IMPORTANCE Testicular adrenal rest tumors (TARTs), often found in male patients with congenital adrenal hyperplasia (CAH), are benign lesions causing testicular damage and infertility. We hypothesize that chronically elevated adrenocorticotropic hormone exposure during early life may promote TART development. OBJECTIVE This study aimed to examine the association between commencing adequate glucocorticoid treatment early after birth and TART development. DESIGN AND PARTICIPANTS This retrospective multicenter (n = 22) open cohort study collected longitudinal clinical and biochemical data of the first 4 years of life using the I-CAH registry and included 188 male patients (median age 13 years; interquartile range: 10-17) with 21-hydroxylase deficiency (n = 181) or 11-hydroxylase deficiency (n = 7). All patients underwent at least 1 testicular ultrasound. RESULTS TART was detected in 72 (38%) of the patients. Prevalence varied between centers. When adjusted for CAH phenotype, a delayed CAH diagnosis of >1 year, compared with a diagnosis within 1 month of life, was associated with a 2.6 times higher risk of TART diagnosis. TART onset was not predicted by biochemical disease control or bone age advancement in the first 4 years of life, but increased height standard deviation scores at the end of the 4-year study period were associated with a 27% higher risk of TART diagnosis. CONCLUSIONS AND RELEVANCE A delayed CAH diagnosis of >1 year vs CAH diagnosis within 1 month after birth was associated with a higher risk of TART development, which may be attributed to poor disease control in early life.

中文翻译：

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先天性肾上腺增生男性婴儿期的激素控制和睾丸肾上腺静息肿瘤的发展：一项回顾性多中心队列研究。

重要性 睾丸肾上腺休息肿瘤 (TART) 常见于先天性肾上腺增生 (CAH) 男性患者，是导致睾丸损伤和不育的良性病变。我们推测，生命早期长期接触促肾上腺皮质激素水平升高可能会促进 TART 的发展。目的 本研究旨在探讨出生后早期开始充足的糖皮质激素治疗与 TART 发展之间的关系。设计和参与者 这项回顾性多中心 (n = 22) 开放队列研究使用 I-CAH 登记系统收集了生命前 4 年的纵向临床和生化数据，包括 188 名男性患者（中位年龄 13 岁；四分位数范围：10-17 岁） ) 患有 21-羟化酶缺乏症 (n = 181) 或 11-羟化酶缺乏症 (n = 7)。所有患者至少接受 1 次睾丸超声检查。结果 72 名患者（38%）检测到 TART。各中心的患病率有所不同。根据 CAH 表型进行调整后，与出生后 1 个月内诊断相比，延迟 >1 年的 CAH 诊断与 TART 诊断风险高 2.6 倍相关。TART 发病并不是通过生命前 4 年的生化疾病控制或骨龄提前来预测的，但在 4 年研究期结束时身高标准差分数的增加与 TART 诊断风险增加 27% 相关。结论和相关性 与出生后 1 个月内诊断的 CAH 相比，延迟 >1 年的 CAH 诊断与 TART 发生的较高风险相关，这可能归因于生命早期疾病控制不佳。