危重肾上腺库欣综合征临床分析

The clinical analysis of severe adrenal Cushing′s syndrome

目的 探讨危重肾上腺库欣综合征治疗方法及其疗效.方法 回顾性分析在本院诊治的22例危重肾上腺库欣综合征（危重组）与136例非危重肾上腺库欣综合征（非危重组） ,通过比较危重组和非危重组之间入院时以及术前准备后差异,危重组手术前后差异,分析危重肾上腺库欣综合征的临床特点、临床处理及手术后的疗效.结果 危重组入院时,血尿皮质醇水平、病程、血压方面均显著高于非危重组（P＜0.05或P＜0.01）,而血钾、ACTH显著低于非危重组[（3.01±0.75对3.62±0.48）mmol/L, P＜0.01;（6.47±2.91对8.21±3.22）pg/ml, P＜0.05],术前准备后舒张压、血钾、空腹血糖与非危重组无明显差异（均P＞0.05）,而后行肾上腺手术治疗,手术3个月后22例患者均不同程度缓解,随访过程中,5例原发性双侧大结节性肾上腺增生（primary bilateral macronodular adrenal hyperplasia, BMAH）及1例原发性色素性肾上腺结节增生（primary pigmented nodular adrenocortical disease, PPNAD）再次行对侧肾上腺全切除术.结论 危重肾上腺库欣综合征患者因长期受高血皮质醇水平影响,其合并症程度更为严重,需在充分的术前准备后行肾上腺手术治疗,若术前无法达到预期,可使用降皮质醇药物或急诊单侧肾上腺切除术.危重BMAH及PPNAD患者可先行单侧肾上腺全切术,因复发风险较高,需密切随访,及时行对侧肾上腺切除术.

Objective To investigate the methods and efficacy of treatment on severe adrenal Cushing′s syndrome. Methods The clinical data of 22 cases with severe adrenal Cushing′s syndrome （ severe group） , and 136 cases with mild or moderate adrenal Cushing′s syndrome （ non-severe group） were reviewed. The clinical features were analyzed by comparing the differences between these two groups when patients were admitted to hospital. We discussed the clinical managements of patients with severe adrenal Cushing′s syndrome by comparing the differences with non-severe group after preoperative preparation, and with themselves before and after preoperative preparation. The effects of surgery were evaluated by comparing the differences between pre-operation and post-operation on patients with severe adrenal Cushing′s syndrome. Results At admission, serum/urine cortisol, disease course, and blood pressure were significantly higher in the severe group than those in non-severe group （ P＜0.05 or P＜0.01） , serum potassium and ACTH level were decreased significantly in the severe group than those innon-severegroup[（3.01±0.75vs3.62±0.48）mmol/L,P＜0.01;（6.47±2.91vs8.21±3.22）pg/ml,P＜0.01] . However, no significant difference was observed in diastolic blood pressure, serum potassium, and fasting plasma glucose between these two groups after preoperative preparation （all P＞0.05）. And then, we performed adrenalectomy. The symptoms of 22 cases with severe adrenal Cushing′s syndrome were obviously alleviated after 3 months. During follow-up, 5 cases of primary bilateral macronodular adrenal hyperplasia （ BMAH） and 1 case of primary pigmented nodular adrenocortical disease （ PPNAD ） were treated with contralateral adrenalectomy. Conclusion Sufficient preoperative preparation is essential for patients with severe adrenal Cushing′s syndrome because of its high level serum cortisol with severe complications. If preparation fails before surgery, cortisol-lowering medication or emergency unilateral adrenalectomy is necessary. Severe patients with BMAH and PPNAD were firstly performed unilateral adrenalectomy and followed-up closely, and then, contralateral adrenalectomy is needed when the recurrence of hypercortisolism recognized.