TY - JOUR
T1 - International practice of corticosteroid replacement therapy in congenital adrenal hyperplasia
T2 - Data from the I-CAH registry
AU - Bacila, Irina
AU - Freeman, Nicole
AU - Daniel, Eleni
AU - Sandrk, Marija
AU - Bryce, Jillian
AU - Ali, Salma Rashid
AU - Abali, Zehra Yavas
AU - Atapattu, Navoda
AU - Bachega, Tania A.
AU - Balsamo, Antonio
AU - Birkebæk, Niels
AU - Blankenstein, Oliver
AU - Bonfig, Walter
AU - Cools, Martine
AU - Costa, Eduardo Correa
AU - Darendeliler, Feyza
AU - Einaudi, Silvia
AU - Elsedfy, Heba Hassan
AU - Finken, Martijn J.J.
AU - Gevers, Evelien
AU - Claahsen-Van der Grinten, Hedi L.
AU - Guran, Tulay
AU - Güven, Ayla
AU - Hannema, Sabine E.
AU - Higham, Claire E.
AU - Iotova, Violeta
AU - van der Kamp, Hetty J.
AU - Korbonits, Marta
AU - Krone, Ruth E.
AU - Lichiardopol, Corina
AU - Luczay, Andrea
AU - Mendonca, Berenice Bilharinho
AU - Milenkovic, Tatjana
AU - Miranda, Mirela C.
AU - Mohnike, Klaus
AU - Neumann, Uta
AU - Ortolano, Rita
AU - Poyrazoglu, Sukran
AU - Thankamony, Ajay
AU - Tomlinson, Jeremy W.
AU - Vieites, Ana
AU - de Vries, Liat
AU - Ahmed, S. Faisal
AU - Ross, Richard J.
AU - Krone, Nils P.
N1 - Funding Information:
This project has received support from the 阀-CAH Registry project that receives unrestricted education grants from Diurnal Ltd and Neurocrine Biosciences. The initial development of the Registry was supported by the Medical Research Council (G1100236), the Seventh European Union Framework Program (201444) and the European Society for Paediatric Endocrinology Research Unit. S R A is supported by the Gardiner Lectureship at the University of Glasgow. N P K is supported by the German Research Foundation (KR3363/3-1).
Publisher Copyright:
© 2021 The authors.
PY - 2021/4
Y1 - 2021/4
N2 - Objective: Despite published guidelines no unified approach to hormone replacement in congenital adrenal hyperplasia (CAH) exists. We aimed to explore geographical and temporal variations in the treatment with glucocorticoids and mineralocorticoids in CAH.Design: This retrospective multi-center study, including 31 centers (16 countries), analyzed data from the International-CAH Registry.Methods: Data were collected from 461 patients aged 0-18 years with classic 21-hydroxylase deficiency (54.9% females) under follow-up between 1982 and 2018. Type, dose and timing of glucocorticoid and mineralocorticoid replacement were analyzed from 4174 patient visits.Results: The most frequently used glucocorticoid was hydrocortisone (87.6%). Overall, there were significant differences between age groups with regards to daily hydrocortisone-equivalent dose for body surface, with the lowest dose (median with interquartile range) of 12.0 (10.0-14.5) mg/m2/day at age 1-8 years and the highest dose of 14.0 (11.6-17.4) mg/m2/day at age 12-18 years. Glucocorticoid doses decreased after 2010 in patients 0-8 years (P < 0.001) and remained unchanged in patients aged 8-18 years. Fludrocortisone was used in 92% of patients, with relative doses decreasing with age. A wide variation was observed among countries with regards to all aspects of steroid hormone replacement.Conclusions: Data from the I-CAH Registry suggests international variations in hormone replacement therapy, with a tendency to treatment with high doses in children.
AB - Objective: Despite published guidelines no unified approach to hormone replacement in congenital adrenal hyperplasia (CAH) exists. We aimed to explore geographical and temporal variations in the treatment with glucocorticoids and mineralocorticoids in CAH.Design: This retrospective multi-center study, including 31 centers (16 countries), analyzed data from the International-CAH Registry.Methods: Data were collected from 461 patients aged 0-18 years with classic 21-hydroxylase deficiency (54.9% females) under follow-up between 1982 and 2018. Type, dose and timing of glucocorticoid and mineralocorticoid replacement were analyzed from 4174 patient visits.Results: The most frequently used glucocorticoid was hydrocortisone (87.6%). Overall, there were significant differences between age groups with regards to daily hydrocortisone-equivalent dose for body surface, with the lowest dose (median with interquartile range) of 12.0 (10.0-14.5) mg/m2/day at age 1-8 years and the highest dose of 14.0 (11.6-17.4) mg/m2/day at age 12-18 years. Glucocorticoid doses decreased after 2010 in patients 0-8 years (P < 0.001) and remained unchanged in patients aged 8-18 years. Fludrocortisone was used in 92% of patients, with relative doses decreasing with age. A wide variation was observed among countries with regards to all aspects of steroid hormone replacement.Conclusions: Data from the I-CAH Registry suggests international variations in hormone replacement therapy, with a tendency to treatment with high doses in children.
KW - Adolescent
KW - Adrenal Cortex Hormones/administration & dosage
KW - Adrenal Hyperplasia, Congenital/drug therapy
KW - Age Factors
KW - Child
KW - Child, Preschool
KW - Female
KW - Fludrocortisone/administration & dosage
KW - Glucocorticoids/administration & dosage
KW - Hormone Replacement Therapy/methods
KW - Humans
KW - Hydrocortisone/administration & dosage
KW - Infant
KW - Infant, Newborn
KW - Male
KW - Practice Patterns, Physicians'/statistics & numerical data
KW - Registries
KW - Retrospective Studies
UR - http://www.scopus.com/inward/record.url?scp=85102965211&partnerID=8YFLogxK
U2 - 10.1530/EJE-20-1249
DO - 10.1530/EJE-20-1249
M3 - Article
C2 - 33460392
SN - 0804-4643
VL - 184
SP - 553
EP - 563
JO - European Journal of Endocrinology
JF - European Journal of Endocrinology
IS - 4
ER -