Tiebosch A T, Frederik P M, van Breda Vriesman P J, Mooy J M, van Rie H, van de Wiel T W, Wolters J, Zeppenfeldt E
Department of Immunology, University of Limburg, Maastricht, the Netherlands.
N Engl J Med. 1989 Jan 5;320(1):14-8. doi: 10.1056/NEJM198901053200103.
Thin-basement-membrane nephropathy, also called benign recurrent hematuria, is characterized by diffuse thinning of the glomerular basement membrane and by hematuria. To determine the incidence of thin-basement-membrane nephropathy among patients with idiopathic hematuria, we conducted a prospective study in the nephrology units of three large hospitals in the Netherlands. Eighty normotensive adults without azotemia underwent renal biopsy because of recurrent macroscopic hematuria (n = 26) or persistent microscopic hematuria (n = 54). Idiopathic IgA nephropathy was found in 27 of the 80 patients. Light microscopical examination showed that 42 patients had normal renal tissue. The remaining 11 patients had mesangioproliferative glomerulonephritis (n = 5), interstitial nephritis (n = 3), or focal global glomerulosclerosis (n = 3). Tissue from the 42 patients whose renal biopsy specimens were normal when examined with light microscopy was analyzed morphometrically with electron microscopy to determine the thickness of the glomerular basement membrane. Two subsets of patients were identified by this analysis. In 18, thin-basement-membrane nephropathy was found (mean basement-membrane thickness [+/- SE], 191 +/- 28 nm; normal, 350 +/- 43 nm); all but one of these 18 patients had microscopic hematuria, which persisted during follow-up (median duration, 50 months). (Of the 54 patients who presented with microscopic hematuria, 17 [31 percent] had thin-basement-membrane nephropathy.) The thickness of the glomerular basement membrane was normal in the other 24 patients (361 +/- 69 nm); during follow-up, hematuria disappeared in all 13 of these patients who had macroscopic hematuria, and hematuria resolved in 5 of the 11 patients who had microscopic hematuria. We conclude that in patients with persistent microscopic hematuria, the incidence of thin-basement-membrane nephropathy is similar to that of idiopathic IgA nephropathy. Morphometric analysis of the thickness of the glomerular basement membrane should be included in the workup of adults with persistent microscopic hematuria that is not of urologic origin.
薄基底膜肾病,也称为良性复发性血尿,其特征是肾小球基底膜弥漫性变薄和血尿。为了确定特发性血尿患者中薄基底膜肾病的发病率,我们在荷兰的三家大型医院的肾病科进行了一项前瞻性研究。80名无氮质血症的血压正常成年人因复发性肉眼血尿(n = 26)或持续性镜下血尿(n = 54)接受了肾活检。80例患者中有27例发现特发性IgA肾病。光镜检查显示42例患者肾组织正常。其余11例患者患有系膜增生性肾小球肾炎(n = 5)、间质性肾炎(n = 3)或局灶性球性肾小球硬化(n = 3)。对42例光镜检查肾活检标本正常的患者的组织进行电子显微镜形态计量分析,以确定肾小球基底膜的厚度。通过该分析确定了两组患者。18例发现薄基底膜肾病(平均基底膜厚度[±SE],191±28 nm;正常为350±43 nm);这18例患者中除1例外均有镜下血尿,且在随访期间持续存在(中位持续时间50个月)。(在54例表现为镜下血尿的患者中,17例[31%]患有薄基底膜肾病。)另外24例患者的肾小球基底膜厚度正常(361±69 nm);在随访期间,所有13例有肉眼血尿的患者血尿消失,11例有镜下血尿的患者中有5例血尿消退。我们得出结论,在持续性镜下血尿患者中,薄基底膜肾病的发病率与特发性IgA肾病相似。对于非泌尿系统来源的持续性镜下血尿的成年人,肾小球基底膜厚度的形态计量分析应纳入检查过程中。