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Endocrine Abstracts

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ea0081p259 | Adrenal and Cardiovascular Endocrinology | ECE2022

Outcome of COVID-19 infections in patients with adrenal insufficiency

Nowotny Hanna , Bryce Jillian , Giordano Roberta , Baronio Federico , Chifu Irina , Cools Martine , van den Akker Erica L.T. , Falhammar Henrik , Appelman-Dijkstra Natasha , Persani Luca , Beccuti Guglielmo , Glasberg Simona , Pereira Alberto M , Sverre Husebye Eystein , Hahner Stefanie , Ahmed Faisal , Reisch Nicole

Background: Only few cases of patients with adrenal disorders affected by coronavirus disease 2019 (COVID-19) have been reported so far. In this study, clinical outcome data of patients with adrenal disorders and COVID-19 infection has been collected by the ESE Rare Disease Committee and ENDO-ERN via the European Registries for Rare Endocrine Conditions (EuRRECa) project.Methods: This questionnaire included 32 questions on collecting quantitative and qua...
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ea0063gp187 | Adrenal and Neuroendocrine - Clinical | ECE2019

Primary adrenal insufficiency in children: results from a large nationwide cohort

Improda Nicola , Esposito Andrea , Capalbo Donatella , Cappa Marco , Ferro Giusy , Balsamo Antonio , Baronio Federico , Russo Gianni , Lascio Alessandra Di , Greggio Nella Augusta , Tosetto Ilaria , Valenzise Mariella , Wasniewska Malgorzata , Maghnie Mohamad , Radetti Giorgio , Longhi Silvia , Betterle Corrado , Salerno Mariacarolina

Background: Primary Adrenal Insufficiency (PAI) is a rare life-threatening disorder. Data on etiology and outcome of PAI in childhood are scanty, with the exception of Congenital Adrenal Hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD). The aim of our study is to evaluate etiology, morbidity and long-term outcome of PAI in a large cohort of children and characterize clinical presentation in subjects with PAI not due to 21OHD CAH.Material and me...
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ea0081p3 | Adrenal and Cardiovascular Endocrinology | ECE2022

Long-term cardiometabolic morbidity in young adults wWith classic 21-hydroxylase deficiency congenital adrenal hyperplasia

Righi Beatrice , Rashid Ali Salma , Bryce Jillian , Tolinson Jeremy , Bonfig Walter , Baronio Federico , Costa Eduardo C , Guaragna-Filho Guilherme , T'Sjoen Guy , Cools Martine , Markosyan Renata , A S S Bachega Tania , C Miranda Mirela , Iotova Violeta , Falhammar Henrik , Ceccato Filippo , Daniel Eleni , Auchus Richard , Ross Richard , Ahmed 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-hydroxylase CAH and to assess the pre...
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ea0078p1 | Adrenal | BSPED2021

Analysis of therapy monitoring in the International Congenital Adrenal Hyperplasia Registry

Lawrence Neil , Bacila Irina , Dawson Jeremy , Bryce Jillian , van den Akker Erica , Sartori Sanchez Bachega Tania Aparecida , Baronio Federico , Birkebaek Niels Holtum , Bonfig Walter , Claahsen Hedi , Correa Costa Eduardo , Devries Liat , Elsedfy Heba , Guven Ayla , Hannema Sabine , Iotova Violeta , van der Kamp Hetty J , Clemente Leon Maria , Lichiardopol Corina Raducanu , Milenkovic Tatjana , Neumann Uta , Nordenstrom Ana , Poyrazoglu Sukran , Probst-Scheidegger Ursina , De Sanctis Luisa , Thankamony Ajay , Vieites Ana , Yavas Zehra , Ahmed Faisal , Krone Nils

Background: Congenital Adrenal Hyperplasia (CAH) requires exogenous steroid replacement and can be monitored with 17-OH Progesterone and Androstenedione. We reviewed real world data to evaluate these markers in relation to hydrocortisone dose in patients treated in 21 centres throughout 14 countries.Method: Retrospective cohort study using pseudonymised data from patients with 21α-Hydroxylase Deficiency recorded in the International Congenital Adren...
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ea0090oc5.2 | Oral Communications 5: Adrenal and Cardiovascular Endocrinology 1 | ECE2023

The association between hormonal control during infancy and testicular adrenal rest tumor development in males with congenital adrenal hyperplasia

