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Endocrine Abstracts (2010) 24 P2

BSPED2010 Poster Presentations (1) (59 abstracts)

Optimization of treatment in children with 21-hydroxylase deficiency using cortisol profiling

K Logan , C Peters , P Hindmarsh & M Dattani

Institute of Child Health and Great Ormond Street Hospital, London, UK.

Introduction: A number of different parameters are used to assess adequacy of treatment in children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Hydrocortisone day curves are established practice in our unit. To determine the value of this procedure, outcomes were monitored using the European Society for Paediatric Endocrinology 2002 guidelines for the management of 21-hydroxylase deficiency.

Methods: Two hourly cortisol profiles were undertaken in 26 children with 21-hydroxylase deficiency (age range: 0.13–16.02 years) between August 2009 and January 2010.

Results: Conforming to the ESPE guidelines we have shown:

  • Comparable or reduced dosage of hydrocortisone (mg/m2 per day) compared to reported European averages in infants and children; age 0–1 years (n=3): mean hydrocortisone 17.3 vs 17.5 mg/m2 per day; age 1–16 years (n=23): mean hydrocortisone 12.9 vs 15 mg/m2 per day.

  • Adequate replacement of glucocorticoid in 92.3% (mean 24 h cortisol >150 nmol/l; range 125–468, mean 207 nmol/l).

  • Hydrocortisone dose alterations following profiling in 38.5% of children (increase in 60%, reduction in 40%).

  • Favourable growth and avoidance of steroid-related side effects demonstrated by:

    Of 77.0% BMI between ±2 SDS (range −2.25 to 3.2, mean +0.60) and 80% height SDS between ±2 SDS of mid-parental height SDS (range −2.23 to 2.95, mean 0.22).

  • Control without complete suppression of adrenal androgens in the majority (androstenedione <8 nmol/l in 81.8%).

In addition profiling enabled:

  • Tailoring of hydrocortisone timing and frequency to improve emulation of physiological secretion in 30.8% (with or without dose adjustment).

  • Exclusion of nocturnal hypoglycaemia by concurrent glucose profiling.

Conclusion: Cortisol profiling in children with 21-hydroxylase deficiency is an effective method of fine-tuning dosage and timing of hydrocortisone treatment.

Volume 24

38th Meeting of the British Society for Paediatric Endocrinology and Diabetes

British Society for Paediatric Endocrinology and Diabetes 

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