成人垂体功能减退:合理的诊断和治疗方法

Mussa H. Almalki
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摘要

垂体激素负责调节生长、发育、代谢、繁殖和体内平衡。垂体功能减退症是一种被定义为部分或完全垂体前叶激素分泌不足,很少有垂体后叶激素分泌不足的情况。这种情况可由脑下垂体或下丘脑疾病引起。垂体功能减退症的年发病率估计为每10万人4.2例,患病率估计为每10万人45.5例。垂体功能减退症的症状各不相同。发病是隐匿的,取决于激素缺乏的数量和严重程度。垂体激素缺乏可导致实质性的临床变化,增加发病率和死亡率的风险。人们通常报告持续的症状和生活质量下降,这两种情况都可以解释,至少部分原因是激素替代策略在模仿正常激素分泌过程方面的固有缺陷。垂体功能减退症的诊断可以直接通过测量降低的基础激素水平。在基础激素水平不确定或已确定部分激素缺乏的情况下,可能有必要对下丘脑-垂体轴进行刺激试验。下丘脑和垂体区可以使用磁共振成像成像,这提供了有用的解剖信息。必要时,可以在诊断方法中加入基因研究。治疗包括个体终末器官激素缺乏的生理替代,并在患者的整个生命中需要仔细监测。建议考虑潜在相互作用的个体化激素替代疗法。本文概述了垂体功能减退症的病理生理学、临床表现、一般诊断指南和治疗方案。
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Hypopituitarism in Adults: Rational Approaches to Diagnosis and Treatment
Abstract Pituitary hormones are responsible for the regulation of growth, development, metabolism, reproduction, and homeostasis. Hypopituitarism is a condition that is defined as partial or complete insufficiency of anterior pituitary hormone secretion, and rarely, posterior pituitary hormone secretion. This condition can result from diseases of the pituitary gland or the hypothalamus. The annual incidence of hypopituitarism has been estimated to be 4.2 per 100,000 yearly, and the prevalence has been estimated at 45.5 per 100,000. The symptoms of hypopituitarism vary. The onset is insidious and depends on the number of hormone deficiencies and their degree of severity. Pituitary hormone deficiency can result in substantial clinical changes that increase the risk of morbidity and mortality. People commonly report persistent symptoms and a decline in their quality of life, both of which can be explained, at least in part, by the inherent shortcomings of hormone replacement strategies in their ability to imitate the normal hormone secretion processes. The diagnosis of hypopituitarism can be straightforward by measuring the lowered basal hormone levels. In cases where the basal hormone levels are uncertain or partial hormone deficiencies have been identified, it may be necessary to perform provocative testing of the hypothalamic–pituitary axis. The hypothalamus and pituitary region can be imaged using magnetic resonance imaging, which provides useful anatomical information. When necessary, genetic studies may be added to the diagnostic approach. The treatment consists of physiological replacement of the individual end-organ hormone deficiencies, and careful monitoring is required throughout the patient's entire life. Individualized hormone replacement therapy that considers potential interactions is recommended. This article provides an overview of the pathophysiology, clinical presentation, general diagnostic guidelines, and treatment options of hypopituitarism.
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