Endocrine Abstracts

Introduction: Congenital adrenal hyperplasia (CAH) varies significantly in clinical presentation and progression causing challenges in management. The ultimate goal of treatment is to achieve normal growth and development while avoiding adrenal crisis and hyperandrogenism.

Aim: Our aim was to ascertain the current practice in the UK on CAH management in children and compare these with the endocrine society recommendations.

Methods: An online survey with ten questions was emailed to the British Society of Paediatric Endocrinology (BSPED) members asking for one response from each centre regarding CAH management.

Results: The survey was completed by 35 out of 92 centres (38% response rate). Tertiary paediatric endocrine centres constituted 63% (22/35) of centres, while 23% (8/35) were district general hospitals (DGHs) providing tertiary endocrine services. The number of patients per centre varied from 15 to 120 amongst the tertiary centres to 5–30 patients in the DGHs. The hydrocortisone dose ranged from 6 to 20 mg/m² per day with 71% (25/35) of centres using 10–15 mg/m² per day. The fludrocortisone dose ranged from 50 to 300 μg/day with 60% (21/35) using 50–150 μg/day. The frequency of clinical reviews was contentious and centres felt it varied depending on the child’s age and clinical status. Reviews were done 3–4 monthly in 68% (24/35) and 6 monthly in 31% (11/35) centres. The frequency of investigations including 17- hydroxyprogesterone (66% 3–6 monthly; 34% yearly), Testosterone/DHEAS (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 centres while surgical (66%) and psychology (80%) input were primarily on an as required basis.

Conclusion: Our survey highlights the diversity in UK in managing children with CAH as compared to the recommendations of the Endocrine Society Guidelines. It demonstrates inconsistent involvement of other specialists which is an essential part of this multifaceted condition.