摘要目的:对POEMS综合征患者人群内分泌代谢异常的情况和临床特点进行分析和总结。方法:对2000年1月至2020年1月中国人民解放军总医院第一医学中心临床确诊的136例POEMS综合征患者的病例资料进行系统回顾性分析。结果:93.38%(127/136)的患者存在内分泌代谢异常，病变累及性腺、甲状腺、肾上腺、胰岛等多个内分泌器官。最常见的是性腺功能减退症(98/136，72.06%)，其次是甲状腺功能减退症(83/136，61.03%)、低钙血症(50/136，36.76%)、高催乳素血症(47/136，34.56%)、糖代谢异常(41/136，30.15%)和肾上腺皮质功能减退症(41/136，30.15%)。单个内分泌器官受累占12.60%(16/127)，多个内分泌器官受累占87.40%(111/127)。多个内分泌器官受累患者中，2、3、4、5、6种内分泌器官受累的情况分别占29.92%(38/127)、30.71%(39/127)、17.32%(22/127)、7.09%(9/127)和2.36%(3/127)。POEMS综合征患者内分泌代谢异常复杂多变，发病至就诊的平均时间为412 d(198～1 055 d)，住院确诊的平均时间为8 d(4～15 d)。结论:POEMS综合征患者内分泌代谢异常表现复杂多样，容易造成误诊、误治。因此，对临床确诊的POEMS综合征患者应当进行详尽的内分泌代谢异常评估并且适时进行激素补充替代治疗。

abstractsObjective:To analyze and summarize the prevalence and clinical characteristics of endocrine-metabolic abnormalities in a large cohort with POEMS syndrome, and to improve the understanding of endocrine-metabolic abnormalities in this special disease.Methods:The retrospective review was performed in patients with a diagnosis of POEMS syndrome at the First Medical Center of Chinese PLA General Hospital between January 2000 and January 2020. The clinical data about endocrine-metabolic abnormalities were extracted from their medical records.Results:The prevalence of endocrine-metabolic abnormalities was 93.38% (127/136) including gonads, thyroid, adrenal, islets and other endocrine organs involved. Hypogonadism was the most common endocrine-metabolic abnormality (98/136, 72.06%), followed by hypothyroidism (83/136, 61.03%), hypocalcemia (50/136, 36.76%), hyperprolactinemia (47/136, 34.56%), abnormal glucose metabolism (41/136, 30.15%), and adrenal insufficiency (41/136, 30.15%). The prevalence of single endocrinopathy and multiple endocrinopathies were 12.60%(16/127) and 87.40%(111/127) respectively. In patients with multiple endocrinopathies, the percentage of 2, 3, 4, 5 and 6 kinds of endocrine axes involved were 29.92%(38/127), 30.71%(39/127), 17.32%(22/127), 7.09%(9/127), and 2.36%(3/127), respectively. The endocrine-metabolic abnormalities in patients with POEMS syndrome were complex and variable. The median time from symptoms onset to confirmed diagnosis of POEMS was 412 d (198-1 055 d), and the median time for clinically definite was 8 d (4-15 d).Conclusion:The endocrine-metabolic abnormality is variable in POEMS syndrome, which can cause misdiagnosis and incorrect therapy. For patients with confirmed diagnosis of POEMS syndromes, a thorough endocrine evaluation should be performed and hormone supplement should be administrated timely.