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首页> 外文期刊>European journal of endocrinology >Cardiovascular risk, metabolic profile, and body composition in adult males with congenital adrenal hyperplasia due to 21-hydroxylase deficiency
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Cardiovascular risk, metabolic profile, and body composition in adult males with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

机译：21-羟化酶缺乏症引起的先天性肾上腺皮质增生的成年男性的心血管风险，代谢特征和身体组成

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ObjectiveLifelong glucocorticoid therapy in patients with congenital adrenal hyperplasia (CAH) or the disease per se may result in increased cardiovascular risk. We therefore investigated cardiovascular and metabolic risk profiles in adult CAH males.Subjects and methodsWe compared CAH males ( n =30), 19–67 years old, with age- and sex-matched controls ( n =32). Subgroups of different ages (<30 years or older) and CYP21A2 genotypes (null, I2splice, and I172N as the mildest mutation) were studied. Anthropometry, fat and lean mass measured by dual-energy X-ray absorptiometry, lipids, liver function tests, homocysteine, lipoprotein-(a), glucose and insulin during an oral glucose tolerance test (OGTT), urine albumin, adrenal hormones, and 24?h ambulatory blood pressure measurements were studied.ResultsCAH males were shorter. Waist/hip ratio and fat mass were higher in older patients and the I172N group. Heart rate was faster in older patients, the I2splice, and I172N groups. Insulin levels were increased during OGTT in all patients and in the I172N group. γ-glutamyl transpeptidase was increased in older patients and in the I172N group. Testosterone was lower in older patients. Homocysteine was lower in younger patients, which may be cardioprotective. The cardiovascular risk seemed higher with hydrocortisone/cortisone acetate than prednisolone. Urinary epinephrine was lower in all groups of patients except in I172N.ConclusionsIndications of increased risk were found in CAH males ≥30 years old and in the I172N group. In contrast, younger CAH males did not differ from age-matched controls. This is likely to reflect a better management in recent years.
机译：目的对患有先天性肾上腺皮质增生（CAH）或疾病本身的患者进行终生糖皮质激素治疗可能会增加心血管疾病的风险。因此，我们调查了成年CAH男性的心血管和代谢风险状况。研究对象和方法我们将19-67岁的CAH男性（n = 30），年龄和性别相匹配的对照组（n = 32）进行了比较。研究了不同年龄（<30岁或更大）和CYP21A2基因型（null，I2splice和I172N为最轻度突变）的亚组。人体测量学，双能量X射线吸收法测量的脂肪和瘦体重，脂质，肝功能测试，同型半胱氨酸，脂蛋白-（a），口服葡萄糖耐量测试（OGTT）中的葡萄糖和胰岛素，尿白蛋白，肾上腺激素和研究了24小时动态血压的测量结果。老年患者和I172N组的腰围/臀围比率和脂肪量较高。老年患者，I2接头和I172N组的心率更快。在所有患者和I172N组中，OGTT期间胰岛素水平均升高。老年患者和I172N组的γ-谷氨酰转肽酶升高。老年患者的睾丸激素水平较低。年轻患者中的同型半胱氨酸水平较低，这可能对心脏有保护作用。氢化可的松/醋酸可的松的心血管风险似乎比泼尼松龙高。除I172N外，所有患者组的尿中肾上腺素均较低。结论在≥30岁的CAH男性和I172N组中发现了风险增加的指征。相反，年轻的CAH男性与年龄匹配的对照组没有差异。这很可能反映了近年来更好的管理。

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