ESPE2021 ePoster Category 1 Adrenal A (10 abstracts)
Long-term Cardiometabolic Morbidity In Young Adults With Classic 21-Hydroxylase Deficiency Congenital Adrenal Hyperplasia
Beatrice Righi 1 , Salma R Ali 1,2 , Jillian Bryce 2 , Jeremy W Tomlinson 3 , Walter Bonfig 4,5 , Federico Baronio 6 , Eduardo C Costa 7 , Guilherme Guaragna Filho 8 , Guy T’Sjoen 9 , Martine Cools 10 , Renata Markosyan 11 , Tania A S S Bachega 12 , Mirela C Miranda 12 , Violeta Iotova 13 , Henrik Falhammar 14,15 , Filippo Ceccato 16 , Marianna R Stancampiano 17 , Gianni Russo 17 , Rade Vukovic 18 , Roberta Giordano 19 , Inas Mazen 20 , Ayla Güven 21 , Feyza Darendeliler 22 , Çukran Poyrazoglu 22 , Liat de Vries 23,24 , Mona Ellaithi 25 , Eleni Daniel 26 , Colin Johnston 27 , Steven J Hunter 28 , Paul V Carroll 29 , Safwaan Adam 30 , Colin G Perry 31 , Tara Kearney 32 , Prakash Abraham 33 , D Aled Rees 34 , Graham P Leese 35 , Nicole Reisch 36 , Nike M M L Stikkelbroeck 37 , Richard J Auchus 38 , Richard J Ross 26 & S Faisal Ahmed 1
1Developmental Endocrinology Research Group, University of Glasgow, Royal Hospital For Sick Children, Yorkhill, Glasgow, United Kingdom; 2Office for Rare Conditions, Royal Hospital for Children & Queen Elizabeth University Hospital, Glasgow, United Kingdom; 3Oxford Centre for Diabetes, Endocrinology & Metabolism, NIHR Oxford Biomedical Research Centre, Churchill Hospital, University of Oxford, Oxford, United Kingdom; 4Department of Paediatrics, Technical University München, Munich, Germany; 5Department of Paediatrics, Klinikum Wels-Grieskirchen, Wels, Austria; 6Pediatric Unit, Department Hospital of Woman And Child, IRCSS S.Orsola-Malpighi University Hospital, Bologna, Italy; 7Pediatric Surgery Service, Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil; 8Department of Pediatrics, School of Medicine, Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, Brazil; 9Department of Endocrinology - Center for Sexology and Gender, Ghent University Hospital, Ghent, Belgium; 10Department of Internal Medicine and Paediatrics, Ghent University and Pediatric Endocrinology Service, Ghent University Hospital, Ghent, Belgium; 11Endocrinology, Yerevan State Medical University Endocrinology Clinic, Yerevan, Armenia; 12Unidade de Endocrinologia do Desenvolvimento, Laboratório de Hormônios e Genética Molecular/LIM42, Disciplina de Endocrinologia, Hospital Das Clinicas, Faculdade De Medicina, Universidade de São Paulo, São Paulo, Brazil; 13UMHAT Sveta Marina, Medical University of Varna, Varna, Bulgaria; 14Department of Molecular Medicine and Surgery, Karolinska Institute, Stockholm, Sweden; 15Department of Endocrinology, Karolinska University Hospital, Stockholm, Sweden; 16Endocrinology Unit, Department of Medicine DIMED, University-Hospital of Padua, Padua, Italy; 17Department of Pediatrics, Endocrine Unit, IRCCS San Raffaele Scientific Institute, Endo-ERN Center for Rare Endocrine Conditions, Milan, Italy; 18Department of Endocrinology, Mother and Child Health Care Institute of Serbia "Dr Vukan Cupic", Belgrade, Serbia; 19Department of Clinical and Biological Sciences and Division of Endocrinology, Diabetes and Metabolism-Department of Medical Sciences, University of Turin, Turin, Italy; 20Department of Clinical Genetics, National Research Center, Cairo, Egypt; 21Pediatric Endocrinology, Baskent University İstanbul Hospital, Istanbul, Turkey; 22Department of Pediatric Endocrinology, Istanbul University, Istanbul Medical School, Istanbul, Turkey; 23Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; 24The Jesse Z and Sara Lea Shafer Institute for Endocrinology and Diabetes, Schneider Children’s Medical Center of Israel, Petah-Tikva, Israel; 25Faculty of Medical Laboratory