Congenital forms of endocrine hypertension are rare and potentially life-threatening disorders, primarily caused by genetic defects affecting adrenal steroid synthesis and activation pathways. These conditions exhibit diverse clinical manifestations, which can be distinguished by their unique molecular mechanisms and steroid profiles. Timely diagnosis and customized management approach are crucial to mitigate unfavorable outcomes associated with uncontrolled hypertension and other related conditions. Treatment options for these disorders depend on the distinct underlying pathophysiology, which involves specific pharmacological therapies or surgical adrenalectomy in some instances. This review article summarizes the current state of knowledge on the therapeutic management of congenital forms of endocrine hypertension, focusing on familial hyperaldosteronism (FH), congenital adrenal hyperplasia, apparent mineralocorticoid excess, and Liddle syndrome. We provide an overview of the genetic and molecular pathogenesis underlying each disorder, describe the clinical features, and discuss the various therapeutic approaches available and their risk of adverse effects, aiming to improve outcomes in patients with these rare and complex conditions.

中文翻译：

---

先天性内分泌高血压的治疗管理。

先天性内分泌高血压是罕见且可能危及生命的疾病，主要是由影响肾上腺类固醇合成和激活途径的遗传缺陷引起的。这些病症表现出不同的临床表现，可以通过其独特的分子机制和类固醇特征来区分。及时诊断和定制管理方法对于减轻与不受控制的高血压和其他相关疾病相关的不利后果至关重要。这些疾病的治疗选择取决于不同的潜在病理生理学，在某些情况下涉及特定的药物治疗或手术肾上腺切除术。本文总结了先天性内分泌高血压治疗管理的最新知识，重点关注家族性醛固酮增多症 (FH)、先天性肾上腺增生、明显盐皮质激素过多和 Liddle 综合征。我们概述了每种疾病的遗传和分子发病机制，描述了临床特征，并讨论了各种可用的治疗方法及其不良反应的风险，旨在改善患有这些罕见和复杂疾病的患者的预后。