与IgA肾病相关的微小变化疾病“罕见的临床谱”:病例报告

Juan Santiago Serna-Trejos, Laura Carolina Neira–Ruiz, Stefanya Geraldine Bermúdez–Moyano, Jennifer Alejandra Hurtado-Leiton, Diego Andrés González-Sánchez
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引用次数: 0

摘要

微小改变病(MCD)是特发性肾病综合征的主要病因之一,表现为强烈的蛋白尿导致水肿和血管内体积减少,其特征是光镜下没有改变,电镜下存在足细胞过程的消失。虽然原因尚不清楚,但据信是由于足细胞和构成该细胞骨架和滤过膜的蛋白质的免疫失调和修饰,导致肾小球基底膜完整性的改变,从而调节了蛋白尿的存在。IgA肾病(IgAN)是发达国家最常见的系膜增生性肾小球疾病,也是亚洲人群慢性肾脏疾病和肾功能衰竭的主要原因之一,其特征是存在系膜IgA沉积。临床表现是非常多变的,从孤立的畸形微血尿到肾功能的慢性恶化。我们提出一个罕见的病例,这两种原发性肾小球病变同时在一个病人。这是为了提高医学敏感性,在诊断共存的这两种原发性肾小球病变作为不典型的表现在临床实践中。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Minimal change disease associated with IgA nephropathy “a rare clinical spectrum”: Case report
Minimal change disease (MCD) is one of the main causes of idiopathic nephrotic syndrome, manifesting with intense proteinuria leading to edema and intravascular volume depletion, characterized by the absence of alterations in light microscopy and the presence of effacement of the podocyte processes in electron microscopy. Although the cause is unknown, it is believed that due to an immunologic dysregulation and modifications of the podocyte and of the proteins that constitute the cytoskeleton of this one and of the filtration diaphragm, generating an alteration in the integrity of the glomerular basement membrane and, therefore, conditioning the presence of proteinuria. IgA nephropathy (IgAN) is the most common mesangial proliferative glomerulopathy in developed countries and one of the main causes of chronic kidney disease and renal failure in Asian population, characterized by the presence of mesangial IgA deposits. The clinical presentation is extremely variable, ranging from isolated dysmorphic microhematuria to chronic deterioration of renal function. We present a rare case of these two primary glomerulopathies concomitantly in one patient. This is intended to increase medical sensitivity in the diagnosis of the coexistence of these two primary glomerulopathies as atypical presentations in clinical practice.
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