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hrp0095rfc5.3 | Adrenals and HPA Axis | ESPE2022

Towards understanding the metabolic phenotype of glucocorticoid deficiency in 21-hydroxylase deficiency

Bacila Irina , Li Nan , Eachus Helen , Storbeck Karl-Heinz , T Cunliffe Vincent , P Krone Nils

Background: Steroid 21-hydroxylase deficiency (21OHD) is the most common form of congenital adrenal hyperplasia. Patients present with cortisol and aldosterone deficiency as well as with hyperandrogenism, leading to virilisation in females and early adrenarche in both sexes. Requiring life-long glucocorticoid (GC) replacement, patients frequently experience daily fluctuations between GC overexposure and deficiency. Increased prevalence of metabolic disease con...

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hrp0097p1-569 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2023

National service evaluation project analysing the quality of care for children and young people with congenital adrenal hyperplasia in the United Kingdom: Data from patients and clinicians

Bacila Irina , R Lawrence Neil , Ji Xiaochen , Faisal Ahmed S , Alvi Sabah , Bath Louise , Blair Jo , Cheetham Tim , Crowne Liz , H Davies Justin , Dattani Mehul , Gevers Evelien , Krone Ruth , Patel Leena , Thankamony Ajay , Randell Tabitha , Ryan Fiona , Elford Sue , Blackett Sallyann , P Krone Nils

Background: The variation in the provision of services in secondary and tertiary care for children and young people living with CAH in the United Kingdom is unknown. We aimed to conduct a nationwide service evaluation to inform from both the patient and clinician perspective.Methods: We conducted an anonymous multicentre survey using online questionnaires for clinicians and CAH patients/carers. Clinical leads from UK cen...

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hrp0098p1-8 | Adrenals and HPA Axis 1 | ESPE2024

Leptin and adiponectin are associated with the glucocorticoid dose and androgen concentrations in children and young persons with congenital adrenal hyperplasia: data from the CAH-UK cohort.

A Bacila Irina , R Lawrence Neil , Alvi Sabah , D Cheetham Timothy , Crowne Elizabeth , Das Urmi , T Dattani Mehul , H Davies Justin , Gevers Evelien , Keevil Brian , E Krone Ruth , Lawrie Allan , Patel Leena , Randell Tabitha , J Ryan Fiona , Thankamony Ajay , Faisal Ahmed S , P Krone Nils

Introduction: Patients with Congenital adrenal hyperplasia (CAH) have increased prevalence of obesity and metabolic problems. The underlining mechanisms are not clearly known. Adipokines are likely involved in this association, however, their role in it is not completely understood.Objective: We studied adiponectin and leptin in children and young persons with CAH, in relation to their body mass, treatment, hormonal and ...

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hrp0098p1-107 | Adrenals and HPA Axis 2 | ESPE2024

Non-invasive biochemical monitoring for Congenital Adrenal Hyperplasia: use of urinary steroid metabolites and salivary 17α-Hydroxyprogesterone

J Tonge Joseph , Bacila Irina , Richard Lawrence Neil , Alvi Sabah , D Cheetham Timothy , Crowne Elizabeth , Das Urmi , Tulsidas Dattani Mehul , H Davies Justin , Gevers Evelien , Keevil Brian , E Krone Ruth , Patel Leena , Randell Tabitha , J Ryan Fiona , Thankamony Ajay , Faisal Ahmed S , Taylor Norman , P Krone Nils

Background: Monitoring disease control in congenital adrenal hyperplasia (CAH) by random serum 17a-Hydroxyprogesterone (17OHP) measurements is invasive and fails to capture total daily adrenal steroid synthesis. Urinary steroid analysis may provide a more suitable, non-invasive method of assessing treatment response in children with CAH, by estimating the total daily excretion of 17OHP metabolites.Method: Urine was colle...

