Background: Three times daily (TID) hydrocortisone (HC) is recommended as the optimal glucocorticoid regimen in growing children with congenital adrenal hyperplasia (CAH). However, a variety of other treatment schemes are used in the clinical setting. Objective: To determine whether there are clinical differences between children being treated with TID HC versus those receiving other glucocorticoid regimens. Further, we sought to determine whether there was evidence of a deleterious effect on growth in children receiving treatment with alternate regimens. Methods: Medical records of children followed in our pediatric endocrinology outpatient clinic for classic CAH secondary to 21-hydroxylase deficiency during the last 10 years were reviewed. Variables analyzed included sex, age at most recent visit, glucocorticoid type, frequency and dose (mg/m2/day), height z-score, BMI z-score, ethnicity, most recent bone age, growth velocity z-score, and provider’s impression of compliance (good or poor). Results: Of 104 children (51% boys) with CAH, 50 (48%) were on TID HC, 43 (41%) were on prednisone or prednisolone, and 5 (5%) were on dexamethasone. An additional 6 (6%) were on HC administered either 2 or 4 times daily. No differences were seen between TID HC and alternate regimen groups with respect to sex, height z-score, BMI z-score, ethnicity, provider assessment of compliance, ratio of bone age to chronologic age, or growth velocity. The average height z-score was -0.40 + 1.31 in the TID HC group compared to -0.87 + 1.33 in the alternate regimen group (p=0.075). Patients receiving TID HC were younger (p=0.027) and on a lower glucocorticoid dose (p=0.001) than those on alternate regimens. Conclusions: Less than half of our patients with CAH were receiving TID HC. Reassuringly, growth parameters and other indices of disease control were equivalent between patients on conventional HC dosing and other therapeutic approaches. These results suggest that a range of glucocorticoid treatment regimens may be equally viable in children with CAH.


中文翻译：

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是否所有先天性肾上腺增生症患者都需要每天服用 3 次氢化可的松才能正常生长？

背景：每日三次 (TID) 氢化可的松 (HC) 被推荐为患有先天性肾上腺增生症 (CAH) 的成长中儿童的最佳糖皮质激素治疗方案。然而，在临床环境中使用了多种其他治疗方案。目的：确定接受 TID HC 治疗的儿童与接受其他糖皮质激素治疗的儿童之间是否存在临床差异。此外，我们试图确定是否有证据表明接受替代方案治疗的儿童对生长有有害影响。方法：回顾过去 10 年在我们儿科内分泌科门诊就诊的 21-羟化酶缺乏症继发经典 CAH 患儿的医疗记录。分析的变量包括性别、最近一次就诊时的年龄、糖皮质激素类型、频率和剂量（mg/m2/天）、身高 z 分数、BMI z 分数、种族、最近的骨龄、生长速度 z 分数以及提供者对依从性的印象（好或差）。结果：在 104 名患有 CAH 的儿童（51% 男孩）中，50 名（48%）接受 TID HC，43 名（41%）接受泼尼松或泼尼松龙，5 名（5%）接受地塞米松。另外 6 名 (6%) 每天服用 2 次或 4 次 HC。在性别、身高 z 分数、BMI z 分数、种族、提供者对依从性的评估、骨龄与实际年龄的比率或生长速度方面，TID HC 和替代方案组之间没有差异。TID HC 组的平均身高 z 得分为 -0.40 + 1.31，而替代方案组为 -0.87 + 1.33 (p=0.075)。接受 TID HC 的患者更年轻 (p=0.027) 并且糖皮质激素剂量较低 (p=0. 001) 比采用替代方案的那些。结论：不到一半的 CAH 患者正在接受 TID HC。令人欣慰的是，接受常规 HC 给药和其他治疗方法的患者的生长参数和其他疾病控制指标是相同的。这些结果表明，一系列糖皮质激素治疗方案对于 CAH 儿童可能同样可行。