Lewandowski Krzysztof C, Malicka Katarzyna, Dąbrowska Katarzyna, Lewiński Andrzej
Department of Endocrinology and Metabolic Diseases, Polish Mother's Memorial Hospital - Research Institute, Lodz, Poland, Poland; Department of Endocrinology and Metabolic Diseases, Medical University of Lodz, Lodz, Poland, Poland.
Endokrynol Pol. 2017;68(4):468-471. doi: 10.5603/EP.2017.0052.
A 36-year-old woman was found to have a low morning ACTH concentration despite a history of Addison's disease. Past medical history: At the age of 23 years the subject developed Graves's disease, which was treated with radioiodine. At about the same time, she claimed to have two episodes of pancreatitis treated with cholecystectomy. About seven months later she was euthyroid on L-thyroxine (TSH 1.51 mIU/mL) but was admitted with hypotension, hyponatraemia (sodium 109 mmol/L), and low morning cortisol (119 nmol/L). Further investigations confirmed primary adrenal failure with ACTH concentration of 779 pg/mL (ref. range 0-60) prior to the dose of hydrocortisone. About nine years later she complained about tiredness. Clinically she was normotensive and not pigmented. BMI 22.3 kg/m². Periods were regular. ACTH concentration was surprisingly low (ACTH 8.53 pg/mL, ref. range 0-46), despite very low cortisol (3.37 nmol/L). She was admitted for further assessment.
Pituitary MRI scan was unremarkable. An insulin tolerance test was performed and showed a clear increase of ACTH (from 15.2 to 165 pg/mL). There was, however, hardly any increase of ACTH after CRH stimulation (from 6.05 pg/mL to 10.2 pg/mL), thus demonstrating central CRH resistance. In summary, this patient developed secondary adrenal failure in the setting of previous Addison's disease. Interestingly, hypoglycaemia (but not CRH) provided a stimulus for ACTH release, thus demonstrating CRH resistance. The case confirms that besides CRH, other factors are responsible for stimulation of the ACTH-cortisol axis during insulin tolerance test.
一名36岁女性虽有艾迪生病史，但晨起促肾上腺皮质激素（ACTH）浓度较低。既往病史：该患者23岁时患格雷夫斯病，接受放射性碘治疗。大约同时，她称有两次胰腺炎发作，接受了胆囊切除术。大约七个月后，她服用左甲状腺素时甲状腺功能正常（促甲状腺激素[TSH]为1.51 mIU/mL），但因低血压、低钠血症（血钠109 mmol/L）和晨起皮质醇水平低（119 nmol/L）入院。进一步检查证实为原发性肾上腺功能衰竭，在给予氢化可的松之前，ACTH浓度为779 pg/mL（参考范围0 - 60）。大约九年后，她抱怨疲劳。临床检查她血压正常，无色素沉着。体重指数（BMI）为22.3 kg/m²。月经规律。尽管皮质醇水平极低（3.37 nmol/L），但ACTH浓度却出奇地低（ACTH为8.53 pg/mL，参考范围0 - 46）。她入院接受进一步评估。
垂体磁共振成像（MRI）扫描无异常。进行了胰岛素耐量试验，结果显示ACTH明显升高（从15.2 pg/mL升至165 pg/mL）。然而，促肾上腺皮质激素释放激素（CRH）刺激后ACTH几乎没有升高（从6.05 pg/mL升至10.2 pg/mL），从而表明存在中枢性CRH抵抗。总之，该患者在既往艾迪生病的基础上发生了继发性肾上腺功能衰竭。有趣的是，低血糖（而非CRH）刺激了ACTH释放，从而证明存在CRH抵抗。该病例证实，在胰岛素耐量试验期间，除CRH外，其他因素也负责刺激ACTH - 皮质醇轴。
