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腹腔镜肾上腺切除术后肾上腺功能不全患者长期糖皮质激素治疗的预测因素。

Factors predicting prolonged glucocorticoid therapy in patients with adrenal insufficiency after laparoscopic adrenalectomy.

作者信息

Ziglioli Francesco, Cataldo Simona, Cavalieri Domenico Maria, Campobasso Davide, Maestroni Umberto

机构信息

University-Hospital of Parma, Department of Urology, Italy.

University-Hospital of Parma, Department of Endocrinology, Italy.

出版信息

Ann Med Surg (Lond). 2022 Mar 10;77:103390. doi: 10.1016/j.amsu.2022.103390. eCollection 2022 May.

Abstract

INTRODUCTION AND IMPORTANCE

Adrenal insufficiency (AI) is common after adrenalectomy for Primary Adrenal Cushing's syndrome (PACS), due to the inhibition of the Hypothalamic-Pituitary-Adrenal Axis (HPAA) by the functioning adrenal mass. The treatment of post-surgical AI is based mainly on glucocorticoid supplementation therapy. To date, however, there is no known predicting factor of the duration of supplementation therapy in patients treated with laparoscopic adrenalectomy for PACS.

CASE PRESENTATION

We report the case of a 22-year-old Caucasian female who presented with dyspnea, osteoporosis, vertebral collapses and fractures of the pelvis. The diagnosis of ACTH-independent Cushing's syndrome was provided. Abdominal MRI revealed a left adrenal mass suggestive for adrenal adenoma, highly suggestive for PACS. The patient underwent left laparoscopic adrenalectomy. After surgery, glucorticoid supplementation therapy was started. More than A-year steroid replacement therapy was necessary before the patient completely recovered the function of the HPAA. During this period the patient was strictly followed up in order to adjust pharmacologic treatment, thus allowing to investigate the possible causes of such a slow and hard recover of the contralateral adrenal gland function.

CONCLUSION

AI is common after adrenalectomy for PACS due to HPAA suppression. The duration of steroid replacement therapy may be vary depending on patient's characteristics and may be uncommonly long, as in our case. We concluded the not only cortisol and ACTH level, but also radiological findings, such as the size of the mass, its functional activity as well as the hypotrophy or atrophy of the contralateral adrenal gland may be predictive of the duration of the steroid therapy. These factors, if correctly studied before surgery, may be of help in tailoring the postoperative management of the patients after adrenalectomy.

摘要

引言与重要性

原发性肾上腺皮质增生症(PACS)患者肾上腺切除术后肾上腺功能不全(AI)较为常见,这是由于功能性肾上腺肿块对下丘脑 - 垂体 - 肾上腺轴(HPAA)的抑制作用。术后AI的治疗主要基于糖皮质激素补充疗法。然而,迄今为止,对于接受腹腔镜肾上腺切除术治疗PACS的患者,尚无已知的补充疗法持续时间的预测因素。

病例报告

我们报告一例22岁的白种女性患者,她出现呼吸困难、骨质疏松、椎体塌陷和骨盆骨折。诊断为促肾上腺皮质激素(ACTH)非依赖性库欣综合征。腹部磁共振成像(MRI)显示左肾上腺肿块,提示肾上腺腺瘤,高度提示PACS。患者接受了左侧腹腔镜肾上腺切除术。术后开始糖皮质激素补充治疗。在患者HPAA功能完全恢复之前,需要超过一年的类固醇替代治疗。在此期间,对患者进行了严格随访,以调整药物治疗,从而能够研究对侧肾上腺功能恢复缓慢且困难的可能原因。

结论

由于HPAA受抑制,PACS患者肾上腺切除术后AI较为常见。类固醇替代治疗的持续时间可能因患者特征而异,并且可能像我们的病例一样异常长。我们得出结论,不仅皮质醇和ACTH水平,而且影像学检查结果,如肿块大小、其功能活性以及对侧肾上腺的萎缩或肥大,都可能预测类固醇治疗的持续时间。这些因素如果在手术前得到正确研究,可能有助于制定肾上腺切除术后患者的术后管理方案。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8951/9142400/742eba12e722/gr1.jpg

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