M. Stasiak, Przemysław Witek, Emilia Adamska-Fita, Andrzej Lewiński
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引用次数: 0
摘要
摘要 库欣病(CD)是内源性皮质醇过多症最常见的病因。奥昔洛司他被证明能有效治疗库欣病,控制库欣病所需的平均剂量小于11毫克/天。奥昔洛司他治疗皮质醇分泌腺瘤(CPA)和 CD 的潜在差异尚未见报道。本研究旨在介绍两名皮质醇分泌性腺瘤(CPA)患者,发现他们对治疗的反应与皮质醇分泌性腺瘤有显著差异。我们展示了一例皮质醇水平与肾上腺肿瘤在初始剂量升级期间的进展呈反向反应的病例(病例 1)。与此同时,高皮质醇症症状严重加剧,并出现了危及生命的低钾血症。进一步快速增加剂量后,皮质醇在 20 毫克/天的剂量下首次出现明显反应,在 45 毫克/天的剂量下出现完全反应。我们还介绍了一个最初对治疗有抵抗力的病例(病例 2)。首次反应和完全反应所需的剂量与病例 1 相同。我们的研究表明,对 CPA 患者进行奥西洛德司他治疗可能需要采用与 CD 不同的方法,即更高的剂量、更快的剂量升级以及可能出现的初始逆反应或无应答。
Response to Osilodrostat Therapy in Adrenal Cushing’s Syndrome
Abstract Cushing’s disease (CD) is the most common cause of endogenous hypercortisolism. Osilodrostat was demonstrated to be efficient in treating CD, and the mean average dose required for CD control was <11 mg/day. Potential differences in osilodrostat treatment between cortisol-producing adenoma (CPA) and CD have not been reported. The aim of this study was to present two patients with CPA in whom significant differences in the response to therapy compared to CD were found. We demonstrated a case of inverse response of cortisol levels with adrenal tumor progression during the initial dose escalation (Case 1). Simultaneously, severe exaggeration of hypercortisolism symptoms and life-threatening hypokalemia occurred. A further rapid dose increase resulted in the first noticeable cortisol response at a dose of 20 mg/day, and a full response at a dose of 45 mg/day. We also present a case that was initially resistant to therapy (Case 2). The doses required to achieve the first response and the full response were the same as those for Case 1. Our study demonstrated that osilodrostat therapy in patients with CPA may require a different approach than that in CD, with higher doses, faster dose escalation, and a possible initial inverse response or lack of response.