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hrp0097p1-3 | Adrenals and HPA Axis | ESPE2023

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 , H Davies Justin , Thomas Tessy , de Vries Liat , Elsedfy Heba , Hussein Amr Nermine , E. Flueck Christa , Globa Evgenia , Zelinska Natalia , Guran Tulay , Yavas Abali Zehra , Guven Ayla , E. Hannema Sabine , Iotova Violeta , Konrad Daniel , P. Krone Nils , Lenherr-Taube Nina , Leka-Emiris Sofia , Vlachopapadopoulou Elpis , R. Lichiardopol Corina , Marginean Otilia , L. Markosyan Renata , Niedziela Marek , Banaszak-Ziemska Magdalena , Neumann Uta , Phan-Hug Franziska , Polyrazoglu Sukran , Probst Ursina , Randell Tabitha , Russo Gianni , Salerno Mariacarolina , Shnorhavorian Margarett , van Eck Judith , Vieites Ana , Wasniewska Malgorzata , F 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 compare it ...

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hrp0098fc4.1 | Adrenals and HPA Axis 1 | ESPE2024

Real world data analysis of contemporary therapy and its association to anthropometric outcomes in 1500 patients with congenital adrenal hyperplasia (CAH).

A Bacila Irina , R Lawrence Neil , Balagamage Chamila , Bryce Jillian , R Ali Salma , Alimussina Malika , Chen Minglu , Bhushan Arya Ved , Atapattu Navoda , A Bachega Tânia , Barat Pascal , Baronio Federico , Bertelloni Silvano , Birkebæk Niels , Bonfig Walter , de Bruin Christiaan , Claahsen – van der Grinten Hedi , Cools Martine , Costa Eduardo , Crowne Elizabeth , H Davies Justin , Debono Miguel , de Vries Liat , Dumic Kubat Katja , Elsedfy Heba , Evliyaoglu Olcay , Flüeck Christa , Gazdagh Gabriella , German Alina , F Gevers Evelien , Globa Evgenia , Guran Tulay , Güven Ayla , Guazzarotti Laura , Hannema Sabine , Hatipoglu Nihal , Janus Dominika , van der Kamp Hetty , Karabouta Zacharoula , Krone Ruth , Lenherr Taube Nina , Leka-Emiri Sofia , Lichiardopol Corina , Mamdouh Hassan Mona , Marginean Otilia , Markosyan Renata , Mazen Inas , Miles Harriet , L Mitchell Anna , Mohnike Klaus , Neumann Uta , Niedziela Marek , Nordenstrom Anna , M O’Connell Susan , Phan-Hug Franziska , Poyrazoglu Sukran , Probst-Scheidegger Ursina , Rey Rodolfo , Russo Gianni , Salerno Mariacarolina , de Sanctis Luisa , N Seneviratne Sumudu , Segev-Becker Anat , Shenoy Savitha , Skae Mars , Thankamony Ajay , Ucar Ahmet , Utari Agustini , Vieites Ana , Wasniewska Malgorzata , Faisal Ahmed S , P Krone Nils

Introduction and Objective: Previous evidence from the I-CAH registry showed wide variation of glucocorticoid (GC) replacement between different countries and centres. We wanted to explore the impact of different GC doses on height and weight in children and young people with CAH.Methods: We analysed data from patients under 18 years with 21-hydroxylase deficiency recorded in the I-CAH registry from assessments since 200...

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ESPE Abstracts

Towards understanding the metabolic phenotype of glucocorticoid deficiency in 21-hydroxylase deficiency

National service evaluation project analysing the quality of care for children and young people with congenital adrenal hyperplasia in the United Kingdom: Data from patients and clinicians

Leptin and adiponectin are associated with the glucocorticoid dose and androgen concentrations in children and young persons with congenital adrenal hyperplasia: data from the CAH-UK cohort.

Non-invasive biochemical monitoring for Congenital Adrenal Hyperplasia: use of urinary steroid metabolites and salivary 17α-Hydroxyprogesterone

Acute Adrenal Insufficiency Related Adverse Events In Children With Congenital Adrenal Hyperplasia (CAH): Changes During The Period 2019-2022 In I-CAH

Real world data analysis of contemporary therapy and its association to anthropometric outcomes in 1500 patients with congenital adrenal hyperplasia (CAH).

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