ISSN 1662-4009 (online)

ESPE Yearbook of Paediatric Endocrinology (2022) 19 8.6 | DOI: 10.1530/ey.19.8.6

ESPEYB19 8. Adrenals Important for Clinical Practice (4 abstracts)

8.6. Characteristics of growth in children with classic Congenital Adrenal Hyperplasia due to 21-hydroxylase deficiency during adrenarche and beyond

Troger T , Sommer G , Lang-Muritano M , Konrad D , Kuhlmann B , Zumsteg U & Flück CE



J Clin Endocrinol Metab. 2022; 107(2): e487-e499. PMID: 34599587https://pubmed.ncbi.nlm.nih.gov/34599587/

Brief Summary: This study describes the growth of adequately treated children with classical Congenital Adrenal Hyperplasia (CAH) due to 21-hydroxylase deficiency during adrenarche and beyond. Patients with and without significant bone age advancement, and thus differing height prediction during adrenarche, showed similar (predicted) final height when reassessed during puberty.

Patients with classical CAH often do not achieve their full growth potential due to the difficulties in balancing the effects of hyperandrogenism and hypercortisolism during childhood and adolescence (1). Whether the increased height velocity during adrenarche has any impact on final height in this patient group is still not clear (2, 3).

This study describes the bone age, growth velocity/height and predicted final height during puberty and adrenarche in 41 children (26 girls) with classical CAH. Longitudinal data were grouped in three time periods: A) Before adrenarche, aged<6 yrs; B) Adrenarche, >= 6 yrs to pubertal onset; C) Puberty, after the onset of puberty. Patients were categorized into two groups during adrenarche: those with accelerating bone age (49%; BA-CA at least 0.1 year higher at age 6-9 yrs than<6 yrs of age) or non-accelerating bone age (all others). At the time of the study, 37/41 patients had developed pubarche (P2) at a mean age of 10.5 yrs in girls (B2 at 10.3 yrs) and 11.7 yrs in boys (G2 at 12.1 yrs). The hydrocortisone (HC) dose was between 14.1 to 16.2 mg/m2/day, while 40/41 patients also received fludrocortisone.

Patients were shorter than peers (−0.4 SDS±0.8 SD) and than their parental target (corrected final height −0.6 SDS±1.0 SD). Children with accelerated BA-CA were taller during adrenarche than those without accelerating BA-CA (mean height SDS 0.5 vs. -0.3 SDS). However, these differences disappeared during puberty. Growth velocity was higher before adrenarche in children with accelerating BA-CA and decreased thereafter. Estimated final height was lower in those with accelerating BA-CA but this difference disappeared at puberty. In the end, final adult height was similarly reduced in both accelerating BA-CA and non-accelerating BA-CA groups (-0.4 SDS vs. -0.3 SDS).

In summary, patients with classical CAH achieve a final height that is close to normal. Although a growth acceleration and bone age progression can be observed in almost 50% of children during the years of adrenarche, this does not result in a more compromised (predicted) final height compared with patients without such bone age progression. These findings indicate that bone age alone should not be used during adrenarche as a clinical marker for metabolic control in patients with classic CAH.

References: 1. Charmandari E, Hindmarsh P, Brook CG. Classic Congenital adrenal Hyperplasia and puberty. Eur J Endocrinol 2004; 151(3): U77–82. 2. Dorr HG. Growth in patients with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Horm Res 2007; 68(5): 93–99. 3. Muthusamy K, Elamin MB, Smushkin G, et al. Adult height in patients with congenital adrenal hyperplasia: a systematic review and metaanalysis. J Clin Endocrinol Metab. 2010; 95(9):4161–4172

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