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马兜铃酸肾病的临床和病理谱

Clinical and pathological spectrums of aristolochic acid nephropathy.

作者信息

Chen Dongmei, Tang Zheng, Luo Chunlei, Chen Huiping, Liu Zhihong

机构信息

Research Institute of Nephrology, Jinling Hospital, Nanjing University School of Medicine, Nanjing, PR China.

出版信息

Clin Nephrol. 2012 Jul;78(1):54-60. doi: 10.5414/cn107414.

DOI:10.5414/cn107414
PMID:22732338
Abstract

AIM

To study the clinical and pathological characteristics of aristolochic acid nephropathy (AAN).

METHODS

86 patients with AAN during 2001 and 2009 in our department were recruited in this retrospective study. The clinical and pathological features were analyzed.

RESULTS

There were 47 males and 39 females, aging from 12 to 69 years old. Abnormal urine analysis and gastro-intestinal diseases were two main underlying causes for patients taking aristolochic acid (AA) containing drugs. All patients suffered from renal function impairment. 19 patients (22.0%) presented with acute kidney injury (AKI), while 67 patients (78%) presented as chronic cases. Among them, 31 patients (36.0%) lacked symptoms, 30 patients (34.8%) were accompanied with hypertension, and 26 patients (30.2%) presented with gastrointestinal symptoms. Laboratory examination revealed elevated urine retinol-binding protein (RBP) (90.7%) and urine N-acetyl-β-glucosaminidase (NAG) (80.2%). Anemia and glucosuria accounted for 64.0% and 58.1%, respectively. Renal biopsy showed prominent tubular brush border ablation (84.2%) in acute cases, while obvious tubular basement membrane (TBM) thickening (81.4%) and interstitial fibrosis were present in chronic cases. During the follow- up, 11 (57.9%) acute cases gained renal function recovery. They had lower urine RBP level and lower incidence of hypokalemia than the non-recovery acute cases. In the chronic group, 27 patients (40.2%) progressed to endstage renal disease (ESRD), with 11 dialysis and 5 renal transplantation cases.

CONCLUSION

AAN patients usually suffered from renal impairment with an associated history of taking AA containing drugs. Proximal renal tubular dysfunction and structure destroying would be the main positive findings in laboratory tests and renal biopsy. Urine RBP and hypokalemia might determine the outcome of acute AAN patients.

摘要

目的

研究马兜铃酸肾病(AAN)的临床及病理特征。

方法

本回顾性研究纳入了2001年至2009年期间在我科就诊的86例AAN患者,分析其临床及病理特征。

结果

患者中男性47例,女性39例,年龄12至69岁。尿常规异常和胃肠道疾病是患者服用含马兜铃酸(AA)药物的两个主要潜在原因。所有患者均有肾功能损害。19例(22.0%)表现为急性肾损伤(AKI),67例(78%)为慢性病例。其中,31例(36.0%)无症状,30例(34.8%)伴有高血压,26例(30.2%)有胃肠道症状。实验室检查显示尿视黄醇结合蛋白(RBP)升高(90.7%)和尿N-乙酰-β-葡萄糖苷酶(NAG)升高(80.2%)。贫血和糖尿分别占64.0%和58.1%。肾活检显示急性病例中肾小管刷状缘显著缺失(84.2%),而慢性病例中可见明显的肾小管基底膜(TBM)增厚(81.4%)和间质纤维化。随访期间,11例(57.9%)急性病例肾功能恢复。与未恢复的急性病例相比,他们的尿RBP水平较低,低钾血症发生率较低。在慢性组中,27例(40.2%)进展至终末期肾病(ESRD),其中11例行透析治疗,5例行肾移植。

结论

AAN患者通常有肾功能损害,并有服用含AA药物的相关病史。近端肾小管功能障碍和结构破坏是实验室检查和肾活检的主要阳性发现。尿RBP和低钾血症可能决定急性AAN患者的预后。

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