23例POEMS综合征临床特点分析

发布时间：2019-03-14 09:45

【摘要】：目的:了解POEMS综合征患者合并的内分泌异常的特点,为在合并内分泌系统异常的患者中识别出POEMS综合征的高危人群提供依据。方法:以"POEMS综合征"为词检索北京大学人民医院病案数据库,共纳入23例患者。使用Epidata 3.0软件进行数据录入,SPSS 19.0统计软件进行数据分析。结果:入组患者平均年龄47岁,男女比例为1.88∶1,首发症状以多发神经病变(56.5%)最为多见,从首发症状至确诊的中位时间为30个月。就诊于神经内科的患者例数最多,诊断的中位时间最短。患者各项临床表现的发生率分别为多发神经病变100.0%、器官肿大82.6%、内分泌异常95.7%、浆细胞克隆性增生52.2%、皮肤改变91.3%,临床体征有视乳头水肿45.5%、血管外负荷增加43.5%、血小板升高43.5%、Castleman病17.4%。在内分泌系统异常中,泌乳素水平异常的发生率最高,为60.0%,其次分别为甲状腺轴及肾上腺轴功能异常。在内分泌系统受累的患者中,2个靶腺受累的发生率最高,占36.5%。结论:内分泌系统异常是POEMS综合征常见的临床表现,对于甲状腺、肾上腺、性腺、甲状旁腺功能异常者以及泌乳素水平或糖耐量异常者,尤其是累及2个或2个以上的内分泌系统功能异常者,应提高警惕,关注多发神经病变的症状和体征,减少POEMS综合征的误诊、漏诊,同时,对POEMS综合征患者需全面评估各个内分泌系统的异常,以使患者得以及时的诊断和治疗。
[Abstract]:Aim: to investigate the characteristics of endocrine abnormalities in patients with POEMS syndrome and to provide evidence for identifying the high risk population of POEMS syndrome in patients with endocrine system abnormalities. Methods: "POEMS syndrome" was used to search the medical record database of Peking University people's Hospital, and 23 patients were included in the database. Epidata 3.0 software was used for data entry and SPSS 19.0 software was used for data analysis. Results: the average age of the patients in the group was 47 years, the ratio of male and female was 1.88%, the most common first symptom was multiple neuropathy (56.5%), and the median time from the first symptom to the diagnosis was 30 months. The number of patients visited the neurology department was the most and the median diagnosis time was the shortest. The incidence of multiple neuropathy, organ enlargement, endocrine abnormality, plasma cell clonality and skin change were 100.0%, 82.6%, 95.7%, 52.2% and 91.3%, respectively. The clinical signs were papillary edema (45.5%), extravascular load (43.5%), platelet (43.5%) and Castleman's disease (17.4%). Among the endocrine system abnormalities, the incidence of abnormal prolactin level was the highest (60.0%), followed by thyroid axis and adrenal axis dysfunction. Among the patients with endocrine system involvement, the incidence of two target glands was the highest, accounting for 36.5%. Conclusion: abnormal endocrine system is a common clinical manifestation of POEMS syndrome. For those with abnormal thyroid, adrenal, gonadal, parathyroid function, and abnormal prolactin level or glucose tolerance, Especially for those with two or more abnormal endocrine system function, we should pay more attention to the symptoms and signs of multiple neuropathy, reduce the misdiagnosis and missed diagnosis of POEMS syndrome, and at the same time, we should pay more attention to the symptoms and signs of multiple neuropathy. In order to diagnose and treat POEMS syndrome, it is necessary to evaluate the abnormalities of each endocrine system in an all-round way.
【作者单位】：北京大学人民医院内分泌科;
【分类号】：R597

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