奥西洛司他治疗异位分泌促肾上腺皮质激素的神经内分泌肿瘤引起的严重库欣综合征。

IF 0.7 Q4 ENDOCRINOLOGY & METABOLISM
Václav Hána, Tomáš Brutvan, Adéla Krausová, Michal Kršek, Václav Hána
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引用次数: 0

摘要

摘要:由异位促肾上腺皮质激素产生肿瘤引起的严重库欣综合征是罕见的,但通常需要快速诊断和治疗皮质醇增多症及其合并症。酮康唑、美吡酮和奥西洛司他对皮质醇增多症的药物治疗目前可用。如果不成功或不充分,可以选择双侧肾上腺切除术。我们报告一名28岁女性,患有由支气管转移性神经内分泌肿瘤(NET)引起的严重库欣综合征。奥西洛司他治疗皮质醇增多症疗效确切,先阻断后滴定。每天一次的剂量最终被用于皮质醇水平的正常化。液相色谱-质谱法测定的雄激素水平在治疗期间略有升高,但没有任何症状。在一名患有无法治愈的支气管网的严重库欣综合征患者中,每天一次简单的奥西洛司他滴定方案可使皮质醇水平正常化。治疗期间出现短暂性皮质醇分泌不足,但氢化可的松很容易治疗。学习要点:由异位促肾上腺皮质激素产生肿瘤引起的库欣综合征是罕见的。皮质醇的上调通常是严重而迅速的,尽管临床症状并不总是完全明显。快速治疗是预防和减少并发症的关键,如骨折、血栓栓塞、出血、高血糖和动脉高压。新型强效甾体生成抑制剂osilodrostat可作为减少皮质醇增多症的一线治疗药物。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Severe Cushing's syndrome from an ectopic adrenocorticotropic hormone-secreting neuroendocrine tumour treated by osilodrostat.

Severe Cushing's syndrome from an ectopic adrenocorticotropic hormone-secreting neuroendocrine tumour treated by osilodrostat.

Severe Cushing's syndrome from an ectopic adrenocorticotropic hormone-secreting neuroendocrine tumour treated by osilodrostat.

Summary: Severe Cushing's syndrome from an ectopic adrenocorticotropic hormone-producing tumour is rare but often demands rapid diagnostics and treatment of hypercortisolism with its comorbidities. Pharmacotherapy of hypercortisolism by ketoconazole, metyrapone and osilodrostat is currently available. If unsuccessful or insufficient a bilateral adrenalectomy is an option. We present a 28-year-old female with severe Cushing's syndrome caused by a bronchial metastatic neuroendocrine tumour (NET). Hypercortisolism was efficiently treated by osilodrostat with block-replace and then titration regimen. A once-daily dose was finally used with normalised cortisol levels. Androgen levels measured by liquid chromatography-mass spectrometry were slightly elevated during the treatment but without any symptoms. A simple once-daily use of osilodrostat with titration regimen led to normalised cortisol levels in a severe Cushing's syndrome patient with an uncurable bronchial NET. Transient hypocortisolism during treatment appeared but was easily treated by hydrocortisone.

Learning points: Cushing's syndrome from an ectopic adrenocorticotropic hormone-producing tumour is rare. Cortisol upregulation is often severe and rapid, though clinical signs are not always fully pronounced. Rapid treatment is a key for preventing and reducing complications such as fractures, thromboembolism, bleeding, hyperglycaemia, and arterial hypertension. The novel potent steroidogenesis inhibitor osilodrostat can be used as first-line treatment for reducing hypercortisolism.

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来源期刊
CiteScore
1.50
自引率
0.00%
发文量
142
审稿时长
9 weeks
期刊介绍: Endocrinology, Diabetes & Metabolism Case Reports publishes case reports on common and rare conditions in all areas of clinical endocrinology, diabetes and metabolism. Articles should include clear learning points which readers can use to inform medical education or clinical practice. The types of cases of interest to Endocrinology, Diabetes & Metabolism Case Reports include: -Insight into disease pathogenesis or mechanism of therapy - Novel diagnostic procedure - Novel treatment - Unique/unexpected symptoms or presentations of a disease - New disease or syndrome: presentations/diagnosis/management - Unusual effects of medical treatment - Error in diagnosis/pitfalls and caveats
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