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引用次数: 0
摘要
一旦排除了外源性病因,库欣综合征(CS)是一种罕见的疾病。然而,一旦确诊,大多数病例都是促肾上腺皮质激素(ACTH)依赖型,其中相当一部分病例是由于垂体以外的原因引起的,即异位 ACTH 综合征(EAS)。区分垂体依赖性 CS、库欣病(CD)和异位源可能存在问题。由于评估 CS 患者的非侵入性检查往往缺乏足够的灵敏度和特异性,因此在检查 ACTH 依赖性 CS 时进行的微创手术--双侧下鼻底窦取样(BIPSS)会非常有帮助。BIPSS 被认为是区分 CD 和 EAS 的金标准。此外,尽管这种鉴别可能确实具有挑战性,但 BIPSS 本身也是一项复杂的检查,尤其是近来由于促肾上腺皮质激素释放激素的广泛停用并被去氨加压素所取代。我们回顾了目前已发表的有关该检查的数据,并根据这些数据和我们自己的经验,讨论了在诊断算法中使用该方法的适当性。
Bilateral inferior petrosal sinus sampling in the differential diagnosis of ACTH-dependent Cushing's syndrome: A reappraisal
Cushing's syndrome (CS) is a rare disorder, once exogenous causes have been excluded. However, when diagnosed, the majority of cases are adrenocorticotropic hormone (ACTH)-dependent, of which a substantial minority are due to a source outside of the pituitary, ectopic ACTH syndrome (EAS). Differentiating among pituitary-dependent CS, Cushing's disease (CD) and an ectopic source can be problematic. Because non-invasive tests in the evaluation of CS patients often lack adequate sensitivity and specificity, bilateral inferior petrosal sinus sampling (BIPSS), a minimally invasive procedure performed during the investigation of ACTH-dependent CS, can be extremely helpful. BIPSS is considered to be the gold standard for differentiating CD from the EAS. Furthermore, although such differentiation may indeed be challenging, BIPSS is itself a complex investigation, especially in recent times due to the widespread withdrawal of corticotrophin-releasing hormone and its replacement by desmopressin. We review current published data on this investigation and, in the light of this and our own experience, discuss its appropriate use in diagnostic algorithms.
期刊介绍:
JIM – The Journal of Internal Medicine, in continuous publication since 1863, is an international, peer-reviewed scientific journal. It publishes original work in clinical science, spanning from bench to bedside, encompassing a wide range of internal medicine and its subspecialties. JIM showcases original articles, reviews, brief reports, and research letters in the field of internal medicine.