一种由于部分肾上腺功能不全而导致的Nelson综合征的独特呈现,未进行双侧肾上腺切除。
A Unique Presentation of Nelson Syndrome Due to Partial Adrenal Insufficiency Without Bilateral Adrenalectomy.
发表日期:2023 Aug
作者:
Priyanka D Desai, Smita Kargutkar, Raveena Nalla
来源:
PHYSICAL THERAPY & REHABILITATION JOURNAL
摘要:
尽管没有进行双侧肾上腺切除手术,但该病例报告调查了一位58岁的亚洲女性,她表现出类似Nelson综合征的临床特征。患者过去患有一个分泌ACTH的垂体巨腺瘤,因此进行了经蝶窦切除术以及放射治疗。术后一年,患者出现严重的面部和颈部色素沉着。根据获得的实验室结果,患者显示出初始较高的ACTH水平和低正常的晨间皮质醇水平,这些都是部分肾上腺功能不全的表现。进行了脑MRI检查,确认了海绵窦中残留肿瘤组织的稳定性。结果指出,肾上腺是色素沉着的原因,患者被诊断为原发性肾上腺功能不全。为了将她的ACTH水平和低正常的晨间皮质醇水平恢复到正常范围,给予了低剂量的氢化可的松,并对其进行了五年的监测。在这段时间里,患者的色素沉着显著改善,并最终完全消失,她的ACTH水平降低,表明氢化可的松是正常化ACTH水平的适当治疗方法。在这种情况下,确定了不响应的肾上腺导致ACTH水平升高,从而导致了Nelson综合征的非典型病例和色素沉着的身体症状。
Copyright © 2023, Desai et al.
Nelson syndrome is a rare disorder, characterized by clinical features arising from an adrenocorticotropic hormone (ACTH)-secreting pituitary macroadenoma after bilateral adrenalectomy. Common symptoms of Nelson syndrome include weight gain, vision problems, and skin hyperpigmentation, among many others. In this case report, a 58-year-old Asian female who displayed clinical features akin to Nelson syndrome despite not undergoing bilateral adrenalectomy is investigated. The patient has a past history of an ACTH-secreting pituitary macroadenoma, for which a transsphenoidal resection was performed along with radiation therapy. A year following this, she displayed severe facial and neck hyperpigmentation. According to the laboratory results obtained, the patient displayed initial high ACTH levels and low-normal AM cortisol levels, which are signs of partial adrenal insufficiency. A brain MRI was performed, which confirmed stable residual tumor tissue in the cavernous sinus. The results pointed to the adrenal glands as the cause of the hyperpigmentation, and the patient was diagnosed with primary adrenal insufficiency. To bring her ACTH levels and low-normal AM cortisol into the proper range, she was given low-dose hydrocortisone and monitored for five years. Over this time period, her hyperpigmentation improved significantly and eventually resolved entirely, and her ACTH levels were lowered, indicating that hydrocortisone was the appropriate treatment for normalizing ACTH levels. In this case, it was determined that unresponsive adrenal glands lead to high ACTH levels, which resulted in an atypical case of Nelson syndrome and the physical symptom of hyperpigmentation.Copyright © 2023, Desai et al.