马兜铃酸肾病的临床与病理表现

The clinical and pathological manifestations of aristolochic acid nephropathy-the report of 58 cases

目的 总结马兜铃酸肾病病人的临床及病理表现 ,进行分型 ,以提高对此病的认识。方法 对经中日友好医院治疗的 5 8例马兜铃酸肾病病人进行了肾功能、电解质、尿酸化功能检查 ,并做肾穿刺进行了光镜、免疫荧光及电镜检查。并对部分病人所服药物的马兜铃酸成分及含量进行检测 (薄层析扫描法 )。结果 根据临床 病理表现特点 ,马兜铃酸肾病可分为 3型 :(1)急性型 (4例 ) :病理呈急性肾小管坏死 ,临床出现急性肾衰竭 ;(2 )肾小管功能障碍型 (7例 ) :病理呈肾小管变性及萎缩 ,临床出现肾小管酸中毒和 /或Fanconi综合征 ;(3)慢性型 (4 7例 ) :病理呈寡细胞性肾间质纤维化 ,临床出现慢性进行性肾衰竭 ,部分病例肾损害进展迅速。用类固醇激素对本病部分病人进行治疗 ,前二型个别病例病情好转。药物分析已证实马兜铃酸存在。结论 含马兜铃酸成分的中草药确可引起肾小管 间质肾病 ,此病至少可分为 3种临床 病理类型 ,各型预后不同。

Objective To realize and classify the aristolochic acid nephropathy (AAN) according to its clinical and pathological manifestations. Methods Fifty eight cases in our Division during October 1998 to August 2001 were reviewed, and their clinical, laboratory and pathological manifestations as well as the response of therapy were analyzed. The aristolochic acid (AA) component in some Chinese traditional drugs taken by our patients was detected with thin layer chromatography (TLC) scan. Results AAN might be divided the following three types: (1) acute AAN ( n =4): acute tubular necrosis and acute renal failure were its pathological and clinical characters, respectively. (2)tubular dysfunctional AAN ( n =7): tubular degradation with atrophy, and renal tubular acidosis and/ or Fanconi syndrome were its main pathological and clinical manifestations, respectively. (3) chronic AAN ( n =47): renal interstitial fibrosis with few infiltrated mononuclear cells, and chronically progressive renal failure were its dominant pathological and clinical findings, respectively. Steroid therapy was tried to treat some patients with AAN, and a few patients in the first two types obtained some good effects. AA component was demonstrated by the TLC scan in the drugs taken by our patients. Conclusion Chinese traditional drugs containing AA are able to cause a special tubulointerstitial nephropathy which may be classified three types with different outcome. The definite effects of steroid on AAN still need to be proved by further studies.