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薄基底膜肾病——肾小球性血尿的常见病因。

Thin-membrane nephropathy--a common cause of glomerular haematuria.

作者信息

Perry G J, George C R, Field M J, Collett P V, Kalowski S, Wyndham R N, Newland R C, Lin B P, Kneale K L, Lawrence J R

机构信息

Repatriation General Hospital Concord, NSW.

出版信息

Med J Aust. 1989;151(11-12):638-42. doi: 10.5694/j.1326-5377.1989.tb139637.x.

DOI:10.5694/j.1326-5377.1989.tb139637.x
PMID:2593909
Abstract

Thin-membrane nephropathy recently has been described as a cause of glomerular haematuria. The prognosis of the condition is unclear but it generally is considered to be benign. In a series of 92 patients with glomerular haematuria, thin-membrane nephropathy was found to be a common cause, occurring in 26 (28%) patients. Sixteen patients were women. The mean age was 42 years. Four patients had a family history of renal disease or haematuria and no patient was deaf. Haematuria had been present from six days to 30 years. Loin pain occurred in 31% of patients. Hypertension was not a feature and mild renal impairment was present in one case only, while a further three cases showed proteinuria at a level of greater than 500 mg of protein per day. Glomerular basement membranes in patients with thin-membrane nephropathy gave a mean (+/- standard deviation) width of 319 + 37 nm which was significantly (P less than 0.002) less than the control value of 394 +/- 61 nm. On the basis of clinical features and serological parameters, thin-membrane nephropathy could not be separated from other renal causes of haematuria but required careful electronmicroscopic examination of renal biopsy material to establish the diagnosis. Limited follow-up has confirmed the good prognosis of the condition.

摘要

薄基底膜肾病最近被描述为肾小球性血尿的一个病因。该病的预后尚不清楚,但一般认为是良性的。在一组92例肾小球性血尿患者中,薄基底膜肾病是常见病因,26例(28%)患者患有该病。其中16例为女性。平均年龄42岁。4例患者有肾脏疾病或血尿家族史,无患者耳聋。血尿出现时间为6天至30年。31%的患者出现腰痛。无高血压表现,仅1例有轻度肾功能损害,另有3例蛋白尿水平超过每日500毫克。薄基底膜肾病患者的肾小球基底膜平均(±标准差)宽度为319 + 37纳米,显著(P < 0.002)小于对照组的394 ± 61纳米。基于临床特征和血清学参数,薄基底膜肾病无法与其他血尿的肾脏病因相区分,但需要对肾活检材料进行仔细的电子显微镜检查以确诊。有限的随访已证实该病预后良好。

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