Georgina L Chrisp1, Ann M Maguire2,3, Maria Quartararo1, Henrik Falhammar4,5,6, Bruce R King7,8, Craig F Munns2,3, David J Torpy9, Shihab Hameed3,10,11, R Louise Rushworth1. 1. School of Medicine, Sydney, The University of Notre Dame Australia, Darlinghurst, New South Wales, Australia. 2. The Children's Hospital, Westmead, New South Wales, Australia. 3. Sydney Medical School, The University of Sydney, Sydney, New South Wales, Australia. 4. Department of Endocrinology, Metabolism and Diabetes, Karolinska University Hospital, Stockholm, Sweden. 5. Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden. 6. Menzies School of Health Research and Royal Darwin Hospital, Tiwi, Northern Territory, Australia. 7. John Hunter Children's Hospital, Newcastle, New South Wales, Australia. 8. University of Newcastle, Newcastle, New South Wales, Australia. 9. Endocrine and Metabolic Unit, Royal Adelaide Hospital and University of Adelaide, Adelaide, South Australia, Australia. 10. Sydney Children's Hospital, Randwick, New South Wales, Australia. 11. School of Women's and Children's Health, University of New South Wales, Kensington, New South Wales, Australia.
Abstract
OBJECTIVE: Episodes of acute adrenal insufficiency (AI)/adrenal crises (AC) are a serious consequence of congenital adrenal hyperplasia (CAH). This study aimed to assess morbidity from acute illness in CAH and identify factors associated with use of IV hydrocortisone, admission and diagnosis of an AC. METHOD: An audit of acute illness presentations among children with CAH to paediatric hospitals in New South Wales, Australia, between 2000 and 2015. RESULTS: There were 321 acute presentations among 75 children with CAH. Two-thirds (66.7%, n = 214) of these resulted in admission and 49.2% (n = 158) of the patients received intravenous (IV) hydrocortisone. An AC was diagnosed in (9.0%). Prior to presentation, 64.2% (n = 206) had used oral stress dosing and 22.1% (n = 71) had been given intramuscular (IM) hydrocortisone. Vomiting was recorded in 61.1% (n = 196), 32.7% (n = 64) of whom had used IM hydrocortisone. Admission, AC diagnosis and use of stress dosing varied significantly between hospitals. IM use varied from 7.0% in one metropolitan hospital to 45.8% in the regional hospital. Children aged up to 12 months had the lowest levels of stress dosing and IV hydrocortisone administration. Higher numbers of prior hospital attendances for acute illness were associated with increased use of IM hydrocortisone. CONCLUSION: Prehospital and in-hospital management of children with CAH can vary between health services. Children under 12 months have lower levels of stress dosing prior to hospital than other age groups. Experience with acute episodes improves self-management of CAH in the context of acute illness in educated patient populations.
OBJECTIVE: Episodes of acute adrenal insufficiency (AI)/adrenal crises (AC) are a serious consequence of congenital adrenal hyperplasia (CAH). This study aimed to assess morbidity from acute illness in CAH and identify factors associated with use of IV hydrocortisone, admission and diagnosis of an AC. METHOD: An audit of acute illness presentations among children with CAH to paediatric hospitals in New South Wales, Australia, between 2000 and 2015. RESULTS: There were 321 acute presentations among 75 children with CAH. Two-thirds (66.7%, n = 214) of these resulted in admission and 49.2% (n = 158) of the patients received intravenous (IV) hydrocortisone. An AC was diagnosed in (9.0%). Prior to presentation, 64.2% (n = 206) had used oral stress dosing and 22.1% (n = 71) had been given intramuscular (IM) hydrocortisone. Vomiting was recorded in 61.1% (n = 196), 32.7% (n = 64) of whom had used IM hydrocortisone. Admission, AC diagnosis and use of stress dosing varied significantly between hospitals. IM use varied from 7.0% in one metropolitan hospital to 45.8% in the regional hospital. Children aged up to 12 months had the lowest levels of stress dosing and IV hydrocortisone administration. Higher numbers of prior hospital attendances for acute illness were associated with increased use of IM hydrocortisone. CONCLUSION: Prehospital and in-hospital management of children with CAH can vary between health services. Children under 12 months have lower levels of stress dosing prior to hospital than other age groups. Experience with acute episodes improves self-management of CAH in the context of acute illness in educated patient populations.
Authors: Valeria Calcaterra; Francesco Bassanese; Andrea Martina Clemente; Rossella Amariti; Corrado Regalbuto; Anna Sala; Gian Vincenzo Zuccotti Journal: Case Rep Endocrinol Date: 2020-12-29
Authors: Georgina L Chrisp; Maria Quartararo; David J Torpy; Henrik Falhammar; R Louise Rushworth Journal: Front Endocrinol (Lausanne) Date: 2022-09-20 Impact factor: 6.055
Authors: Salma R Ali; Jillian Bryce; Houra Haghpanahan; James D Lewsey; Li En Tan; Navoda Atapattu; Niels H Birkebaek; Oliver Blankenstein; Uta Neumann; Antonio Balsamo; Rita Ortolano; Walter Bonfig; Hedi L Claahsen-van der Grinten; Martine Cools; Eduardo Correa Costa; Feyza Darendeliler; Sukran Poyrazoglu; Heba Elsedfy; Martijn J J Finken; Christa E Fluck; Evelien Gevers; Márta Korbonits; Guilherme Guaragna-Filho; Tulay Guran; Ayla Guven; Sabine E Hannema; Claire Higham; Ieuan A Hughes; Rieko Tadokoro-Cuccaro; Ajay Thankamony; Violeta Iotova; Nils P Krone; Ruth Krone; Corina Lichiardopol; Andrea Luczay; Berenice B Mendonca; Tania A S S Bachega; Mirela C Miranda; Tatjana Milenkovic; Klaus Mohnike; Anna Nordenstrom; Silvia Einaudi; Hetty van der Kamp; Ana Vieites; Liat de Vries; Richard J M Ross; S Faisal Ahmed Journal: J Clin Endocrinol Metab Date: 2021-01-01 Impact factor: 5.958