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Published on: September 15, 2017
Absence of clinically relevant growth acceleration in untreated children with non-classical congenital adrenal
Karijn J Pijnenburg-Kleizen1, George F Borm, Barto J Otten
1Department of Paediatric Endocrinology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands. K.Kleizen@cukz.umcn.nl
Insights
Growth acceleration in children with non-classical congenital adrenal hyperplasia (NC-CAH) is minimal. Bone age acceleration is more significant, indicating NC-CAH may be present even without obvious growth spurts.
Area of Science:
- Pediatric Endocrinology
- Genetics and Genetic Diseases
- Metabolic Disorders
Background:
- Congenital adrenal hyperplasia (CAH) involves elevated adrenal androgens, impacting growth and bone maturation.
- Non-classical CAH (NC-CAH) presents with milder androgen excess, potentially leading to subtle growth velocity changes in untreated children.
Purpose of the Study:
- To investigate growth patterns and bone maturation in untreated children with non-classical CAH (NC-CAH).
- To determine if subtle growth changes in NC-CAH warrant further diagnostic consideration.
Main Methods:
- Twenty-four untreated NC-CAH patients were categorized into symptomatic and asymptomatic groups.
- Height standard deviation scores (HSDS) corrected for target height (HSDS-THSDS) and bone age acceleration (BA(c)) were analyzed.
- Linear mixed models were employed to assess growth and bone age parameters.
Main Results:
- Symptomatic NC-CAH patients showed a slight annual increase in HSDS-THSDS (0.06 SDS/year).
- Mean bone age acceleration (BA(c)) in symptomatic patients was 2.21 years (SDS 0.66, p < 0.0001).
- Asymptomatic NC-CAH patients exhibited no significant growth acceleration or BA(c).
Conclusions:
- Growth acceleration in untreated NC-CAH children is often subtle and may not be apparent on standard growth charts.
- Bone age acceleration (BA(c)) is a more pronounced indicator in NC-CAH.
- The absence of increased growth velocity does not rule out NC-CAH; bone age assessment is crucial for diagnosis.
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.
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