微小变化病

Shyam B. Bansal
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引用次数: 1

摘要

微小变化病是儿童肾病综合征最常见的病因,也是成人肾病综合征第三常见的病因。有新的见解在发病机制的疾病,它现在被认为是足细胞疾病。新的生物标志物已经被确定来解释发病机制。儿童的治疗几乎是标准化的,但在成人中,证据并不那么有力,治疗主要是从儿童的随机试验和成人的非对照或回顾性研究中推断出来的。儿童和类固醇敏感患者的长期预后良好。类固醇抵抗是预后不良的标志。基因研究有助于检测突变患者,因为他们对免疫抑制药物没有反应。随着他克莫司、霉酚酸酯和利妥昔单抗等新的免疫抑制药物的发现,MCD的治疗手段也越来越广泛,这些药物有助于治疗类固醇耐药和类固醇依赖性肾病综合征。
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Minimal change disease

Minimal change disease is the commonest cause of nephrotic syndrome in children and third most common cause in adults. There are new insights in the pathogenesis of disease, and it is now considered a podocyte disorder. New biomarkers have been identified to explain the pathogenesis. The treatment in children is almost standardised, however in adults, the evidence is not so robust and treatment is mostly extrapolated from randomized trials in children and uncontrolled or retrospective studies in adults. The long term prognosis of disease is excellent in children and steroid sensitive patients. Steroid resistance is a marker of poor prognosis. Genetic studies are helpful in detecting patients with mutations, as, they do not respond to immunosuppressive drugs. The therapeutic armamentarium of treatment of MCD has widened with discovery of new immunosuppressive drugs like tacrolimus, mycophenolate mofetil and rituximab, which are helpful in treatment of steroids resistant and steroid dependent nephrotic syndrome.

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