摘要
目的探讨马兜铃酸肾病(aristolochic acid nephropathy,AAN)合并范可尼综合征(Fanconi syndrome,FS)患者的临床表现和生化特点。方法回顾性分析1998年11月至2010年9月我院肾内科诊断AAN合并FS患者38例,观察患者临床表现、实验室检查指标和疗效。结果 38例患者中完全型FS12例,不完全型FS26例,主要表现为近端肾小管酸中毒伴物质转运障碍,包括明显低磷、低尿酸血症、肾性糖尿、氨基酸尿和低分子量蛋白尿。临床表现以乏力、恶心、夜尿增多、腰背痛为主要,部分患者有明显多饮多尿、骨痛、手足抽搐及泌尿系感染。4例患者接受肾活检,结果显示有明显小管-间质病变。糖皮质激素治疗可延缓肾功能损害的进展,对FS有一定疗效。结论马兜铃酸肾病可合并FS,需完善相关检查明确诊断,糖皮质激素对AAN继发的FS有一定疗效。
Objective To analyze the clinical features of Fanconi syndrome in patients with aristolochic acid nephropathy（AAN）. Methods All 38 patients with Fanconi syndrome in AAN who were hospitalized during 1998 to 2010 were enrolled into this study. Clinical manifestation,laboratory parameters and prognosis were observed. Results The patients were characterized by proximal renal tubular acidosis and multiple renal tubular transport dysfunction,including hypophosphatemia,hypouricemia,renal glycosuria,aminoaciduria,low-molecular-weight proteinuria. The clinical manifestations commonly presented with weakness,nausea,nocturia,back pain,some patients had obvious polydipsia,polyuria,ostalgia,tetania and urinary tract infection. Renal pathohistological studies in 4 patients showed evident renal tubulointerstitial changes. Steroid therapy might delay the progression of renal failure in AAN patients,and it had a certain curative effect on Fanconi syndrome in aristolochic acid nephropathy. Conclusions AAN may be associated with Fanconi syndrome. Further examination is needed to make a definite diagnosis. Steroids therapy has benefit effect on Fanconi syndrome in aristolochic acid nephropathy.
出处
《北京医学》
CAS
2011年第2期144-148,共5页
Beijing Medical Journal
关键词
马兜铃酸肾病
范可尼综合征
Aristolochic acid nephropathy Fanconi syndrome
