伴多发性内分泌腺瘤病的促肾上腺皮质激素依赖性库欣综合征的临床特点分析

Clinical features of adrenocorticotropic hormone dependent Cushing’s syndrome combined with multiple endocrine neoplasia

目的探讨伴多发性内分泌腺瘤病(MEN)的促肾上腺皮质激素依赖性库欣综合征的临床特点。方法12例伴多发性内分泌腺瘤病的促肾上腺皮质激素依赖性库欣综合征患者,对其一般资料、临床表现、实验室及影像学特征进行回顾性分析。结果12例患者发病原因:库欣病9例,异位促肾上腺皮质激素综合征3例;年龄(44.31±2.82)岁;MEN1型10例,MEN2型2例。症状表现:亚库欣综合征4例,显性库欣综合征8例;全部患者均合并骨量减少及骨质疏松,7例患者合并泌尿系统结石,3例患者合并高血压,2例患者合并糖尿病,1例患者合并低钾血症。实验室检查结果显示,患者血清皮质醇为(453.87±157.94)nmol/L,促肾上腺皮质激素为(8.22±1.05)pmol/L,24 h尿游离皮质醇为(50.42±18.65)μg;1例患者垂体催乳素升高,9例患者甲状旁腺激素升高,7例患者钙离子升高。通过影像学检查结果可知,8例患者为垂体微腺瘤,1例患者为垂体大腺瘤;5例患者为肾上腺腺瘤,2例患者为肾上腺增粗,1例患者为肾上腺嗜铬细胞瘤,1例患者为腹主动脉旁副神经节瘤。结论伴多发性内分泌腺瘤病的促肾上腺皮质激素依赖性库欣综合征患者的症状具有一定的复杂性,对其主要表现进行分析,包括骨量减少与骨质疏松,其病因主要为垂体微腺瘤;MEN1型在临床较为常见。

Objective To discuss the clinical features of adrenocorticotropic hormone dependent Cushing's syndrome combined with multiple endocrine neoplasia(MEN).Methods For 12 patients with adrenocorticotropic hormone dependent Cushing's syndrome combined with multiple endocrine neoplasia,their general data,clinical manifestations,laboratory examination results and imaging features were analyzed retrospectively.Results Causes of 12 patients,9 cases of Cushing's disease and 3 cases of ectopic adrenocorticotropic hormone syndrome;the age range was(44.31±2.82)years;10 cases were MEN1,2 cases were MEN2.Symptoms:4 cases of sub-Cushing syndrome and 8 cases of overt Cushing syndrome;all patients had combined bone mass reduction and osteoporosis,7 patients had combined urinary calculi,3 patients had combined hypertension,2 patients had combined diabetes mellitus,and 1 patient had combined hypokalemia.The laboratory examination results showed that patients had the serum cortisol of(453.87±157.94)nmol/L,the adrenocorticotropic hormone of(8.22±1.05)pmol/L,and the 24 h urinary free cortisol of(50.42±18.65)μg;1 patient had increased pituitary prolactin,9 patients had increased parathyroid hormone,and 7 patients had increased calcium ion.The results of imaging examination showed that 8 patients had pituitary microadenomas and 1 patient had pituitary macroadenoma;5 patients had adrenal adenomas,2 patients had adrenal thickening,1 patient had adrenal pheochromocytoma,and 1 patient had paraaortic paraganglioma.Conclusion The symptoms of adrenocorticotropic hormone dependent Cushing's syndrome combined with multiple endocrine neoplasia are complex.The main manifestations,including osteopenia and osteoporosis,are analyzed.The main etiology is pituitary microadenoma;MEN1 is common in clinic.