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肾上腺功能不全及双侧肾上腺肿块的罕见病因。

An unusual cause of adrenal insufficiency and bilateral adrenal masses.

作者信息

Tee Su Ann, Gan Earn Hui, Kanaan Mohamad Zaher, Price David Ashley, Hoare Tim, Pearce Simon H S

机构信息

Department of Endocrinology, Newcastle Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK.

Institute of Genetic Medicine, Newcastle University, Newcastle Upon Tyne, UK.

出版信息

Endocrinol Diabetes Metab Case Rep. 2018 Jul 6;2018. doi: 10.1530/EDM-18-0030. eCollection 2018.

DOI:10.1530/EDM-18-0030
PMID:30002834
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6038009/
Abstract

UNLABELLED

Primary adrenal insufficiency secondary to syphilis is extremely rare, with only five cases being reported in the literature. We report a case of adrenal insufficiency as a manifestation of infection (tertiary syphilis). A 69-year-old, previously fit and well Caucasian male was found to have adrenal insufficiency after being admitted with weight loss, anorexia and postural dizziness resulting in a fall. Biochemical testing showed hyponatraemia, hyperkalaemia, and an inadequate response to Synacthen testing, with a peak cortisol level of 302 nmol/L after administration of 250 µg Synacthen. Abdominal imaging revealed bilateral adrenal hyperplasia with inguinal and retroperitoneal lymphadenopathy. He was started on hydrocortisone replacement; however, it was not until he re-attended ophthalmology with a red eye and visual loss 1 month later, that further work-up revealed the diagnosis of tertiary syphilis. Following a course of penicillin, repeat imaging 5 months later showed resolution of the abnormal radiological appearances. However, adrenal function has not recovered and 3 years following initial presentation, the patient remains on both glucocorticoid and mineralocorticoid replacement. In conclusion, this case highlights the importance of considering syphilis as a potential differential diagnosis in patients presenting with adrenal insufficiency and bilateral adrenal masses, given the recent re-emergence of this condition. The relative ease of treating infectious causes of adrenal lesions makes accurate and timely diagnosis crucial.

LEARNING POINTS

Infectious causes, including syphilis, should be excluded before considering adrenalectomy or biopsy for any patient presenting with an adrenal mass.It is important to perform a full infection screen including tests for human immunodeficiency virus, other blood-borne viruses and concurrent sexually transmitted diseases in patients presenting with bilateral adrenal hyperplasia with primary adrenal insufficiency.Awareness of syphilis as a potential differential diagnosis is important, as it not only has a wide range of clinical presentations, but its prevalence has been increasing in recent times.

摘要

未标注

梅毒继发的原发性肾上腺皮质功能减退极为罕见,文献中仅报道过5例。我们报告1例肾上腺皮质功能减退作为梅毒感染(三期梅毒)表现的病例。一名69岁、既往健康的白种男性,因体重减轻、厌食和体位性头晕导致跌倒入院后,被发现存在肾上腺皮质功能减退。生化检查显示低钠血症、高钾血症,以及对促肾上腺皮质激素试验反应不足,给予250μg促肾上腺皮质激素后皮质醇峰值水平为302nmol/L。腹部影像学检查显示双侧肾上腺增生伴腹股沟和腹膜后淋巴结病。他开始接受氢化可的松替代治疗;然而,直到1个月后他因眼红和视力丧失再次就诊眼科时,进一步检查才确诊为三期梅毒。经过一个疗程的青霉素治疗后,5个月后的重复影像学检查显示异常影像学表现消退。然而,肾上腺功能并未恢复,在初次就诊3年后,患者仍在接受糖皮质激素和盐皮质激素替代治疗。总之,鉴于这种疾病近期的再度出现,本病例凸显了在肾上腺皮质功能减退和双侧肾上腺肿块患者中考虑梅毒作为潜在鉴别诊断的重要性。肾上腺病变感染病因相对易于治疗,这使得准确及时的诊断至关重要。

学习要点

对于任何出现肾上腺肿块的患者,在考虑肾上腺切除术或活检之前,应排除包括梅毒在内的感染病因。对于出现双侧肾上腺增生伴原发性肾上腺皮质功能减退的患者,进行全面的感染筛查,包括检测人类免疫缺陷病毒、其他血源病毒和同时存在的性传播疾病,这很重要。认识到梅毒作为潜在鉴别诊断的重要性,因为它不仅有广泛的临床表现,而且其患病率近年来一直在上升。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/6038009/bec8b284d529/edmcr-2018-180030-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/6038009/3fec904e3c92/edmcr-2018-180030-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/6038009/bec8b284d529/edmcr-2018-180030-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/6038009/3fec904e3c92/edmcr-2018-180030-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/6038009/bec8b284d529/edmcr-2018-180030-g002.jpg

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本文引用的文献

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Syphilis: Re-emergence of an old foe.梅毒:宿敌的再度出现。
Microb Cell. 2016 Jun 27;3(9):363-370. doi: 10.15698/mic2016.09.523.
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A case of adrenal insufficiency secondary to syphilis and difficulties in the diagnostic work-up.梅毒继发肾上腺功能不全 1 例及诊断困难
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Gummatous syphilis of the adrenal gland.肾上腺树胶肿性梅毒。
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