维持性血液透析患者合并退缩人综合征5例临床分析

Shrinking man syndrome complicated with maintenance hemodialysis patients:Clinical analysis of 5 cases

目的分析维持性血液透析患者并发退缩人综合征的临床特点并对其加强预防、积极治疗。方法选择北京朝阳医院血液净化中心规律血液透析合并退缩人综合征患者5例,收集患者一般资料、临床症状、身高变化、生化指标(血清钙、磷、碱性磷酸酶、甲状旁腺素、血红蛋白和血细胞比容)、骨密度、维生素D3冲击量及时间、红细胞生成素用量。结果 5例患者透析龄均在10年以上;身高缩短均>10cm;且都伴有贫血症状、生活自理能力下降、骨关节疼痛、骨畸形,其中1例患者伴有狮面征;5例患者均有高钙血症、骨质疏松、碱性磷酸酶及全段甲状旁腺激素明显升高,且全段甲状旁腺激素持续>2000pg/ml超过3年,经维生素D3冲击治疗无效。结论退缩人综合征是维持性血液透析患者合并难治性继发性甲状旁腺功能亢进症的一种特殊类型,早期监测并规范治疗继发性甲状旁腺功能亢进,可避免退缩人综合征的发生。

Objective To analyze the clinical features of shrinking man syndrome in maintenance hemodialysis（MHD） patients,and to discuss the prevention and treatment of the syndrome.Methods We recruited 5 shrinking man syndrome patients undergoing hemodialysis in Blood Purification Center of Beijing Chao-Yang Hospital,Capital Medical University.Parameters including general status,clinical manifestations,height,biochemical examinations（serum total calcium,inorganic phosphate,alkaline phosphatase,intact parathyroid hormone,Hb and hematocrit）,bone mineral density,dose and time of vitamin D3 pulse therapy and dose of EPO were collected.Results The hemodialysis age of the 5 patients was more than 10 years.Their body height shortened over 10 cm.All of them had anemia,decreased independent living ability,joint and bone pain,and bone deformities.The face appearance of Sagliker syndrome was found in one patient.All of them had hypercalcemia,osteoporosis,higher serum ALP and serum iPTH.Serum iPTH was as high as 2000pg/ml for more than 3 years,and was unresponsive to pulse administration of vitamin D3.Conclusions Shrinking man syndrome is a specific type of refractory secondary hyperparathyroidism found in MHD patients.Early detection and appropriate treatment of secondary hyperparathyroidism are useful for the prevention of shrinking man syndrome in these patients.