Schroder Mariska , Neacşu Mihaela , Sweep Fred , Span Paul N , Adriaansen Bas , van Herwaarden Antonius , Bryce Jillian , Ahmed Faisal , Ali Salma , Aparecida Sartori Sanchez Bachega Tania , Baronio Federico , Holtum Birkebaek Niels , Bonfig Walter , Clemente Maria , Cools Martine , de Bruin Christiaan , de Vries Liat , Elsedfy Heba , Globa Evgenia , Guran Tulay , Guven Ayla , Amr Nermine Hussein , Januś Dominika , Konrad Daniel , Markosyan Renata , Miranda Mirela , Poyrazoğlu Şukran , Rees Aled , Salerno Mariacarolina , Stancampiano Marianna Rita , Vieitis Ana , Abali Zehra Yavas , - van der Grinten Hedi Claahsen

Background: Testicular Adrenal Rest Tumors (TART) in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency (21OHD) or 11-hydroxylase deficiency (11OHD) are benign lesions causing testicular damage and infertility. We hypothesize that high ACTH exposure due to poor hormonal control during early life is promoting development of TART later in life.Objective: This study aims to examine the relation between early CAH diagnosis and cons...
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ea0073pep4.8 | Presented ePosters 4: Reproductive and Developmental Endocrinology | ECE2021

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

Nowotny Hanna F. , Blankenstein Oliver , Neumann Uta , Ahmed S. Faisal , Allen Stephanie , Baronio Federico , Battelino Tadej , Bertherat Jérôme , Bonomi Marco , de la Perrière Aude Brac , Tardy Véronique , Brucker Sara , Cappa Marco , Chanson Philippe , Bouvattier Claire , Colao Annamaria , Cools Martine , Davies Justin , Fenske Wiebke K. , Ghigo Ezio , Højbjerg Gravholt Claus , Hübner Angela , Husebye Eystein Sverre , Juul Anders , Kiefer Florian W. , Léger Juliane , Meyer Gesine , Phylactou Leonidas A. , Rohayem Julia , Russo Gianni , Scaroni Carla , Touraine Philippe , Unger Nicole , Hedi L. Claahsen-van der Grinten , Vojtková Jarmila , Yeste Diego , Günther Dörr Helmut , Lajic Svetlana , Reisch Nicole

BackgroundPrenatal 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 longterm 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 21hydroxylase def...
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ea0094p16 | Adrenal and Cardiovascular | SFEBES2023

Acute adrenal insufficiency related adverse events in children with congenital adrenal hyperplasia (CAH): Changes during the period 2019-2022 in I-CAH

Tseretopoulou Xanthippi , R Ali Salma , Bryce Jillian , Amin Nadia , Atapattu Navoda , Bachega Tania , Baronio Federico , H Birkebaek Niels , Bonfig Walter , Davies Justin H. , Thomas Tessy , de Vries Liat , Elsedfy Heba , H Amr Nermine , E Flueck Christa , Globa Evgenia , Zelinska Natalia , Guran Tulay , Yavas Abali Zehra , Guven Ayla , E Hannema Sabine , Iotova Violeta , Konrad Daniel , Lenherr-Taube Nina , P Krone Nils , Leka-Emiris Sofia , Vlachopapadopoulou Elpis , R Lichiardopol Corina , Marginean Otilia , L Markosyan Renata , Neumann Uta , Niedzela Marek , Phan-Hug Franziska , Poyrazoglu Sukran , Probst Ursina , Randell Tabitha , Russo Gianni , Carolina Salerno Maria , Shnorhavorian Margarett , van Eck Judith , Vieites Ana , Wasniewska Malgorzata , Faisal Ahmed Syed

Background: In 2019, the International CAH Registry (I-CAH) performed a benchmarking exercise of acute adrenal insufficiency related adverse events including adrenal crises (AC) and sick day episodes (SDE).Methods: In 2022, I-CAH data on children aged <18 years at first visit with 21-hydroxylase deficiency CAH from 35 centres in 19 countries were analysed to examine the current occurrence of SDE and AC and compared t...
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ea0095oc8.4 | Oral Communications 8 | BSPED2023

Association of newborn screening of congenital adrenal hyperplasia with outcomes in the first 90 days of life: a multi-centre I-CAH registry analysis of contemporary practice

