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嗜铬细胞瘤的不典型表现。

Unusual presentation of phaeochromocytoma.

作者信息

Hope D C D, Palan J M

机构信息

St George's Hospital, London, UK.

出版信息

BMJ Case Rep. 2016 May 10;2016:bcr2016214719. doi: 10.1136/bcr-2016-214719.

Abstract

A 44-year-old woman, with a background of heart, lung and renal transplantation secondary to cystic fibrosis and type 1 diabetes, presented with tachycardia, hyperglycaemia, nausea and vomiting. She was initially managed for diabetic ketoacidosis with severe dehydration. However, persistent episodic hypertension and tachycardia led the investigating team to identify significantly raised urinary metanephrines and a left-sided adrenal mass; Iodine-123-meta-iodobenzylguanidine single photon emission computer tomography scan (MIBG SPECT/CT) showed avid uptake of tracer, confirming a left-sided phaeochromocytoma. She was started on medical management and is awaiting an elective laparoscopic procedure. This case describes the presentation of a unilateral phaeochromocytoma as ketoacidosis in a patient with type 1 diabetes with no other apparent precipitant. This highlights the metabolic counter-regulatory effect of excess catecholamines in addition to the inotropic/chronotropic effects that are associated with this adrenal tumour. Recognition of atypical signs and symptoms may point towards an atypical precipitant of diabetic ketoacidosis-a medical emergency presenting to front-line clinicians.

摘要

一名44岁女性,有因囊性纤维化和1型糖尿病继发的心、肺和肾移植史,出现心动过速、高血糖、恶心和呕吐。她最初因严重脱水的糖尿病酮症酸中毒接受治疗。然而,持续性发作性高血压和心动过速促使调查团队发现尿间甲肾上腺素显著升高以及左侧肾上腺肿块;碘-123-间碘苄胍单光子发射计算机断层扫描(MIBG SPECT/CT)显示示踪剂摄取活跃,证实为左侧嗜铬细胞瘤。她开始接受药物治疗,正在等待择期腹腔镜手术。本病例描述了一名无其他明显诱因的1型糖尿病患者单侧嗜铬细胞瘤表现为酮症酸中毒。这突出了过量儿茶酚胺的代谢反调节作用以及与这种肾上腺肿瘤相关的变力性/变时性作用。认识非典型体征和症状可能提示糖尿病酮症酸中毒的非典型诱因——这是一种一线临床医生会遇到的医疗急症。

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