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

Righi, B. et al. (2023) Long-term cardiometabolic morbidity in young adults with classic 21-hydroxylase deficiency congenital adrenal hyperplasia. Endocrine, 80(3), pp. 630-638. (doi: 10.1007/s12020-023-03330-w) (PMID:36857009) (PMCID:PMC10199864)

Abstract

Purpose: To study the current practice for assessing comorbidity in adults with 21-hydroxylase CAH and to assess the prevalence of comorbidity in these adults. Methods: A structured questionnaire was sent to 46 expert centres managing adults with CAH. Information collected included current therapy and surveillance practice with a particular focus on osteoporosis/osteopaenia, hyperlipidaemia, type 2 diabetes/hyperinsulinaemia, hypertension, CV disease, obesity. Results: Of the 31 (67%) centres from 15 countries that completed the survey, 30 (97%) screened for hypertension by measuring blood pressure, 30 (97%) screened for obesity, 26 (84%) screened for abnormal glucose homoeostasis mainly by using Hb1Ac (73%), 25 (81%) screened for osteoporosis mainly by DXA (92%), 20 (65%) screened for hyperlipidaemia and 6 (19%) screened for additional CV disease. Of the 31 centres, 13 provided further information on the six co-morbidities in 244 patients with a median age of 33 yrs (range 19, 94). Of these, 126 (52%) were females and 174 (71%) received fludrocortisone in addition to glucocorticoids. Of the 244 adults, 73 (30%) were treated for at least one comorbidity and 15 (21%) for more than 2 co-morbidities. Of 73, the patients who were treated for osteoporosis/osteopaenia, hyperlipidaemia, type 2 diabetes/hyperinsulinaemia, hypertension, CV disease, obesity were 43 (59%), 17 (23%), 16 (22%), 10 (14%), 8 (11), 3 (4%) respectively. Conclusion: Cardiometabolic and bone morbidities are not uncommon in adults with CAH. There is a need to standardise the screening for these morbidities from early adulthood and to explore optimal therapy through routine collection of standardised data.

• Item Type: Articles
• Status: Published
• Refereed: Yes
• Glasgow Author(s) Enlighten ID: Ahmed, Professor Syed Faisal and Markosyan, Dr Renata and Stancampiano, Dr Marianna and Bryce, Dr Jillian and Ali, Dr Salma and RIGHI, Dr BEATRICE
• Authors: Righi, B., Ali, S. R., Bryce, J., Tomlinson, J. W., Bonfig, W., Varonio, F., Costa, E. C., Guaragna-Filho, G., T'Sjoen, G., Cools, M., Markosyan, R., Bachega, T. A.S.S., Miranda, M. C., Iotova, V., Falhammar, H., Ceccato, F., Stancampiano, M. R., Russo, G., Daniel, E., Auchus, R. J., Ross, R. J., and Ahmed, S. F.
• College/School: College of Medical Veterinary and Life Sciences > School of Medicine, Dentistry & Nursing
• Journal Name: Endocrine
• Publisher: Springer
• ISSN: 1355-008X
• ISSN (Online): 1559-0100
• Published Online: 01 March 2023
• Copyright Holders: Copyright © 2023 The Authors
• First Published: First published in Endocrine 80(3): 630-638
• Publisher Policy: Reproduced under a Creative Commons License