Bretones Patricia, Riche Benjamin, Pichot Emmanuel, David Michel, Roy Pascal, Tardy Véronique, Kassai Behrouz, Gaillard Ségolène, Bernoux Delphine, Morel Yves, Chatelain Pierre, Nicolino Marc, Cornu Catherine
J Pediatr Endocrinol Metab. 2016 Dec 1;29(12):1379-1388. doi: 10.1515/jpem-2016-0156.
In congenital adrenal hyperplasia (CAH), adjusting hydrocortisone dose during childhood avoids reduced adult height. However, there are currently no CAH-specific charts to monitor growth during treatment. Our objective was to elaborate growth reference charts and bone maturation data for CAH patients.
We conducted a retrospective observational cohort study, in 34 French CAH centers. Patients were 496 children born 1970-1991 with genetically proven 21-hydroxylase deficiency. Their growth and bone maturation data were collected until age 18 together with adult height, puberty onset, parental height, and treatment. The mean (SD) heights were modeled from birth to adulthood. The median±1 SD and ±2 SDs model-generated curves were compared with the French references. A linear model for bone maturation and a logistic regression model for the probability of short adult height were built.
Growth charts were built by sex for salt wasting (SW) and simple virilizing (SV) children treated before 1 year of age. In girls and boys, growth was close to that of the general French population up to puberty onset. There was almost no pubertal spurt and the mean adult height was shorter than that of the general population in girls (-1.2 SD, 156.7 cm) and boys (-1.0 SD, 168.8 cm). Advanced bone age at 8 years had a strong impact on the risk of short adult height (OR: 4.5 per year advance).
The 8-year bone age is a strong predictor of adult height. It will help monitoring the growth of CAH-affected children.
在先天性肾上腺皮质增生症（CAH）中，儿童期调整氢化可的松剂量可避免成人身高降低。然而，目前尚无用于监测CAH治疗期间生长情况的特定图表。我们的目标是为CAH患者精心制作生长参考图表和骨成熟数据。
我们在34个法国CAH中心进行了一项回顾性观察队列研究。患者为496名1970年至1991年出生、经基因证实患有21 - 羟化酶缺乏症的儿童。收集他们直至18岁的生长和骨成熟数据，以及成人身高、青春期开始时间、父母身高和治疗情况。对从出生到成年的平均（标准差）身高进行建模。将模型生成的中位数±1标准差和±2标准差曲线与法国参考标准进行比较。构建了骨成熟的线性模型和成人身高矮小概率的逻辑回归模型。
为1岁前接受治疗的失盐型（SW）和单纯男性化型（SV）儿童按性别绘制了生长图表。在女孩和男孩中，直至青春期开始，生长情况与法国普通人群相近。几乎没有青春期生长突增，女孩（-1.2标准差，156.7厘米）和男孩（-1.0标准差，168.8厘米）的平均成人身高低于普通人群。8岁时骨龄提前对成人身高矮小风险有强烈影响（每提前一年的比值比：4.5）。
8岁骨龄是成人身高的有力预测指标。它将有助于监测受CAH影响儿童的生长情况。
