Department of Internal Medicine, Seoul National University Hospital, Seoul, South Korea.
Department of Internal Medicine, Seoul National University College of Medicine, Seoul, South Korea.
Front Endocrinol (Lausanne). 2021 Oct 12;12:761258. doi: 10.3389/fendo.2021.761258. eCollection 2021.
There is a lack of studies regarding the long-term outcomes of Asian adults with classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. We hypothesized that adults with CAH are at higher metabolic risk than their age-, and sex-matched controls. We further investigated the long-term health outcome-related factors in adults with CAH. We compared metabolic risk between adults with CAH (71 men, 93 women) and age-, and sex-matched controls (190 men, 261 women) from the Korean National Health and Nutrition Examination Survey data. The presence of obesity, testicular adrenal rest tumors (TARTs), and menstrual irregularity was assessed. Hormone status and treatment regimens were compared according to the presence of adverse outcomes. The median age was 27.0 y and 28.0 y for men and women, respectively. Adults with CAH had a higher waist circumference (88.0 vs. 82.3 cm in men, and 83.5 . 72.3 cm in women), and blood pressure (125.0 vs. 113.0 mmHg in men, and 120.0 . 104.0 mmHg in women) than age- and sex-matched controls (<0.05 for all). The 2.7-fold increased risk for hypertension (men) and 2.0-fold increased risk for obesity (women) was significant in patients with CAH (<0.05 for both). Obese adults with CAH showed significantly higher adrenal limb thicknesses (men) and 17-hydroxyprogesterone and dehydroepiandrosterone sulfate levels (women) (<0.05 for both). TARTs occurred in 58.1% of men and did not differ by hormone or treatment regimen. Irregular menstruation was observed in 57.1% of women, with higher dehydroepiandrosterone sulfate levels in those with irregular periods. Adults with CAH had a higher metabolic risk than the general population. Poor disease control may increase their risk of metabolic morbidity and menstrual irregularity.
由于 21-羟化酶缺乏，亚洲经典先天性肾上腺皮质增生症（CAH）成年患者的长期结局缺乏研究。我们假设 CAH 成年患者的代谢风险高于年龄和性别匹配的对照组。我们进一步研究了 CAH 成年患者的长期健康结局相关因素。我们比较了韩国国家健康和营养检查调查数据中 CAH 成年患者（71 名男性，93 名女性）和年龄及性别匹配对照组（190 名男性，261 名女性）的代谢风险。评估了肥胖、睾丸肾上腺残余肿瘤（TART）和月经不规律的存在情况。根据不良结局的存在比较了激素状况和治疗方案。男性和女性的中位年龄分别为 27.0 岁和 28.0 岁。CAH 成年患者的腰围（男性为 88.0 厘米，女性为 83.5 厘米）和血压（男性为 125.0 毫米汞柱，女性为 120.0 毫米汞柱）高于年龄和性别匹配的对照组（所有比较均<0.05）。CAH 患者发生高血压的风险增加 2.7 倍（男性）和肥胖的风险增加 2.0 倍（女性），差异具有统计学意义（均<0.05）。肥胖的 CAH 成年患者的肾上腺侧支厚度（男性）和 17-羟孕酮和脱氢表雄酮硫酸酯水平（女性）显著更高（均<0.05）。58.1%的男性出现 TART，且激素或治疗方案之间无差异。57.1%的女性出现月经不规律，且不规则经期患者的脱氢表雄酮硫酸酯水平更高。CAH 成年患者的代谢风险高于一般人群。疾病控制不佳可能会增加他们发生代谢性疾病和月经不规律的风险。
