients (< 18 years-old) follow...

hrp0094p2-13 | Adrenals and HPA Axis | ESPE2021

Utilisation Of The I-CAH Registry As A Tool For Facilitating Health Quality Improvement Strategies- Results Of An International Survey Of Specialist Centres

Ali Salma R. , Bryce Jillian , McMillan Martin , O’Connell Michele , Davies Justin H. , Ahmed S. Faisal ,

Background: Recent studies using data from the International Congenital Adrenal Hyperplasia (CAH) Registry (www.i-cah.org) (1), have provided valuable insight into the determinants of reported acute adrenal insufficiency (AI) related adverse events, demonstrating that the I-CAH Registry can be used as a tool to create clinical benchmarks in CAH care.Methods: Care quality reports were created for 32 centres who participat...

hrp0094p2-218 | Fat, metabolism and obesity | ESPE2021

Impact of overweight and obesity in pediatrics. Metabolic syndrome and its components

San Martin Amaya , Vilella Leyre , Garcia Casales Zurine , Calvo Ariadne , Rocha C , Sarasua Miranda Ainhoa , Diez-Lopez Ignacio ,

The current lifestyle, with a diet increasingly removed from the Mediterranean diet, together with the habitual sedentary lifestyle, is associated with many of the so-called non-communicable diseases, such as the metabolic syndrome. Every day these pathologies are more frequent in pediatrics, so this study has tried to establish the prevalence of metabolic syndrome and its components in overweight and obese children and assess its impact. METHODS A descriptive study has been c...

hrp0094p2-248 | Growth hormone and IGFs | ESPE2021

Reduction in pappalysin and stanniocalcin levels explain the decrease in IGF-I bioavailability in anorexia nervosa

Martin-Rivada Alvaro , Guerra-Cantera Santiago , Campillo-Calatayud Ana , Camarneiro Ricardo , Graell Montserrat , Barrios Vicente , Argente Jesus ,

Background: Pappalysins (PAPP-A, PAPP-A2) and stanniocalcins (STC-1, STC-2) are novel regulators of the GH-IGF axis, modulating IGF bioavailability through control of cleavage of IGFBPs and release of IGF from the ternary complex. Changes in the peripheral IGF system, including low IGF concentrations independently of GH secretion, have been reported in scenarios of malnutrition such as anorexia nervosa (AN). The role of pappalysins and stanniocalcins in the pa...

hrp0097t20 | Section | ESPE2023

Hyperparathyroidism is associated with inferior event free survival in lymphatic childhood malignancies in a single center retrospective analysis

Grasemann Corinna , Höppner Jakob , Tippelt Stephan , Grabow Desiree , Cario Gunnar , Zimmermann Martin , Reinhardt Dirk , M Schündeln Michael

Introduction: Vitamin D status is investigated as a modifier for the risk and course of multiple malignant diseases. Findings point towards a detrimental role of low vitamin D levels for event free and overall survival (EFS/OS). However, vitamin D deficiency is often associated with secondary hyperparathyroidism, which may influence EFS/OS in childhood malignancies.Methods: Real world data from 1547 cases (873 male) of c...

hrp0097p1-4 | Adrenals and HPA Axis | ESPE2023

Major importance of genetic background in cortisol metabolism: Salivary diurnal glucocorticoid profiles in monozygotic twins with intra-twin birthweight-differences

Schulte Sandra , Eberhardt Nora , Roedig Thea , Schreiner Felix , Plamper Michaela , Bartmann Peter , Holterhus Paul-Martin , Kulle Alexandra , Gohlke Bettina

Objective: Although low birthweight (bw) and unfavourable intrauterine conditions have been associated with metabolic sequelae in later life, little is known about their impact on glucocorticoid metabolism. We studied monozygotic twins with intra-twin bw-differences from birth to adolescence to analyse the long-term impact of bw and catch-up growth on glucocorticoid metabolism.Methods: 46 monozygotic prepubertal twin-pai...

hrp0097p2-166 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2023

Value of antimullerian hormone (AMH) in the diagnosis of precocious puberty: revaluation of the covid-19 post-pandemic cohort

Diez-Lopez Ignacio , Aurora Vilella San Martín Leyre , Sarasua Miranda Ainhoa , San Martin Orayen Amaia , Maeso Mendez Sandra

During the pandemic, an increase in the cases of premature thelarche consultations was observed. Our group published a series in 2021 of 75 cases. This effect could have been caused by an increase in body fat mass in girls, suggesting a “trigger effect” in the activation of the gonadal axis, causing an increase in the number of cases of early puberty (SEEP Oviedo 2022). AMH has been correlated as a predictor of the moment of menarche (doi: 10.1515/jpem-2014-0521)</...