Children's National Hospital, Washington, DC 20010, USA.
The Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, MD 20892-1932, USA.
J Clin Endocrinol Metab. 2024 Jan 18;109(2):498-504. doi: 10.1210/clinem/dgad514.
Children with congenital adrenal hyperplasia (CAH) are at risk for early puberty. Gonadotropin-releasing hormone analog (GnRHa) is frequently used and can decrease bone mineral density (BMD).
Our aim was to investigate the effect of GnRHa therapy on BMD in a longitudinal study of patients with CAH spanning both childhood and adulthood.
Sixty-one patients with classic CAH due to 21-hydroxylase deficiency (20 treated with GnRHa) were followed with dual-energy X-ray absorptiometry (DXA) scans at puberty onset, attainment of adult height, and during early adulthood.
Whole body, lumbar spine, femoral neck, total hip, and distal radius BMD z-score at adult height. Longitudinal BMD and adult height were also assessed.
Twenty patients received GnRHa for an average of 4.5 ± 2 years. There were no differences in BMD between GnRHa-treated and -untreated groups at adult height for all sites. Overall, the follow-up DXA during early adulthood showed decreases in BMD z-scores for whole body (P = .01), lumbar spine (P < .0001), femoral neck (P = .06), total hip (P = .009), and distal radius (P = .05). GnRHa treatment correlated with improved height outcomes compared to predicted height at puberty onset after adjusting for midparental height (P = .02). Patients in both groups achieved similar adult height.
In children with CAH, GnRHa does not compromise BMD. However, BMD decreases with time and during the second and third decades of life is a possible effect of chronic supraphysiologic glucocorticoids. Children with CAH who experience early puberty benefit from GnRHa treatment as evidenced by the positive effect on height.
患有先天性肾上腺皮质增生症(CAH)的儿童有早期性成熟的风险。促性腺激素释放激素类似物(GnRHa)经常被使用,并且可以降低骨密度(BMD)。
我们的目的是通过对 CAH 患者进行跨越儿童期和成年期的纵向研究,调查 GnRHa 治疗对 BMD 的影响。
61 例由于 21-羟化酶缺乏导致的经典 CAH 患者(20 例接受 GnRHa 治疗)接受双能 X 射线吸收法(DXA)扫描,以评估青春期开始、成年身高获得和成年早期时的 BMD。
成年身高时的全身、腰椎、股骨颈、总髋和桡骨远端 BMD z 评分。还评估了纵向 BMD 和成年身高。
20 例患者平均接受 GnRHa 治疗 4.5 ± 2 年。在成年身高时,GnRHa 治疗组和未治疗组之间在所有部位的 BMD 均无差异。总体而言,在成年早期的随访 DXA 检查中,全身(P =.01)、腰椎(P <.0001)、股骨颈(P =.06)、总髋(P =.009)和桡骨远端(P =.05)的 BMD z 评分均下降。经调整父母身高的中间值后,GnRHa 治疗与青春期开始时的身高预测值相比,与身高改善相关(P =.02)。两组患者的成年身高均相似。
在 CAH 儿童中,GnRHa 不会影响 BMD。然而,随着时间的推移,BMD 会下降,在成年后的第二和第三个十年中,这可能是慢性超生理糖皮质激素的影响。早期性成熟的 CAH 儿童受益于 GnRHa 治疗,因为这对身高有积极影响。