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动脉血栓形成作为内源性库欣综合征的主要表现

Arterial thrombosis as primary presentation of endogenous Cushing's syndrome.

作者信息

Alexander Vijay, Koshy Maria, Dasgupta Riddhi, Carey Ronald Albert

机构信息

Department of Internal Medicine, Christian Medical College, Vellore, Tamil Nadu, India.

Department of Endocrinology, Diabetes and Metabolism, Christian Medical College, Vellore, Tamil Nadu, India.

出版信息

BMJ Case Rep. 2019 Feb 6;12(2):e227491. doi: 10.1136/bcr-2018-227491.

DOI:10.1136/bcr-2018-227491
PMID:30733248
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6381946/
Abstract

Cushing's syndrome is known to present with a characteristic set of clinical manifestations and complications, well described in literature. However, hypercoagulability remains an under recognised entity in Cushing's syndrome. A 31-year-old woman from Southern India presented with history of fever, left upper quadrant pain and progressive breathing difficulty for 3 weeks. Clinical examination revealed discriminatory features of Cushing's syndrome. Laboratory investigations showed biochemical features of endogenous ACTH-dependent Cushing's syndrome. Imaging of the abdomen revealed splenic collection, left-sided empyema and extensive arterial thrombosis. Gadolinium enhanced dynamic MRI of the pituitary gland revealed no evidence of an adenoma while a Ga-68 DOTATATE positron emission tomography CT scan ruled out an ectopic Cushing's. A diagnosis of endogenous Cushing's syndrome causing a prothrombotic state with extensive arterial thrombosis was made. She was initiated on oral anticoagulation and oral ketoconazole for medical adrenal suppression. She subsequently underwent bilateral adrenalectomy and was well at follow-up.

摘要

库欣综合征以一系列特征性临床表现和并发症为特点,在文献中有详细描述。然而,高凝状态在库欣综合征中仍是一个未被充分认识的问题。一名来自印度南部的31岁女性,有发热、左上腹疼痛和进行性呼吸困难3周的病史。临床检查发现了库欣综合征的鉴别特征。实验室检查显示为内源性促肾上腺皮质激素依赖性库欣综合征的生化特征。腹部影像学检查发现脾脓肿、左侧脓胸和广泛的动脉血栓形成。钆增强动态垂体磁共振成像未发现腺瘤证据,而Ga-68 DOTATATE正电子发射断层扫描计算机断层扫描排除了异位库欣综合征。诊断为内源性库欣综合征导致促血栓形成状态并伴有广泛动脉血栓形成。她开始接受口服抗凝治疗和口服酮康唑以进行药物性肾上腺抑制。她随后接受了双侧肾上腺切除术,随访时情况良好。

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本文引用的文献

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Ectopic ACTH syndrome complicated by multiple opportunistic infections treated with percutaneous ablation of the adrenal glands.异位促肾上腺皮质激素综合征合并多处机会性感染,采用经皮肾上腺消融术治疗。
BMJ Case Rep. 2017 Nov 14;2017:bcr-2017-221580. doi: 10.1136/bcr-2017-221580.
2
Thrombophilia Testing in Provoked Venous Thromboembolism: A Teachable Moment.诱发静脉血栓栓塞症中的血栓形成倾向检测:一个可资借鉴的案例。
JAMA Intern Med. 2017 Aug 1;177(8):1195-1196. doi: 10.1001/jamainternmed.2017.1815.
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Acute mesenteric ischemia and hepatic infarction after treatment of ectopic Cushing's syndrome.异位库欣综合征治疗后出现急性肠系膜缺血和肝梗死。
Endocrinol Diabetes Metab Case Rep. 2017 Feb 28;2017. doi: 10.1530/EDM-16-0144. eCollection 2017.
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Comparison of 68Ga-DOTANOC PET/CT and contrast-enhanced CT in localisation of tumours in ectopic ACTH syndrome.68Ga-DOTANOC PET/CT 与增强 CT 对异位 ACTH 综合征肿瘤定位的比较。
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Guidance for the evaluation and treatment of hereditary and acquired thrombophilia.遗传性和获得性血栓形成倾向的评估与治疗指南。
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Cushing's syndrome: epidemiology and developments in disease management.库欣综合征:流行病学和疾病管理的进展。
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Massive thoracoabdominal aortic thrombosis in a patient with iatrogenic Cushing syndrome.一名医源性库欣综合征患者发生大量胸腹主动脉血栓形成。
Korean J Radiol. 2014 Sep-Oct;15(5):637-40. doi: 10.3348/kjr.2014.15.5.637. Epub 2014 Sep 12.
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Cardiac structure and function in Cushing's syndrome: a cardiac magnetic resonance imaging study.库欣综合征的心脏结构和功能:一项心脏磁共振成像研究。
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Indian J Endocrinol Metab. 2013 Dec;17(Suppl 3):S612-7. doi: 10.4103/2230-8210.123548.
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