先天性肾上腺皮质增生症的心脏代谢问题

IF 22 1区 医学 Q1 ENDOCRINOLOGY & METABOLISM Endocrine reviews Pub Date : 2024-09-06 DOI:10.1210/endrev/bnae026
Robert Krysiak, Hedi L Claahsen-van der Grinten, Nicole Reisch, Philippe Touraine, Henrik Falhammar
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引用次数: 0

摘要

治疗典型的先天性肾上腺增生症(CAH)的目的是补充缺乏的激素,减少雄激素过多。然而,即使在早期诊断和终身激素替代的时代,CAH 的存在仍然与众多并发症和死亡率的增加有关。本文旨在就 CAH 患者的心脏代谢风险撰写一篇权威、平衡的综述。作者检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目录,以找到相关文章。将其风险与其他形式肾上腺功能不全的风险进行了比较。所查阅的文章大多是近期发表的,其结果相互矛盾,部分原因可能是纳入标准和治疗方法不同、样本量较小以及基因与环境之间存在相互作用。然而,许多研究表明,CAH 的存在与体重增加、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的风险增加有关。据报道,这些并发症在典型 CAH 患者中的发生率高于非典型 CAH 患者,部分原因与该疾病相关的激素和功能异常和/或过度治疗和治疗不当的影响有关。对现有研究的分析表明,典型 CAH 患者的心血管代谢风险增加。过高的心血管和代谢发病率可能是多因素的,与糖皮质激素过度治疗、不完善的肾上腺激素替代治疗、雄激素过多和肾上腺髓质功能衰竭有关。未来需要对新的治疗方法对心脏代谢的影响进行有针对性的研究。
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Cardiometabolic Aspects of Congenital Adrenal Hyperplasia.

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.

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来源期刊
Endocrine reviews
Endocrine reviews 医学-内分泌学与代谢
CiteScore
42.00
自引率
1.00%
发文量
29
期刊介绍: Endocrine Reviews, published bimonthly, features concise timely reviews updating key mechanistic and clinical concepts, alongside comprehensive, authoritative articles covering both experimental and clinical endocrinology themes. The journal considers topics informing clinical practice based on emerging and established evidence from clinical research. It also reviews advances in endocrine science stemming from studies in cell biology, immunology, pharmacology, genetics, molecular biology, neuroscience, reproductive medicine, and pediatric endocrinology.
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