Endocrine Abstracts

Background: Impaired anthopometric indices in patients with 21-hydroxylase deficiency congenital adrenal hyperplasia (CAH) are commonly described. This study aims to establish international benchmarks for children with CAH that can be used for comparing and improving care.

Methods: CAH cases in the I-CAH registry with height and weight data collected from birth to 18.5yrs were included. One clinic visit per year of life was selected for each case by proximity to birthday and country specific reference data were used when available to calculate standard deviation scores (SDS) for height. BMI SDS were calculated using WHO reference data. Multifactorial analysis was undertaken describing medians and ranges by year of life, sex and age bands.

Results: 16,021 longitudinal visits between 1969 – 2025 from 1,282 cases with CAH across 26 countries in 5 continents were available and from these, 8,025 single visits per year of life were analysed with a median of 9 visits per case (1, 18) at a median age of 7yrs (1, 18) at time of visit. Median height SDS was -1.0 (-4.0, 3.7) in the 1-3yrs age band (n, 882), 0.4 (-3.4, 4.0) in the 7-9yrs age band (n, 682) and -1.1 (-3.9, 2.1) in the 16-18yrs age band (n, 312). Median BMI SDS for 4-6yrs (n, 768), 7-9yrs (n, 682) and 16-18yrs (n, 312) was 0.7 (-3.8, 4.0), 1.1 (-3.7, 4.0) and 0.7 (-2.4, 4.0), respectively. For these groups, 296 (38.5%), 354 (51.9%) and 112 (39.1%) were >1 BMI SDS, respectively. There was marked international variation between centres compared to these global benchmarks, with mean height SDS for around 10% of centres being more than 3SD above or below the mean for all centres. Of the 1282 cases, 635 (50% ) were born before 2011 and more recent year of birth was associated with greater height SDS in the age groups spanning 10-18yrs (P <0.05).

Conclusions: The rich international resource of real-world anthropometry data in I-CAH has allowed the creation of anthropometric benchmarks that show temporal and regional variations. It is anticipated that the production of centre-specific anthropometric reports will facilitate an improvement in the quality of care in CAH.