一名妊娠前期患者的库欣病与假性库欣综合征的鉴别诊断》(The Differential Diagnosis between Cushing's Disease and Pseudocushing Syndrome in a Pre-agonic Patient.

Simone Antonio De Sanctis, Sabrina Chiloiro, Eloisa Sofia Tanzarella, Filippo Bongiovanni, Antonella Giampietro, Gennaro De Pascale, Laura De Marinis, Massimo Antonelli, Alfredo Pontecorvi, Antonio Bianchi
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摘要

背景:假性库欣综合征包括几种可在高应激情况下发生的疾病,其生化特征与库欣综合征相似。我们报告了一例因实验室结果重叠而难以鉴别诊断的病例:一名 74 岁的男性因呼吸困难加重一个月、前几个月体重下降 15 公斤、气喘、肌张力减退和肌肉萎缩而住进我院。此外,由于出现急性缺氧性高碳酸血症呼吸衰竭,患者接受了无创通气治疗,随后因对甲氧西林敏感的金黄色葡萄球菌和克雷伯氏菌引起的肺炎和呼吸衰竭被送入重症监护室。抗生素治疗已经开始。在重症监护室治疗期间,患者因呼吸功能恶化而接受了气管插管,并因出现脓毒性休克而接受了肌力药物治疗。对激素进行了检测,结果显示存在 ACTH 依赖性皮质醇过多症。Nugent、Liddle和地塞米松抑制CRH刺激试验的结果表明,患者存在非肿瘤性ACTH依赖性皮质醇过多症。垂体对比磁共振成像显示腺体肥大,腹部计算机断层扫描排除了肾上腺病变。不幸的是,患者出现了多器官功能衰竭,最终不治身亡。尸检结果证实没有垂体和其他神经内分泌肿瘤,并显示双侧肾上腺功能减退:我们的临床病例描述了一名在脓毒症和前驱期出现假性库欣综合征的患者,其鉴别诊断十分困难,而小剂量地塞米松抑制试验和CRH试验的联合应用则为其提供了确凿和正确的诊断方向。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
The Differential Diagnosis between Cushing's Disease and Pseudocushing Syndrome in a Pre-agonic Patient.

Background: The pseudo-Cushing's encompass several disorders that can occur in high-stress situations and that show biochemical features like those of Cushing's syndrome. We present a case with difficult differential diagnosis for overlapping laboratory findings.

Case report: A 74-year-old man was admitted to our hospital for worsening dyspnoea for a month, 15 kilograms of weight loss in the previous months, asthenia, hypotonia, and muscle hypotrophy. Moreover, due to the onset of acute hypoxic-hypercapnic respiratory failure, the patient was treated with non-invasive ventilation and then admitted to the intensive care unit, for pneumonia and respiratory failure due to Meticillin-sensitive Staphylococcus aureus and Klebsiella Aerogenes. Antibiotic therapy was started. During the treatment in the ICU, the patient underwent endotracheal intubation for the worsening of respiratory function, and inotropic drug therapy was introduced for the development of septic shock. Hormones were tested, showing an ACTH-dependent hypercortisolism. The results of Nugent, Liddle, and the dexamethasone- suppressed CRH stimulation tests suggested a not-neoplastic ACTH-dependent hypercortisolism. The pituitary contrasted magnetic resonance image showed gland hypertrophy, and the abdominal computed tomography ruled out adrenal lesions. Unfortunately, the patient developed a multi-organ failure and died. The autopsy finding confirmed the absence of pituitary and other neuroendocrine tumors and showed bilateral adrenal hypotrophy.

Conclusion: Our clinical case described a patient with pseudo-Cushing's syndrome during sepsis and pre-agonist phase, with a difficult differential diagnosis, in which the combination of the low-dose dexamethasone suppression test and the CRH test allowed a conclusive and correct diagnostic orientation.

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