Ting Julie Anne, McRae Susanna A, Schwartz Daniel, Barbour Sean J, Riazy Maziar
Division of Nephrology, University of British Columbia, Vancouver, BC, V5Z 1M9, Canada.
Department of Pathology, University of British Columbia, Vancouver, BC, V6Z 1Y6, Canada.
Int J Nephrol Renovasc Dis. 2022 Jun 21;15:207-214. doi: 10.2147/IJNRD.S364890. eCollection 2022.
Lipoprotein glomerulopathy (LPG) is caused by a mutation in the apolipoprotein E gene () gene and is characterized by lipoprotein thrombi in glomerular capillaries. Here, we describe a case of LPG, the first to be reported from Canada and the first case of LPG in North America to be associated with the Tokyo/Maebashi mutation (p.Leu162_Lys164del, traditional nomenclature 142_144del). A 49-year-old man of Chinese descent with a previous diagnosis of dyslipidemia and a new diagnosis of hypertension was found to have proteinuria on routine urinalysis. Renal biopsy showed markedly dilated glomerular capillaries filled with pale staining mesh-like material that stained positive for Oil-Red-O, consistent with lipoprotein thrombi. gene sequencing confirmed the diagnosis of LPG. The patient was treated with fenofibrate and perindopril. His lipid profile normalized and proteinuria dropped to minimal levels. Repeat renal biopsy 2 years after the first showed resolution of lipoprotein thrombi but with rare residual granular densities by electron microscopy consistent with lipoprotein in the subendothelial space, supporting the hypothesis that this subendothelial material contains precursors to lipoprotein thrombi.
脂蛋白肾小球病（LPG）由载脂蛋白E基因（）突变引起，其特征是肾小球毛细血管内存在脂蛋白血栓。在此，我们描述一例脂蛋白肾小球病病例，这是加拿大首例报告的病例，也是北美首例与东京/前桥突变（p.Leu162_Lys164del，传统命名法为142_144del）相关的脂蛋白肾小球病病例。一名49岁华裔男性，既往诊断为血脂异常，新诊断为高血压，在常规尿液分析中发现蛋白尿。肾活检显示肾小球毛细血管明显扩张，充满淡染的网状物质，油红O染色呈阳性，符合脂蛋白血栓。基因测序确诊为脂蛋白肾小球病。该患者接受非诺贝特和培哚普利治疗。他的血脂谱恢复正常，蛋白尿降至最低水平。首次肾活检2年后再次肾活检显示脂蛋白血栓消退，但电子显微镜下罕见残留颗粒密度，与内皮下间隙的脂蛋白一致，支持该内皮下物质含有脂蛋白血栓前体的假说。
