hrp0084p1-46 | Diabetes | ESPE2015

Trends in Insulin Therapy in 50 861 Children and Adolescents with Type 1 Diabetes from Austria and Germany Between 2000 and 2014

Bohn Barbara , Wiegand Susanna , Kummer Sebastian , Menzel Ulrike , Kordonouri Olga , Bottcher Claudia , Frohlich-Reiterer Elke , Holl Reinhard W.

Background: Over the last two decades, treatment of type 1 diabetes became more intensified and changes in the type of insulin used were reported.Objective and hypotheses: We hypothesised that there are also changes in insulin dosage and in the ratio of prandial to basal insulin. Our aim was to analyse potential trends in paediatric subjects with type 1 diabetes from Austria and Germany between 2000 and 2014.Method: 50 861 subjects...

hrp0089fc14.1 | Multisystem Endocrine Disorders | ESPE2018

Awareness and Participation in Rare Disease Registries Within the European Reference Network on Rare Endocrine Conditions (Endo-ERN)

Ali Salma R , Bryce Jillian , Cools Martine , Korbonits Marta , Beun Johan G , Taruscio Domenica , Beuschlein Felix , Danne Thomas , Dattani Mehul , Dekkers Olaf , Linglart Agnes , Netchine Irene , Nordenstrom Anna , Patocs Attila , Persani Luca , Smyth Arlene , Sumnik Zdenek , Visser W Edward , Hiort Olaf , Pereira Alberto M , Ahmed S Faisal

Background: Registries are of key importance for a centre of expertise. Endo-ERN consists of 71 reference centres (RCs) that cover several groups of rare endocrine conditions within 8 themes (www.endo-ern.eu). It is unclear if awareness, participation and availability of registries is uniform for all conditions within Endo-ERN.Objective: To determine the extent of engagement in registries of Endo-ERN members.<p cl...

hrp0094p2-111 | Diabetes and insulin | ESPE2021

Prevalence of skin reactions among pediatrics patients with Type 1 Diabetes users of glucose sensors.

German Angulo Paloma , Del Valle Ferreras Marcos , Cristina Lopez Menau Maria , Lopez Sanchez Belen , Luna Bastante Loreto , Remedios Mateo Laura , Corredor Andres Beatriz ,

Introduction: Glucose sensors are medical devices used in glycaemic control of patients with type 1 diabetes (T1D) . Its use prevents multiple capillary measurements, improving disease control and quality of life. However, these systems are not exempt from unforeseen events. The rise in the number of users has resulted in the increasing detection of local skin reactions by these devices, being the most frequent the eczematous ones, which may correspond to irri...

hrp0092p1-167 | Adrenals and HPA Axis (1) | ESPE2019

Development of an International Benchmark for Sick Day Episodes as a Core Clinical Outcome in People with Congenital Adrenal Hyperplasia

Ali Salma , Daniel Eleni , Bryce Jillian , Ikiroma Adalia , Lewsey James , Ross Richard , Krone Ruth , Acerini Carlo , Krone Nils , Das Urmi , Tomlinson Jeremy , Korbonits Marta , Higham Claire , Darendeliler Feyza , Guran Tulay , Guven Ayla , Attapatu Navoda , Milenkovic Tatjana , Raducanu-Lichiardopol Corina , Hannema Sabine , Claahsen Hedi , Finken Martijn , Baronio Federico , Balsamo Antonio , Einaudi Silvia , de Vries Liat , Luczay Andrea , Neumann Uta , Blankenstein Oliver , Mohnike Klaus , Bonfig Walter , Elsedfy Heba , Birkebaek Niels , Iotova Violeta , Bachega Tania , Mendonca Berenice , Cools Martine , Costa Eduardo Correa , Filho Guilherme Guaragna- , Rey Rodolfo , Ahmed S. Faisal

Background: Congenital adrenal hyperplasia (CAH) is a rare condition characterised by adrenal insufficiency and a life-long risk of adrenal crises. There is a paucity of information on the epidemiology of acute adverse events in this population.Objective: To investigate the frequency, aetiology and consequences of acute adverse events attributed to adrenal insufficiency in CAH.Methods</stro...

hrp0086s9.1 | Recent advances in congenital adrenal hyperplasia | ESPE2016

Long-term Health in Congenital Adrenal Hyperplasia: Lessons from a National Study

Nordenstrom Anna

Congenital adrenal hyperplasia (CAH) is lethal in its most severe forms if not treated with glucocorticoids. However, glucocorticoids may increase the cardiovascular and metabolic morbidity. The long term outcome in CAH was studied using the Swedish national CAH registry, 588 patients 335 females and 253 males, >80% with known severity of CAH; were compared with 100 controls per patient matched for sex, and year and place of birth. Information on mortalty, cause of death, ...

hrp0094p1-8 | Adrenal A | ESPE2021

Long-term Cardiometabolic Morbidity In Young Adults With Classic 21-Hydroxylase Deficiency Congenital Adrenal Hyperplasia

