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成人起病型范科尼综合征:中草药肾病早期广泛的肾小管间质病变和肾小球病变

Adult onset Fanconi syndrome: extensive tubulo-interstitial lesions and glomerulopathy in the early stage of Chinese herbs nephropathy.

作者信息

Kazama Itsuro, Matsubara Mitsunobu, Michimata Mari, Suzuki Michiko, Hatano Ryo, Sato Hiroshi, Ito Sadayoshi

机构信息

Division of Molecular Medicine, Tohoku University Graduate School of Medicine, 2-1 Seiryo-cho, Aoba-ku, Sendai 980-8575, Japan.

出版信息

Clin Exp Nephrol. 2004 Sep;8(3):283-7. doi: 10.1007/s10157-004-0296-9.

DOI:10.1007/s10157-004-0296-9
PMID:15480910
Abstract

A 33-year-old woman was transferred to our hospital with a 5-month history of taking Chinese herbal medicine containing aristolochic acid. She presented with metabolic acidosis, severe anemia, hypophosphatemia and uric aciduria, and generalized aminoaciduria and glycosuria, features which were consistent with the clinical manifestations of Fanconi syndrome. Renal biopsy was performed when her plasma creatinine was 0.7 mg/dl and creatinine clearance was 46 ml/min per 1.73 m(2). The renal specimen showed extensive interstitial edema with focal fibrosis, tubular atrophy, and focal glomerulopathy, which suggested the presence of glomerular endothelial damage or glomerular ischemia. Although steroid therapy ameliorated the plasma electrolyte levels, renal failure progressed, and hemodialysis therapy was initiated approximately 18 months after the time of renal biopsy. This patient demonstrated the early renal lesions of Chinese herbs nephropathy in association with various clinical manifestations. The characteristic glomerulopathy found in the present patient is considered to be an additional renal lesion, leading to the renal failure.

摘要

一名33岁女性因服用含马兜铃酸的中草药5个月后被转至我院。她出现代谢性酸中毒、严重贫血、低磷血症和尿酸尿症,以及全身性氨基酸尿症和糖尿症,这些特征与范科尼综合征的临床表现相符。当她的血浆肌酐为0.7mg/dl且肌酐清除率为每1.73m² 46ml/min时进行了肾活检。肾脏标本显示广泛的间质水肿伴局灶性纤维化、肾小管萎缩和局灶性肾小球病变,提示存在肾小球内皮损伤或肾小球缺血。尽管类固醇治疗改善了血浆电解质水平,但肾衰竭仍在进展,肾活检后约18个月开始进行血液透析治疗。该患者表现出与各种临床表现相关的中草药肾病早期肾脏病变。本患者中发现的特征性肾小球病变被认为是导致肾衰竭的另一种肾脏病变。

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