马兜铃酸肾病临床及病理改变多样性

目的：探讨有或无肾炎病史马兜铃酸肾病患者发病后临床表现及肾脏病理改变。方法：11例表现为急性肾功能衰竭的马兜铃酸肾病患者，按发病前肾功能正常时有或无肾炎病史分为有肾炎史组5例和无肾炎史组6例。比较2组发病时临床表现、实验室检查指标及肾组织活检病理改变。结果：有肾炎史组表现为少尿型急性肾功能衰竭（40％），同时伴有大量蛋白尿（60％）、镜下血尿（40％）及高血压（40%），尿糖定量、尿N-乙酰-β-D 葡萄糖苷酶、尿视黄醇结合蛋白及尿氨基酸均比正常明显升高，但无肾炎病史组升高更明显。肾脏病理改变同时伴有不同性质和程度的肾小球痛变及肾间质炎性细胞浸润。无肾炎痛史组表现为非少尿型急性肾功能衰竭（100％）．镜下血尿（16％）,无或仅少量蛋白尿，无高血压。尿糖定量、尿N-乙酰-B-D葡萄糖苷酶、尿视黄醇结合蛋白及尿氨基酸升高更为明显；肾脏病理改变呈急性肾小管坏死。结论：马兜铃酸肾病患者发病前有或无肾炎病史，发病后临床表现及肾脏病理改变明显不同。无肾炎病史者肾小管功能损伤明显．肾小管功能相关检查可协助诊断，尿糖阳性，是提示临床诊断的一个重要线索。

Clinical and pathological study on aristolochic acid nephropathy

Objective To compare the clinical and renal histological characteristics between patients with and without nephritis. Methods Eleven patients with aristolochic acid nephropathy manifested with acute renal failure were divided into group A （n=5, with the history of nephritis） and group B （n=6, without the history of nephritis）. The clinical manifestations, lab examinations and renal biopsy histology were compared between two groups. Results Group A was characterized by oliguria acute renal failures（40%）, massive proteinuria（60%）, microscopic hematuria（40%）, hypertension （40%）, and high rates of glucosuria and aminoaciduria and high levels of NAG and RBP, which were not as high as those in group B. Renal biopsy showed acute tubular necrosis with different kinds and different degrees of glomerulonephritis and tubuilointerstitial inflammation. Group B was presented with acute renal failure（100%） , low rate of microscopic hematuria （16%）, absence of hypertension, and no or less proteinuria. The patients in group B showed much higher levels of aminoaciduria, NAG and RBP as compared with group A. Renal biopsy showed acute tubular necrosis. Conclusion There are some significant differences in clinical and pathological changes between patients with and without nephritis prior to aristolochic acid nephropathy. The patients without nephritis show obvious impaired renal tubular. Renal tubular determination can help to make diagnosis. Glucosuria is an important marker for aristolochic acid nephropathy.