hrp0095p2-300 | Thyroid | ESPE2022

Thyroid Function and Congenital Heart Defects in Children with Down Syndrome at Hasan Sadikin General Hospital, Bandung, Indonesia

Andriyani Dwi , Novina Novina , Rayani Apandi Putria , Faisal Faisal , Budi Kuswiyanto Rahmat , Endah Rahayuningsih Sri

Introduction: Down Syndrome (DS) is a genetic disorder that has a high mortality rate and a large number of comorbidities such as hypothyroidism and heart disease. Thyroid hormones have significant effects on cardiovascular. This study aims to describe thyroid function and congenital heart defects in Down Syndrome children at dr. Hasan Sadikin General Hospital.Methods: This is a cross-sectional study. The inclusion crite...

hrp0092p3-139 | GH and IGFs | ESPE2019

Responses to Growth Hormone (GH) Therapy in Children with Short Stature with Normal GH Secretion and Slow Growth Velocity

Elawwa Ahmed , Soliman Ashraf

Background: Variability still exist about the growth response to growth hormone (GH) therapy in children with idiopathic short stature We describe the growth response to GH therapy ( 0.05 mg/kg/day) for > 2 years in 20 prepubertal children with idiopathic short stature (ISS) who had slow growth velocity ( < -1 SD), normal GH response to provocation and who were significantly shorter than their mid-parents height SDS MPHtSDS (-1 difference).<p class...

hrp0095p1-248 | Diabetes and Insulin | ESPE2022

Carbohydrate counting efficacy in glycaemic control among children and adolescents with type 1 diabetes mellitus: a systematic review and meta-analysis

Amalia Gassani , Faisal Putro Utomo Muhammad , Aman Bhakti Pulungan

Background: Carbohydrate counting is an essential part of comprehensive diabetes management and is required for children and adolescents with type 1 diabetes mellitus (T1DM), according to available guidelines. However, there is no specific evidence-grading for carbohydrate counting. This review was done to determine the effectiveness of carbohydrate counting in improving glycaemic control among children and adolescents with T1DM as measured by glycated haemogl...

hrp0092p1-10 | Adrenals and HPA Axis | ESPE2019

Global Practice of Glucocorticoid and Mineralocorticoid Treatment in Children and Adults with Congenital Adrenal Hyperplasia – Insights from the I-CAH Registry

Bacila Irina-Alexandra , Blankenstein Oliver , Neumann Uta , van der Grinten Hedi L Claahsen , Krone Ruth , Acerini Carlo , Bachega Tania SS , Miranda Mirella C , Mendonca Berenice , Birkebaek Niels H , Cools Martine , Milenkovic Tatjana , Bonfig Walter , Tomlinson Jeremy , Elsedfy Heba , Balsamo Antonio , Hannema Sabine , Higham Claire , Atapattu Navoda , Lichiardopol Corina , Guran Tulay , Abali Zehra , Mohnike Klaus , Finken Martijn JJ , Vieites Ana , Darendeliler Feyza , Guven Ayla , Korbonits Marta , de Vrie Liat , Costa Eduardo , Einaudi Silvia , van der Kamp Hetty , Iotova Violeta , Ross Richard , Faisal Ahmed S , Krone Nils P

Introduction: Despite existing guidelines there is no unified approach to glucocorticoid and mineralocorticoid replacement in congenital adrenal hyperplasia (CAH). Consequently, treatment varies in adults and children as well as across countries.Objective: We used data from the I-CAH Registry to identify geographical and temporal variations in the treatment with glucocorticoids and mineralocorticoids of children and adul...

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...

hrp0089p1-p232 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology P1 | ESPE2018

Gonadectomy for Adults with DSD Conditions in the International Disorders of Sex Development Registry

Lucas-Herald Angela K , Kyriakou Andreas , Bryce Jillian , Rodie Martina , Acerini Carlo , Arlt Wiebke , Balsamo Antonio , Baronico Federico , Bertelloni Silvano , Brooke Antonia , Claahsen van der Grinten Hedi , Cools Martine , Darendeliler Feyza , Davies Justin H , Desloovere An , Ellaithi Mona , Fica Simona , Gawlik Aneta , Guran Tulay , Hannema Sabine , Hiort Olaf , Holterhus Paul-Martin , Iotova Violeta , Jennane Farida , Lachlan Katherine , Li Dejun , Lisa Lidka , Mazen Inas , Mladenov Wilchelm , Mohnike Klaus , Nedelea Lavinia , Niedziela Marek , Nordenstrom Anna , Poyrazoglu Sukran , Rey Rodolfo , Tadokoro-Cuccaro Rieko , Weintrob Naomi , Faisal Ahmed Syed

