ESPE Abstracts (2016) 86 P-P2-62

ESPE2016 Poster Presentations Adrenal P2 (49 abstracts)

Final Height Data in a Cohort of Patients with Congenital Adrenal Hyperplasia Treated with Tailored Doses of Hydrocortisone

Gloria Shir Wey Pang a , Gill Rumsby b , Peter C Hindmarsh a, & Mehul T Dattani a,

aGreat Ormond Street Hospital, London, UK; bUniversity College London Hospitals, London, UK

Background: Patients with congenital adrenal hyperplasia (CAH) are glucocorticoid deficient and require cortisol replacement to maintain homeostasis and prevent adrenal crises. Hydrocortisone dosing needs to be individualized because of variable cortisol clearance rates. Patients are thus prone to be either over or undertreated, both of which compromises final height.

Objective and hypotheses: Performing 24-h cortisol profiling serially may allow for more accurate titration of hydrocortisone dosage, resulting in a possible improvement in final height.

Method: Paediatric patients with CAH due to 21 hydroxylase deficiency were retrospectively recruited from a single tertiary centre between 1990 and 2015. We included those patients who had reached their final height and had had at least two serial 24-h cortisol profiles performed.

Results: We identified 50 children, 34 children (7 M) had neonatal onset CAH whilst 16 (8 M) had late onset simple virilising disease. Their final heights were compared to mean adult height of the population (British 1990 growth reference centiles published by Cole et al) and mid parental height. The mean of hydrocortisone dose prescribed from 3 to 18 years of age was lower than that previously reported in an ESPE survey.

Table 1. (for abstract P2-P62)
Final Height (SD)(cm)Mean adult height SDS (SE)Mid-parental height SDS (SE)Mean BMI (kg/m2)Mean HC dosages (mg/m2 per day) (SD)
Neonatal onset170±5.0−0.98±0.37−0.92±0.3526.0±2.613.95±2.86
  CAH Boys
Neonatal onset160±5.2−0.57±0.110.96±0.1224.7±8.214.55±2.88
  CAH Girls
Late onset168±6.7−1.17±0.42−1.4±0.5030.0±12.613.79±3.20
  Simple virilising boys
Late onset165±8.20.24±0.280.03±0.0825.6±3.310.50±2.80
  Simple virilising girls

Conclusion: Apart from late onset virilising boys, final height lies within one SDS from mean adult height of the population and the mean mid-parental height, and BMI is close to normal range. These results are encouraging compared to previous similar cross sectional studies and affirm that cortisol profiling may optimise dosing with better control of CAH and improved final height.

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