BACKGROUND: Congenital adrenal hyperplasia (CAH) in children varies in presentation and progression with several challenges in optimal management. Effective treatment is to achieve normal growth and development while avoiding adrenal crisis and hyperandrogenisation. AIM: Our aim was to ascertain the current practice in the UK on CAH management in children in comparison with the recommendations made by the Endocrine Society. METHODS: An online survey was emailed to the British Society of Paediatric Endocrinology (BSPED) members requesting a response from each centre regarding CAH management. RESULTS: The survey was completed by 35 out of 92 centres (38% response rate). Tertiary centres constituted 22/35, while 8/35 were district general hospitals providing tertiary services. Treatment varied among centres with 25/35 using 10-15 mg/m2/day of hydrocortisone and 21/35 of centres using 50-150 μg/day of fludrocortisone. The frequency of clinical reviews was contentious and varied depending on the child's age and clinical status. Reviews were done 3-4 monthly in 68% and 6 monthly in 31% of centres. The frequency of investigations including 17-hydroxyprogesterone (66% 3-6 monthly; 34% yearly), testosterone/dehydroepiandrosterone sulphate (37% 6 monthly; 51% yearly), renin/aldosterone (31% 6 monthly; 69% yearly) and bone age (83% yearly, 6% 2 yearly) varied significantly among centres. Genetic counselling was provided at diagnosis in 69% of the centres while surgical (66%) and psychology (80%) input were provided on an as required basis. CONCLUSION: Our survey highlights the diversity in managing children with CAH in the UK as compared with the recommendations of the Endocrine Society. It also demonstrates inconsistent involvement of essential specialist services, which are essential for optimal management of this condition.
BACKGROUND:Congenital adrenal hyperplasia (CAH) in children varies in presentation and progression with several challenges in optimal management. Effective treatment is to achieve normal growth and development while avoiding adrenal crisis and hyperandrogenisation. AIM: Our aim was to ascertain the current practice in the UK on CAH management in children in comparison with the recommendations made by the Endocrine Society. METHODS: An online survey was emailed to the British Society of Paediatric Endocrinology (BSPED) members requesting a response from each centre regarding CAH management. RESULTS: The survey was completed by 35 out of 92 centres (38% response rate). Tertiary centres constituted 22/35, while 8/35 were district general hospitals providing tertiary services. Treatment varied among centres with 25/35 using 10-15 mg/m2/day of hydrocortisone and 21/35 of centres using 50-150 μg/day of fludrocortisone. The frequency of clinical reviews was contentious and varied depending on the child's age and clinical status. Reviews were done 3-4 monthly in 68% and 6 monthly in 31% of centres. The frequency of investigations including 17-hydroxyprogesterone (66% 3-6 monthly; 34% yearly), testosterone/dehydroepiandrosterone sulphate (37% 6 monthly; 51% yearly), renin/aldosterone (31% 6 monthly; 69% yearly) and bone age (83% yearly, 6% 2 yearly) varied significantly among centres. Genetic counselling was provided at diagnosis in 69% of the centres while surgical (66%) and psychology (80%) input were provided on an as required basis. CONCLUSION: Our survey highlights the diversity in managing children with CAH in the UK as compared with the recommendations of the Endocrine Society. It also demonstrates inconsistent involvement of essential specialist services, which are essential for optimal management of this condition.
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