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目的探讨系统性淀粉样变性导致的继发性心肾综合征的诊断与治疗。方法选择系统性淀粉样变性导致继发性心肾综合征患者12例,回顾性分析患者的临床、心电图、心脏超声、肾脏病理等表现。结果 12例患者均以“胸闷气短”为首发症状,均有低血压倾向,伴肾功能不全,舌体肥厚。心电图肢导联为低电压,心脏超声左室壁厚度为(18.2±3.2)mm。肾脏活检HE染色光镜下均见系膜区有粉红色淀粉样物质沉积,电镜观察下可见无分支皱褶结构排列,均为来源于免疫球蛋白轻链λ。结论系统性淀粉样变同时累及心脏、肾脏时,掌握其特异性表现,可以早期明确诊断,减少误诊、漏诊。
Objective To investigate the diagnosis and treatment of secondary cardio-renal syndrome caused by systemic amyloidosis. Methods Twelve patients with SS caused by systemic amyloidosis were selected. Clinical, electrocardiogram, echocardiography and renal pathology were retrospectively analyzed. Results All the 12 patients had “chest tightness, shortness of breath and shortness of breath” as the first symptom. All of them had hypotensive tendency, with renal insufficiency and tongue hypertrophy. ECG lead for low voltage, left ventricular wall thickness of cardiac ultrasound (18.2 ± 3.2) mm. Kidney biopsy under HE staining, there was a pink amyloid deposition in the mesangial area, electron microscopy showed that there is no branching fold structure, are derived from immunoglobulin light chain λ. Conclusions Systematic amyloidosis affects heart and kidney at the same time, and its specific expression can be mastered to diagnose early and reduce misdiagnosis and missed diagnosis.