【摘要】 目的 通过报道1例以皮肤紫癜和肾损害为主要临床表现的免疫球蛋白轻链κ亚型淀粉样变性病(immunoglobulin light-chain kappa subtypes amyloidosis)的病例,并结合文献系统复习淀粉样变的分型、病理、临床表现、诊断与鉴别诊断及治疗原则,以期提高对该病的认识和重视,避免误漏诊,早期获得正确合理的治疗。方法 患者1例,多处反复皮肤紫癜及肾病综合征(nephrotic syndrome,NS)、镜下血尿、水肿等肾脏受累为主的临床表现,曾为多家医院诊断为“过敏性紫癜性肾炎”。入我院经肾穿刺病理活检。病理诊断:AL-κ亚型肾淀粉样变性病。根据以上临床资料,总结复习相关文献。结果 患者经对症及口服激素治疗,病情稳定。结论 肾淀粉样变性病是中老年继发性肾病综合征常见的病因之一,并非罕见,应予高度重视。凡具有大量蛋白尿或NS的中老年患者,特别是伴皮肤紫癜、低血压、肝大、舌肥大、心脏受累等应进一步行血、尿免疫固定电泳检查,对可疑患者应尽早进行肾活检,争取早期明确诊断、以期早期合理治疗,提高生存质量和生存时间。更多还原

【Abstract】 Objective To report one case of renal damage and skin purpura as the main clinical manifestations of AL-kappa subtype amyloidosis.To systematically review classification,pathology,clinical manifestation-,diagnosis and differential diagnosis and treatment principle of amyloidosis in order to improve the understanding of the disease and to avoid the misdiagnosis and missed diagnosis,and to get the correct and reasonable treatment early.Methods A 53-year old man presented skin purpura and nephrotic syndrome(NS),the microscopic hematuria,edema and kidney involvements as clinical manifestations,accompanied by significant weight loss,low blood pressure,tongue body hypertrophy,hepatomegaly and cardiac involvement.The kidney puncture biopsy pathological examination revealed the diagnosis of AL-kappa subtype renal amyloidosis.According to the above clinical data we summarized literatures.Results The patient got a stable state through the symptomatic treatment.Conclusions Renal amyloidosis is one of common causes of the middle-aged and old secondary nephrotic syndrome,which should be highly valued.Whoever has the massive proteinuria or nephrotic syndrome in elderly patients,especially whom with skin purpura,low blood pressure,hepatomegaly,tongue hypertrophy and cardiac involvement,further test of blood and urine immune fixation electrophoresis is necessary.For the patients suspicious of renal amyloidosis,renal biopsy is performed as early as possibly.Early diagnosis and reasonable treatment early can improve life quality and survival time.更多还原