Height in congenital adrenal hyperplasia improves with low hydrocortisone dose
Height outcome in children with congenital adrenal hyperplasia may be improved with a low hydrocortisone dose and use of mineralocorticoid therapy, according to findings published in Endocrine Practice.
Carla Bizzarri, MD, of the unit of endocrinology and diabetes at Bambino Gesù Children’s Hospital in Rome, and colleagues evaluated 57 children (mean age at diagnosis, 0.47 years; 31 boys) with congenital adrenal hyperplasia (CAH) treated with hydrocortisone and hydrocortisone only to assess factors that affect growth trajectory.
Fludrocortisone treatment was used in 75.4% of participants.
Overall, mean final height standard deviation score (SDS) was –0.74 and mean target adjusted final height SDS was –0.31. Boys had a lower target adjusted final height (–0.44) compared with girls (–0.13; P = .001), whereas mean total pubertal growth was higher in boys (21.9 cm) compared with girls (19.2 cm; P = .19).
Target adjusted final height SDS was better in participants who received mineralocorticoid therapy and in participants who were monitored by morning basal hormone evaluation. Participants who received mineralocorticoid therapy had significantly lower BMI SDS compared with participants who did not receive mineralocorticoid therapy. Onset of puberty was later in boys (mean age at puberty, 11.6 years) compared with girls (mean age at puberty, 10.06 years). The course of disease was complicated by central precocious puberty in 17.3% of participants. Participants with central precocious puberty had a lower target adjusted final height SDS (–1.15) compared with the rest of the study group (–0.14; P < .001).
Positive correlations were found between delta-4-androstenedione levels (P = .001) and adrenocorticotropic hormone (ACTH; P = .03) with target adjusted final height SDS, whereas it was negatively correlated to hydrocortisone dose (P < .001). Hydrocortisone dose was also negatively related to mean height velocity SDS (P = .006) and total pubertal growth (P = .001). Hydrocortisone dose adversely affected targeted adjusted final height SDS (P < .001).
“Height outcome of patients with classical CAH treated from early infancy with hydrocortisone and fludrocortisone seems to be better than height outcome reported in patients with older age at diagnosis and different steroid treatments,” the researchers wrote. “Factors contributing to improved height outcome seem to be low hydrocortisone dose, use of mineralocorticoid therapy, no suppression of [hypothalamic–pituitary–adrenal] axis, and steroid therapy adjustment based on fasting basal hormones. During puberty, hydrocortisone therapy should be administered at the lowest dose able to maintain a satisfactory hormone balance in order to optimize pubertal growth spurt and final height.” – by Amber Cox
Disclosure: The researchers report no relevant financial disclosures.