Babu Ambika R, Dwarakanathan Arcot A
Department of Medicine, Rush-Presbyterian-St. Luke's Medical Center, Rush University, 1725 West Harrison Street, Suite 250, Chicago, IL 60612-3824, USA.
Endocr Pract. 2003 May-Jun;9(3):229-32. doi: 10.4158/EP.9.3.229.
To present a case of a young woman with new-onset diabetes mellitus resistant to insulin attributable to Cushing's syndrome caused by ectopic production of corticotropin by a metastatic gastrinoma.
We summarize the clinical presentation and the pertinent laboratory values in a patient with Cushing's syndrome. A review of the literature regarding ectopic production of corticotropin by gastrinomas is also presented.
A 26-year-old woman with dehydration, severe hyperglycemia, and hypokalemia was seen in consultation. The patient required large doses of insulin to control plasma glucose, and further work-up confirmed the presence of Cushing's syndrome caused by ectopic production of corticotropin from a metastatic gastrinoma.
This case is unusual in that the patient was relatively young and the clinical presentation of Cushing's syndrome was dominated by uncontrolled diabetes, insulin resistance, and hypokalemia. At the time of this diagnosis, the patient already had evidence of multiple liver metastatic lesions from a pancreatic gastrinoma. The rapid occurrence of difficult-to-treat diabetes and hypokalemia should raise the suspicion of Cushing's syndrome from ectopic production of corticotropin. In fact, patients with metastatic pancreatic tumors and poorly controlled diabetes with hypokalemia should undergo evaluation for Cushing's syndrome, even in the absence of the typical stigmas, because of rapid development of the disease and high levels of corticotropin.
报告一例年轻女性新发糖尿病，对胰岛素抵抗，其病因是转移性胃泌素瘤异位分泌促肾上腺皮质激素导致的库欣综合征。
我们总结了一名库欣综合征患者的临床表现及相关实验室检查结果。同时也对胃泌素瘤异位分泌促肾上腺皮质激素的相关文献进行了综述。
会诊了一名26岁脱水、严重高血糖和低钾血症的女性患者。该患者需要大剂量胰岛素来控制血糖，进一步检查证实存在由转移性胃泌素瘤异位分泌促肾上腺皮质激素所致的库欣综合征。
该病例不同寻常之处在于患者相对年轻，且库欣综合征的临床表现以难以控制的糖尿病、胰岛素抵抗和低钾血症为主。在确诊时，患者已有胰腺胃泌素瘤多发肝转移灶的证据。难治性糖尿病和低钾血症的快速出现应引起对异位分泌促肾上腺皮质激素所致库欣综合征的怀疑。事实上，患有转移性胰腺肿瘤且糖尿病控制不佳伴有低钾血症的患者，即使没有典型体征，也应接受库欣综合征评估，因为该病发展迅速且促肾上腺皮质激素水平高。