Introduction: Depending on the underlying diagnosis, Disorders of Sex Development (DSD) can be associated with an increased risk of germ cell cancers. To date, however, knowledge regarding the indications and timing of gonadectomy is lacking.Methods: The International-DSD (I-DSD) Registry was interrogated for anonymised information regarding the diagnosis, karyotype, sex of rearing and timing of gonadectomy, if undertaken, of all individuals of any karyo...

hrp0094fc7.1 | Fetal Endocrinology and Multisystem Disorders | ESPE2021

The Use Of e-REC For Capturing The Occurrence Of COVID-19 Infections In People With Rare Endocrine Conditions

Bryce Jillian , Di Guisto Vicki , Ali Salma R. , Alexandraki Krystallenia , Badiu Corin , Baronio Federico , Biermasz Nienke R. , Brandi Maria Luisa , Castinetti Frederic , Ceccato Filippo , Chifu Irina-Oana , Cools Martine , Danne Thomas , Druce Maralyn , Esposito Daniela , Falhammar Henrik , Fugazzola Laura , Gan Hoong-Wei , Giordano Roberta , Isidori Andrea M. , Johannsson Gudmundur , Karavitaki Niki , Linglart Agnes , Luger Anton , Maffei Pietro , Marazuela Monica , Jaksic Vladka Pandzic , Paschou Stavroula A. , Persani Luca , Domingo Manuel Puig , Reisch Nicole , Schalin-Jantti Camilla , Akker van den Erica L.T. , Vassiliadi Dimitra , Young Jacques , Appelman-Dijkstra Natasha , Grozinsky-Glasberg Simona , Pereira Alberto M. , Ahmed S. Faisal ,

Introduction: Following the onset of the COVID-19 pandemic in early spring 2020, there was a need to identify the burden of this infection on people with rare endocrine conditions. The European Registries For Rare Endocrine Conditions (EuRRECa) was launched in 2018 in collaboration with Endo-ERN, ESPE and ESE to support the needs of the wider endocrine community. The project consists of an e-reporting (e-REC) platform that allows monthly reporting of new clini...

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...

hrp0094p1-143 | Sex Endocrinology and Gonads B | ESPE2021

Current clinical practice of prenatal dexamethasone treatment in at risk pregnancies for classic 21 hydroxylase deficiency in Europe

Nowotny Hanna F. , Neumann Uta , Tardy-Guidollet Veronique , Ahmed S. Faisal , Baronio Federico , Battelino Tadej , Bertherat Jerome , Blankenstein Oliver , Bonomi Marco , Bouvattier Claire , de la Perriere Aude Brac , Brucker Sara , Cappa Marco , Chanson Philippe , Grinten Hedi L. Claahsen van der , Colao Annamaria , Cools Martine , Davies Justin H. , Gunther Dorr Helmut , Fenske Wiebke K. , Ghigo Ezio , Gravholt Claus H. , Huebner Angela , Husebye Eystein Sverre , Igbokwe Rebecca , Juul Anders , Kiefer Florian W. , Leger Juliane , Menassa Rita , Meyer Gesine , Neocleous Vassos , Phylactou Leonidas A , Rohayem Julia , Russo Gianni , Scaroni Carla , Touraine Philippe , Unger Nicole , Vojtkova Jarmila , Yeste Diego , Lajic Svetlana , Reisch Nicole ,

Background: Prenatal dexamethasone treatment (Pdex) has been used since the 1980s to prevent virilization in female offspring suspected to have congenital adrenal hyperplasia (CAH). However, due to lack of strong evidence for its best practice as well as limited data regarding long term adverse effects, use of dex is highly controversial. This study reveals the current medical practice regarding Pdex in female fetuses at risk of CAH due to 21 hydroxylase defic...