Background/Aims: In classical congenital adrenal hyperplasia (CAH), elevation of adrenal androgens leads to accelerated growth and bone maturation with compromised adult height. In untreated children with non-classical CAH (NC-CAH), in which adrenal androgens are generally only slightly increased, growth velocity may not be significantly elevated.
Methods: Twenty-four patients were included and divided into a symptomatic and an asymptomatic group. Height was expressed as height standard deviation scores (HSDS) and corrected for target height (HSDS-THSDS). Bone maturation was expressed as bone age acceleration (BA c = bone age - calendar age). Linear mixed models with random factor patient were used for the analysis of growth and bone age.
Results: In symptomatic patients (n = 17), HSDS-THSDS only slightly increased by 0.06 SDS per year (95% CI 0.02-0.10). Mean BA c was 2.21 years (SDS 0.66, p < 0.0001). In asymptomatic patients (n = 7), no significant growth acceleration or BA c was found.
Conclusions: In untreated NC-CAH children, growth acceleration is small and generally not visible on their growth charts. BA c is more pronounced. Therefore, the absence of an increase in growth velocity does not exclude the diagnosis of NC-CAH. When considering this diagnosis, bone age acceleration should also be taken into account. Copyright

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doi.org/10.1159/000337217, hdl.handle.net/1765/68205
Hormone Research in Paediatrics
Department of Pediatrics

Pijnenburg-Kleizen, K., Borm, G. F., Otten, B., Schott, D., van den Akker, E., Stokvis-Brantsma, W. H., … Claahsen-Van Der Grinten, H. (2012). Absence of clinically relevant growth acceleration in untreated children with non-classical congenital adrenal hyperplasia. Hormone Research in Paediatrics, 77(3), 164–169. doi:10.1159/000337217