微小变化病
医学
肾病综合征
足细胞
局灶节段性肾小球硬化
肾小球肾炎
疾病
鉴别诊断
肾活检
肾病科
病理
病理生理学
活检
内科学
肾
蛋白尿
作者
Joachim Müller-Deile,Henk Schenk,Marianne Schiffer
出处
期刊:Der Internist
[Springer Nature]
日期:2019-05-01
卷期号:60 (5): 450-457
被引量:9
标识
DOI:10.1007/s00108-019-0590-y
摘要
Minimal change disease (MCD) or minimal change glomerulonephritis and focal segmental glomerulosclerosis (FSGS) are the two major causes of nephrotic syndrome in children and young adults. Both disease entities resemble each other and can sometimes only be discriminated on the basis of their clinical courses. MCD and FSGS display two classical examples that share a common pathophysiology in which the glomerular podocyte and the cytoskeleton of its foot processes play important roles. Therefore, the term "podocytopathy" was introduced for both diseases. In this article, we compare their differences and similarities, and summarized new data on pathophysiology and treatment. In adults, only a renal biopsy including electron microscopy allows for the discrimination of MCD and FSGS and other differential diagnoses. The identification of a primary or secondary form of the disease is based on the clinical course. Data from studies on the treatment are sparse; hence, treatment is still based on high-dose steroids followed by additional immunosuppressive agents. In secondary forms, treatment of the underlying disease is elementary.
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