Clinical and pathological spectrums of aristolochic acid nephropathy

Clinical and pathological spectrums of aristolochic acid nephropathy
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DOI:
10.5414/cn107414
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发表时间:
2012-07-01
影响因子:
1.1
通讯作者:
Liu, Zhihong
Liu, Zhihong
中科院分区:
医学4区
文献类型:
--
作者:
Chen, Dongmei;Tang, Zheng;Liu, Zhihong

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目的:研究马兜铃酸肾病(AAN)的临床和病理特征。方法:回顾性研究我科2001年至2009年86例AAN患者。分析其临床和病理特征。结果:男47例,女39例,年龄12~69岁。尿液分析异常和胃肠道疾病是患者服用含马兜铃酸(AA)药物的两个主要原因。所有患者均患有肾功能损害,其中19名患者(22.0%)表现为急性肾损伤(AKI),67名患者(78%)表现为慢性肾损伤。其中,31例(36.0%)无症状,30例(34.8%)伴有高血压,26例(30.2%)有消化道症状。实验室检查显示尿液视黄醇结合蛋白(RBP)(90.7%)和尿液N-乙酰-β-氨基葡萄糖苷酶(NAG)(80.2%)升高。贫血和糖尿分别占64.0%和58.1%。肾活检显示急性病例肾小管刷状缘明显消融(84.2%),慢性病例肾小管基底膜(TBM)明显增厚(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。慢性组中有27例(40.2%)进展为终末期肾病(ESRD),其中11例进行透析,5例进行肾移植。结论:AAN 患者通常患有肾功能损害,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将是实验室检查和肾活检的主要阳性结果。尿 RBP 和低钾血症可能决定急性 AAN 患者的预后。
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-beta-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 end-stage 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.