ESPE Abstracts (2015) 84 P-2-201


Improved Linear Growth in Patients with Classical Congenital Adrenal Hyperplasia

Silvia Romaa,b, Carla Bizzarria, Giuseppe Scirèa,b, Gian Luigi Spadonia,b, Marco Cappaa & Chiara Maggiolia


aUnit of Endocrinology and Diabetes, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy; b‘Tor Vergata’ University, Rome, Italy

Background: Poor linear growth is still one of the main concerns in children with congenital adrenal hyperplasia (CAH). An impairment of linear growth, adversely affecting final height, has been related both to overtreatment with glucocorticoid replacement therapy and to poor control of adrenal androgen levels.

Objective and hypotheses: To define factors that influence linear growth and final height in patients with classical CAH.

Method: The medical records of 25 patients (12 females, 13 males) followed from early infancy until adulthood in our clinic were analysed and the clinical observations were divided into four groups according to age and puberty (0–2 years, 2 years up to puberty onset, pubertal years, post-pubertal years). Differences in the mean dose of hydrocortisone and average levels of Δ4-androstenedione and 17-hydroxyprogesterone between the four groups were evaluated using one-way ANOVA. Multivariate analysis was used to study factors independently affecting final height.

Results: Mean final height was 167.8±7.1 cm in males (−1.3±1.1 SDS) and 158±6.5 cm in females (−0.8±1 SDS). Final height corrected for parental height was −1.01±1.3 in males and −0.37±0.5 in females. Mean total pubertal growth spurt was 23.1±4.6 cm in males and 19.8±6.5 cm in females. Significantly higher doses of hydrocortisone were required during the first 2 years of life and during puberty (P<0.05). Final height was adversely affected by the average dose of hydrocortisone (P<0.001) and by the level of Δ4-androstenedione (P<0.045) during follow up.

Conclusion: The main goals of therapy in children with CAH are the control of glucocorticoid deficiency and the suppression of adrenal androgen hyper-secretion. Particularly during puberty, the daily dose of hydrocortisone should be maintained as low as possible to obtain a normal pubertal growth spurt and optimize final height. Final height of CAH patients followed from early infancy, particularly females, seems to be now less impaired than previously reported.

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