Department of Pediatrics, University of Minnesota Children's Hospital, Minneapolis, MN.
Global Health, Rollins School of Public Health of Emory University, Atlanta, GA.
J Pediatr. 2015 Mar;166(3):743-50. doi: 10.1016/j.jpeds.2014.11.027. Epub 2014 Dec 31.
To determine the relationships between peripubertal and pubertal timing and growth, along with glucocorticoid exposure, to the reduced final adult height seen in patients with congenital adrenal hyperplasia (CAH).
Chart review of 104 children with classic CAH (41 males: 28 salt-wasting, 13 simple-virilizing; 63 females: 38 salt-wasting, 25 simple-virilizing) were selected from a cohort from 3 medical institutions in Minnesota. Triple logistic modeling of longitudinal data was performed to determine patterns of peripubertal and pubertal growth.
Hydrocortisone dose was similar between subtypes and during all growth periods. Simple-virilizing boys (P < .01) and girls (P < .01) were diagnosed later than their salt-wasting counterparts. Height at take-off SDS was reduced for patients with salt-wasting (boys: P < .01; girls: P < .01), and bone age at take-off SDS was more advanced for patients with simple-virilizing (boys: P < .01; girls: P = .05). Bone age at pubertal onset SDS was advanced for all patients, but more so for boys and girls with simple-virilizing. Although all patients had reduced final adult height SDS, this was more pronounced in patients with salt-wasting.
Reduced final adult height SDS in patients with salt-wasting vs simple-virilizing may be attributable in part to a later age of diagnosis and resultant less prolonged exposure to hydrocortisone. This finding suggests that duration of hydrocortisone treatment in the peripubertal period, independent of the hydrocortisone dose, may affect final adult height in patients with CAH.
确定青春期前和青春期时机与生长之间的关系，以及糖皮质激素暴露与先天性肾上腺增生症（CAH）患者成年终身高降低之间的关系。
从明尼苏达州的 3 家医疗机构的队列中选择了 104 名经典 CAH 患儿（41 名男性：28 名盐耗竭型，13 名单纯性男性化；63 名女性：38 名盐耗竭型，25 名单纯性男性化）的图表回顾。对纵向数据进行三因素逻辑回归模型分析，以确定青春期前和青春期生长模式。
盐耗竭型和所有生长期间的羟考酮剂量在亚型之间相似。单纯性男性化男孩（P<.01）和女孩（P<.01）的诊断时间晚于盐耗竭型患者。盐耗竭患者的起飞 SDS 身高较低（男孩：P<.01；女孩：P<.01），起飞 SDS 骨龄较先进为单纯性男性化患者（男孩：P<.01；女孩：P=.05）。青春期开始 SDS 的骨龄对于所有患者都是提前的，但对于单纯性男性化的男孩和女孩更为明显。尽管所有患者的成年终身高 SDS 均降低，但盐耗竭患者更为明显。
盐耗竭型与单纯性男性化患者的成年终身高 SDS 降低，部分可能归因于诊断年龄较晚，导致接受氢化可的松的时间缩短。这一发现表明，在青春期期间，氢化可的松治疗的持续时间，而不是氢化可的松剂量，可能会影响 CAH 患者的成年终身高。