Sciences, Al-Neelain University, Khartoum, Sudan; 26Department of Oncology and Metabolism, University of Sheffield, Sheffield, United Kingdom; 27Department of Endocrinology, St Albans City Hospital, West Hertfordshire Hospitals Trust, St Albans, United Kingdom; 28Department of Endocrinology and Diabetes, Royal Victoria Hospital, Belfast, United Kingdom; 29Department of Endocrinology, Guy’s & St. Thomas’ NHS Foundation Trust, London, United Kingdom; 30The Christie NHS Foundation Trust, Manchester, United Kingdom; 31Queen Elizabeth University Hospital, Glasgow, United Kingdom; 32Department of Endocrinology, Salford Royal Foundation Trust, Salford, United Kingdom; 33Department of Diabetes and Endocrinology, Aberdeen Royal Infirmary, Aberdeen, United Kingdom; 34Neuroscience and Mental Health Research Institute, Cardiff University, Cardiff, United Kingdom; 35Ninewells Hospital, Dundee, United Kingdom; 36Medizinische Klinik IV, Klinikum der Universität München, München, Germany; 37Department of Internal Medicine, Division of Endocrinology, Radboud University Medical Center, Nijmegen, Netherlands; 38Division of Metabolism, Endocrinology and Diabetes, University of Michigan, Ann Arbor, MI, USA
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 prevalence of cardiometabolic morbidity.
Methods: Data were collected using a structured questionnaire sent to 46 centres managing adults with CAH within three overlapping networks: International Congenital Adrenal Hyperplasia (I-CAH) Registry, CaH Adult Study Executive (CaHASE) Consortium UK and European Reference Network on Rare Endocrine Conditions (Endo-ERN). Information collected included current therapy and surveillance practice of adults with CAH with emphasis on cardiometabolic conditions.
Results: Thirty-one (67%) centres from 15 countries completed the survey. Thirty (97%) centres screened patients for hypertension by measuring blood pressure during clinical routine examination. Thirty (97%) screened for obesity by mainly using BMI (87%) and weight (81%). Twenty-six (84%) centres screened for abnormal glucose homeostasis by mainly using Hb1Ac (61%) and fasting plasma glucose (42%). Twenty-five (81%) centres screened for osteoporosis mainly by using DXA scans (74%). Twenty (65%) centres screened for hyperlipidaemia using fasting lipids. Five (19%) routinely screened patients for other CV disease. Out of 255 adults with a median age of 32 yrs (range, 19,94) from 13 centres, 93 (36%) had obesity/overweight, 58 (23%) osteoporosis/osteopenia, 50 (20%) hyperlipidaemia, 20 (8%) T2DM/hyperinsulinaemia, 18 (7%) hypertension and 10 (4%) CV disease. Of these 255, 78 (30%) were receiving therapy for cardiometabolic morbidity and, of these, 17 (7%) were treated for 2 or more comorbidities. Of the total affected by each comorbidity, the number of patients who received therapy for obesity/overweight, osteoporosis/osteopenia, hyperlipidaemia, hypertension, CV disease and T2DM/hyperinsulinaemia was 3 (3%), 43 (74%), 17 (34%), 10 (56%), 8 (80%) and 18 (90%), respectively. The median age at start of therapy for obesity/overweight, osteoporosis/osteopenia, hyperlipidaemia, hypertension/CV disease and T2DM/hyperinsulinaemia was 27 yrs (17,55), 34 (18,63), 55 (19,79), 57 (39,72) and 27 (14,78), respectively. For some conditions such as hypertension there was a wide range of drugs used (8 drugs in 18 patients).
Conclusions: Cardiometabolic morbidities are not uncommon in adults with CAH. There is a need for greater standardisation of the screening for these morbidities from early adulthood and there is a need to explore optimal therapy through routine collection of standardised data.
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