Treatment of classic congenital adrenal hyperplasia (CAH) is directed at replacing deficient hormones and reducing androgen excess. However, even in the era of early diagnosis and lifelong hormonal substitution, the presence of CAH is still associated with numerous complications and also with increased mortality. The aim of this article was to create an authoritative and balanced review concerning cardiometabolic risk in patients with CAH. The authors searched all major databases and scanned reference lists of all potentially eligible articles to find relevant articles. The risk was compared with that in other forms of adrenal insufficiency. The reviewed articles, most of which were published recently, provided conflicting results, which can be partially explained by differences in the inclusion criteria and treatment, small sample sizes and gene-environmental interactions. However, many studies showed that the presence of CAH is associated with an increased risk of weight gain, worsening of insulin sensitivity, high blood pressure, endothelial dysfunction, early atherosclerotic changes in the vascular wall and left ventricular diastolic dysfunction. These complications were more consistently reported in patients with classic than non-classic CAH and were in part related to hormonal and functional abnormalities associated with this disorder and/or to the impact of over- and undertreatment. An analysis of available studies suggests that individuals with classic CAH are at increased cardiometabolic risk. Excess cardiovascular and metabolic morbidity is likely multifactorial, related to glucocorticoid overtreatment, imperfect adrenal hormone replacement therapy, androgen excess and adrenomedullary failure. Cardiometabolic effects of new therapeutic approaches require future targeted studies.

中文翻译：

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先天性肾上腺增生症的心脏代谢方面。


典型先天性肾上腺增生症 (CAH) 的治疗旨在补充缺乏的激素并减少雄激素过多。然而，即使在早期诊断和终生激素替代的时代，CAH 的存在仍然与许多并发症和死亡率增加有关。本文的目的是对 CAH 患者的心脏代谢风险进行权威且平衡的综述。作者搜索了所有主要数据库并扫描了所有可能符合条件的文章的参考文献列表以查找相关文章。该风险与其他形式的肾上腺功能不全的风险进行了比较。所审查的文章大部分是最近发表的，提供了相互矛盾的结果，这可以部分解释为纳入标准和治疗方法的差异、小样本量以及基因-环境相互作用。然而，许多研究表明，CAH 的存在与体重增加风险增加、胰岛素敏感性恶化、高血压、内皮功能障碍、血管壁早期动脉粥样硬化变化和左心室舒张功能障碍有关。与非经典 CAH 患者相比，这些并发症在经典 CAH 患者中的报告更为一致，部分与该疾病相关的激素和功能异常和/或过度治疗和治疗不足的影响有关。对现有研究的分析表明，患有典型 CAH 的个体心脏代谢风险增加。心血管和代谢疾病发病率过高可能是多因素造成的，与糖皮质激素过度治疗、肾上腺激素替代疗法不完善、雄激素过多和肾上腺髓质衰竭有关。 新治疗方法的心脏代谢作用需要未来的针对性研究。