Djamali A, Cristol J P, Turc-Baron C, Beucler I, Baldet P, Mourad G
Service de Néphrologie, Hôpital Lapeyronie, CHU de Montpellier, Paris.
Presse Med. 1996 May 18;25(17):798-802.
Lipoprotein glomerulopathy is defined by the presence of lipidic deposits in the capillary lumen giving them a dialted and microaneurysmal aspect and the presence of quantitative and/or qualitative alterations of plasma apolipoprotein E. We describe here the long-term follow-up of a young female patient who presented with corticoresistant nephrotic syndrome in 1979 and progressed to chronic renal failure requiring dialysis in 1990. The three renal biopsies performed during the follow-up showed markedly enlarged capillary loops due to intra-luminal fibrinolipidic material deposition forming true intracapillary thrombi. She received a cadaver renal transplant in 1993. One year after transplantation, nephrotic syndrome reappeared and graft biopsy showed recurrence of the initial glomerular disease on the transplant. The plasma lipid profile showed hypercholesterolemia, hypertriglyceridemia, and elevated plasma ApoE levels with an abnormal Apo E phenotype. Our case report is a new typical case of lipoprotein glomerulopathy with recurrence of the initial disease on the renal allograft.
脂蛋白肾小球病的定义是毛细血管腔内存在脂质沉积，使其呈现扩张和微动脉瘤样外观，以及血浆载脂蛋白E存在定量和/或定性改变。我们在此描述一名年轻女性患者的长期随访情况，该患者于1979年出现皮质激素抵抗性肾病综合征，并于1990年进展为需要透析的慢性肾衰竭。随访期间进行的三次肾活检显示，由于管腔内纤维脂质物质沉积形成真正的毛细血管内血栓，毛细血管袢明显增宽。她于1993年接受了尸体肾移植。移植后一年，肾病综合征再次出现，移植肾活检显示移植肾出现了最初肾小球疾病的复发。血浆脂质谱显示高胆固醇血症、高甘油三酯血症，血浆载脂蛋白E水平升高且载脂蛋白E表型异常。我们的病例报告是脂蛋白肾小球病的一个新的典型病例，移植肾出现了最初疾病的复发。
