Departmental Section of Native Kidney Histopathology, Azienda Ospedaliero Universitaria Pisana, Pisa - Italy.
J Nephrol. 2011 May-Jun;24(3):381-5. doi: 10.5301/JN.2011.7772.
It is well known that the abnormal accumulation of lipids can occur in kidneys of patients affected by some metabolic disorders due either to inherited enzymatic deficiency or to an acquired lipid alteration as in nephrotic syndrome. Lipoprotein glomerulopathy (LG), briefly described in a patient of Koitabashi in 1987 in a review on renal lipidoses authored by Faraggiana and Churg, represents an emerging novel storage renal disease. This rare and unique nephropathy is characterized by the presence of lipoprotein thrombi in dilated glomerular capillary lumina associated with type III hyperlipoproteinemia, and high serum levels of apolipoprotein E (apo E). Several specific studies conducted by Saito et al on his patients from 1989, revealed that it was an hereditary disease with an autosomal recessive pattern that predominantly affects patients of Asian ancestry, mainly the Japanese population, but which very seldom, can also occur in white subjects. The disorder is probably due to an inherited altered lipid metabolism due to a mutation of the apo E genetic code. Clinically, LG is characterized by proteinuria generally associated with nephrotic syndrome and progressive renal insufficiency. We describe the cases of 2 Italian adult white male patients affected by LG, admitted in our nephrology unit in 2004 and in 2009, respectively.
众所周知，由于遗传酶缺乏或获得性脂质改变，如肾病综合征，一些代谢紊乱的患者的肾脏可能会出现脂质异常堆积。脂蛋白肾小球病（LG）于 1987 年在 Faraggiana 和 Churg 撰写的关于肾脏脂质代谢紊乱的综述中，在一名 Koitabashi 患者中被简要描述，它是一种新兴的新型储存性肾脏疾病。这种罕见且独特的肾病的特征是在扩张的肾小球毛细血管腔内存在脂蛋白血栓，与 III 型高脂蛋白血症和载脂蛋白 E（apo E）血清水平升高有关。Saito 等人对其 1989 年患者进行的几项特定研究表明，这是一种常染色体隐性遗传疾病，主要影响亚洲血统的患者，主要是日本人群，但在白种人中也很少见。该疾病可能是由于载脂蛋白 E 基因突变导致的遗传性脂质代谢改变。临床上，LG 的特征是蛋白尿，通常与肾病综合征和进行性肾功能不全相关。我们描述了 2004 年和 2009 年分别在我们肾病科就诊的 2 名意大利成年白人男性 LG 患者的病例。