BN Lim David , Bryce Jillian , R Ali Salma , Tseretopoulou Xanthippi , H Birkebaek Niels , E Hannema Sabine , Campos-Martorell Ariadna , Clemente Maria , Neumann Uta , E Fluck Christa , Metzger Sara , E Krone Ruth , German Alina , Baronio Federico , Atapattu Navoda , N Seneviratne Sumudu , Cools Martine , Mieke Thery , Guran Tulay , Yavas Abali Zehra , Fu Antony , Janus Dominika , Wasniewska Malgorzata , Coco Roberto , Russo Gianni , Rita Stancampiano Marianna , Bonfig Walter , Salerno Mariacarolina , L Claahsen-Van Der Grinten Hedi , P.H. Adriaansen Bas , Bachega Tania , C Miranda Mirela , Marginean Otilia , De Sanctis Luisa , Probst Ursina , Lenherr-Taube Nina , Konrad Daniel , O'Connell Michele , Gawlik Aneta , E Sandberg David , Shnorhavorian Margarett , Krone Nils , Faisal Ahmed S , Davies Justin H

Aims: We studied the association of newborn screening (NBS) on early infancy outcomes in 21-hydroxylase deficiency (21OHD) congenital adrenal hyperplasia (CAH), using I-CAH registry data.Methods: From 2018-2023, data was available for 121 infants with 21OHD CAH (52 male) during the first 90 days of life from 26 centres in 15 countries. NBS was performed in 15 centres from 9 countries.Result...
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ea0094p161 | Adrenal and Cardiovascular | SFEBES2023

An international study of the association between local health care resources and acute adrenal insufficiency events in children with congenital adrenal hyperplasia

Tseretopoulou Xanthippi , R Ali Salma , Bryce Jillian , Nadia Amin , Atapattu Navoda , Bachega Tania , Baronio Federico , H Birkebaek Niels , Bonfig Walter , Claahsen-Van der Grinten L. Hedi , Cools Martine , de Sanctis Luisa , de Vries Liat , Elsedfy Heba , E Flueck Christa , Fu Antony , Guaragna-Filho Guilherme , Guran Tulay , Guven Ayla , E Hannema Sabine , Iotova Violeta , Konrad Daniel , Lenherr-Taube Nina , Korbonits Marta , P Krone Nils , Krone Ruth , Leka-Emiris Sofia , Lichiardopol R Corina , Luczay Andrea , L Markosyan Renata , Mazen Inas , Milenkovic Tatjana , Mohnike Klaus , Neumann Uta , Niedzela Marek , Nordenstrom Anna , Phan-Hug Franziska , Poyrazoglu Sukran , Probst Ursina , Randell Tabitha , Vieites Ana , Russo Gianni , Thankamony Ajay , van den Akker Erica , van Eck Judith , van der Kamp Hetty , G Wasniewska Malgorzata , Ahmed Syed Faisal

Background: The reported occurrence and management of acute adrenal insufficiency–related adverse events in children vary widely between centres and may depend on available resources.Methods: Real world data from the I-CAH Registry from 44 centres [32 from high income (HIC) and 12 from low/middle income (LMIC) countries] and a total number of 607 children were linked to the results of a health care survey of local r...
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ea0095p86 | Adrenal 2 | BSPED2023

An international study of the association between local health care resources and acute adrenal insufficiency events in children with congenital adrenal hyperplasia

Tseretopoulou Xanthippi , Ali Salma R , Bryce Jillian , Amin Nadia , Atapattu Navoda , Bachega Tania , Baronio Federico , Birkebaek Niels H , Bonfig Walter , Claahsen-Van der Grinten Hedi L , Cools Martine , de Sanctis Luisa , de Vries Liat , Elsedfy Heba , Flueck Christa E , Fu Antony , Guaragna-Filho Guilherme , Guran Tulay , Guven Ayla , Hannema Sabine E , Iotova Violeta , Konrad Daniel , Lenherr-Taube Nina , Korbonits Marta , Krone Nils P , Krone Ruth , Leka-Emiris Sofia , Lichiardopol Corina R , Luczay Andrea , Markosyan Renata L , Mazen Inas , Milenkovic Tatjana , Mohnike Klaus , Neumann Uta , Niedzela Marek , Nordenstrom Anna , Phan-Hug Franziska , Poyrazoglu Sukran , Probst Ursina , Randell Tabitha , Vieites Ana , Russo Gianni , Thankamony Ajay , van den Akker Erica , van Eck Judith , van der Kamp Hetty , Wasniewska Malgorzata G , Ahmed Faisal S

Background: The reported occurrence and management of acute adrenal insufficiency–related adverse events in children vary widely between centres and may depend on available resources.Methods: Real world data from the I-CAH Registry that included a total of 2478 patient years follow-up on 607 children from 44 centres were studied and their association to the results of a health care survey of these centres that enqui...
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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