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作 者:刘江红[1] 王芳[2] 李忠音 笪宇威[1] 张新卿[1]
机构地区:[1]首都医科大学宣武医院神经内科,100053 [2]卫生部北京医院免疫科,100730 [3]四川省简阳市人民医院神经内科,641400
出 处:《中国神经免疫学和神经病学杂志》2011年第5期336-338,共3页Chinese Journal of Neuroimmunology and Neurology
摘 要:目的分析POEMS综合征患者的临床特点,以提高临床医师对其认识。方法回顾性分析作者医院收治的20例POEMS综合征患者临床表现、实验室检查及治疗方法等资料。结果 20例患者都有多发性周围神经病、单克隆丙种球蛋白病和内分泌病表现,其他主要临床特征有脏器肿大(95%)、皮肤改变(80%)、血管外容量过度负荷(75%,包括水肿、多浆膜腔积液)、视乳头水肿(45%)。治疗以静脉输入丙种球蛋白(IVIG)及糖皮质激素为主,但总体疗效欠佳。结论 POEMS综合征是一种少见的多系统损害性疾病,其临床表现复杂多样。IVIG及糖皮质激素效果不明显。Objective To analyze the clinical features of POEMS syndrome and to improve the clinicians~ recognition of the syndrome. Methods The clinical manifestations, laboratory examinations and treatments of 20 patients with POEMS syndrome were analyzed retrospectively. Results All the 20 cases showed polyneuropathy, monoclonal gammopathy and endocrinopathy. The other clinical characteristics were organomegaly (95%), skin changes (80%), extravascular volume overload (75%, including edema, serous cavity hydrops), papilledema (45%). The patients were mainly treated by intravenous immunoglobulin and/or corticosteroid, however, no apparent curative effects were observed. Conclusions POEMS syndrome is a rare multi-system disorder and presents with rather complicated manifestations. IVIG and/or corticosteroid do not show obvious therapeutic effects
分 类 号:R747.9[医药卫生—神经病学与精神病学]
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