ESPE Abstracts (2014) 82 P-D-3-2-635

Congenital Adrenal Hyperplasia: Survey of the Management in Children Across UK

Usha Niranjana & Anuja Natarajanb

aSheffield Children’s Hospital, Sheffield, UK; bDoncaster Royal Infirmary, Doncaster, UK

Background: The ultimate goal in the management of congenital adrenal hyperplasia (CAH) in children is to achieve normal growth and development which can be a challenge. The consensus guidance recommends hydrocortisone (10–20 mg/m2 per day) and fludrocortisone(50–200 μg/day) therapy titrated carefully with regular monitoring.

Objective and hypotheses: To determine the current practise in UK regarding the management of CAH in children as there is ongoing debate about the optimal management.

Method: A survey was mailed to all the members of the British Society of Paediatric Endocrinology (n=440) asking for a response regarding CAH management from each centre (n=92).

Results: The response rate was 38% (n=35). Tertiary paediatric endocrine centres constituted 63% while 23% provided tertiary service in a district general hospital (DGH). The number of children with CAH managed by each centre varied from 15 to 120 for tertiary centres to 5–13 for DGH. The dose of hydrocortisone varied significantly from 6–8 μg/m2 per day to 12–20 mg/m2 per day with majority (71%) using 10–15 mg/m2 per day. Similarly fludrocortisone dose varied from 50–300 μg/day with majority (26%) using 50–100 μg/day. The frequency of clinic visits was controversial and centres felt it varied depending on the child’s age and clinical status. The majority (46%) did 3 monthly reviews while others did 4–6 monthly (54%) reviews. The frequency and type of investigations: 17 hydroxy-progesterone (63% 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, 9% as indicated) varied with centres. Genetic counselling was provided at diagnosis in 69% of centres while surgical (66%) and psychology (80%) input were primarily as required.

Conclusion: This pilot survey across the UK highlights the on-going lack of consensus on a unified guidance for managing patients with CAH in the paediatric population. The survey also points to the lack of consistent involvement of other specialists which is an essential part of the management in this group of patients.

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