Barisoni Laura, Schnaper H William, Kopp Jeffrey B
Department of Pathology, New York University School of Medicine, New York, New York, USA.
Clin J Am Soc Nephrol. 2007 May;2(3):529-42. doi: 10.2215/CJN.04121206. Epub 2007 Apr 11.
A spectrum of proteinuric glomerular diseases results from podocyte abnormalities. The understanding of these podocytopathies has greatly expanded in recent years, particularly with the discovery of more than a dozen genetic mutations that are associated with loss of podocyte functional integrity. It is apparent that classification of the podocytopathies on the basis of morphology alone is inadequate to capture fully the complexity of these disorders. Herein is proposed a taxonomy for the podocytopathies that classifies along two dimensions: Histopathology, including podocyte phenotype and glomerular morphology (minimal-change nephropathy, focal segmental glomerulosclerosis, diffuse mesangial sclerosis, and collapsing glomerulopathy), and etiology (idiopathic, genetic, and reactive forms). A more complete understanding of the similarities and differences among podocyte diseases will help the renal pathologist and the nephrologist communicate more effectively about the diagnosis; this in turn will help the nephrologist provide more accurate prognostic information and select the optimal therapy for these often problematic diseases. It is proposed that final diagnosis of the podocytopathies should result from close collaboration between renal pathologists and nephrologists and should whenever possible include three elements: Morphologic entity, etiologic form, and specific pathogenic mechanism or association.
一系列蛋白尿性肾小球疾病是由足细胞异常引起的。近年来，对这些足细胞病的认识有了极大的扩展，尤其是发现了十多种与足细胞功能完整性丧失相关的基因突变。显然，仅基于形态学对足细胞病进行分类不足以充分体现这些疾病的复杂性。本文提出了一种足细胞病分类法，该分类法从两个维度进行分类：组织病理学，包括足细胞表型和肾小球形态（微小病变肾病、局灶节段性肾小球硬化、弥漫性系膜硬化和塌陷性肾小球病），以及病因（特发性、遗传性和反应性形式）。对足细胞疾病之间异同的更全面理解将有助于肾脏病理学家和肾病学家就诊断进行更有效的沟通；这反过来又将帮助肾病学家提供更准确的预后信息，并为这些常常棘手的疾病选择最佳治疗方法。建议足细胞病的最终诊断应由肾脏病理学家和肾病学家密切合作得出，并且应尽可能包括三个要素：形态学实体、病因形式以及特定的致病机制或关联。