Righi Beatrice , Ali Salma R , Bryce Jillian , Tomlinson Jeremy W , Bonfig Walter , Baronio Federico , Costa Eduardo C , Filho Guilherme Guaragna , T’Sjoen Guy , Cools Martine , Markosyan Renata , Bachega Tania A S S , Miranda Mirela C , Iotova Violeta , Falhammar Henrik , Ceccato Filippo , Stancampiano Marianna R , Russo Gianni , Vukovic Rade , Giordano Roberta , Mazen Inas , Guven Ayla , Darendeliler Feyza , Poyrazoglu Cukran , Vries Liat de , Ellaithi Mona , Daniel Eleni , Johnston Colin , Hunter Steven J , Carroll Paul V , Adam Safwaan , Perry Colin G , Kearney Tara , Abraham Prakash , Rees D Aled , Leese Graham P , Reisch Nicole , Stikkelbroeck Nike M M L , Auchus Richard J , Ross Richard J , Ahmed S Faisal ,

Background: Congenital adrenal hyperplasia (CAH) and long-term glucocorticoid treatment may be associated with an increased risk of developing cardiometabolic sequelae such as abnormal glucose homeostasis, hyperlipidaemia, hypertension, cardiovascular (CV) disease, obesity and osteoporosis.Objectives: To study the current practice amongst expert centres for assessing cardiometabolic outcomes in adult patients with 21-hyd...

hrp0086p1-p379 | Gonads &amp; DSD P1 | ESPE2016

Health-Related Quality of Life and Psychological Wellbeing in Adults with Diverse Sex Development

Bennecke Elena , Thyen Ute , Gruters Annette , Lux Anke , Kohler Birgit

Background: Rare congenital conditions with incongruence of chromosomal, gonadal, and phenotypic sex have been summarized as disorders of sex development (DSD). Included in DSD are conditions with diverse genetic etiology, varying levels of prenatal androgen effects, phenotypes, and subsequently, different medical treatments. Quality of life (QoL) and psychological wellbeing are indicators of successful psychosocial adaptation to the conditions. Studies addressing these issues...

hrp0084fc3.4 | Diabetes | ESPE2015

Wolfram Syndrome: Natural History and Genotype–Phenotype Correlation Based on EURO-WABB Registry Show Gender Differences in Disease Severity

Dias Renuka , Richens Caitlin , Astuti Dewi , Nightingale Peter , Ayme Segolene , Heredia Miguel Lopez de , Nunes Virginia , Maffei Pietro , McCafferty Susan , Młynarski Wojciech , Parkinson Kay , Paquis-Flucklinger Veronique , Rohayem Julia , Sinnott Richard , Tillmann Vallo , Tranebjaerg Lisbeth , Barrett Timothy

Background: Wolfram syndrome (WS) is a rare autosomal recessive disorder, characterised by early-onset diabetes and optic atrophy. It is caused by mutations in WFS1.Objective and hypotheses: This study aimed to comprehensively review the natural history of WS in a large cohort of patients from the EURO-WABB registry.Method: Data from EURO-WABB patients with WS was analysed in conjunction with the Leiden Open Variation Data...

hrp0089p1-p072 | Diabetes &amp; Insulin P1 | ESPE2018

Increasing Use of Continuous Glucose Monitoring (CGM) Among Youth with Type 1 Diabetes (T1D): Icomparison of Youth from the T1D Exchange (T1DX) and the DPV Initiative

Miller Kellee , Hermann Julia , Maahs David , Hofer Sabine , Foster Nicole , Holl Reinhard

Objectives: To assess change in rates of pediatric CGM use over the past 5 years across demographic and clinical characteristics and association with hemoglobin A1c (HbA1c), data from 2 registries were compared: the US-based T1DX and the German/Austrian DPV.Methods: Registry participants in DPV and T1DX aged <18yrs with T1D duration ≥ 1yr with available data in any of the following years were included in the analysis (N for each year by registr...

hrp0084p2-335 | Fat | ESPE2015

Network Coordinated Primary Care Intervention in Obese Children and Adolescents: Almost a Decade of Experience

Guilmin-Crepon Sophie , Arsan Amine , Boizeau Priscilla , Alberti Corinne , Fidalgo Laurent , Chevallier Bertrand , Lubelski Patricia , Cheymol Jacques , Zaluski Alain , Tounian Patrick , Carel Jean-Claude , on behalf of REPOP Ile-de-France

Background: French health authorities have developed a national program to organize outpatient care networks for the prevention and management of obesity in children and adolescents (Réseau de Prise en Charge de l’Obésité Pédiatrique (REPOP)). To date, REPOP Ile-de-France has more than 250 primary care physicians with dietitians, nurses, educators, and psychologists trained in the management of childhood obesity as part of a standard care pathway, work...