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Pathogenetische Aspekte des nephrotischen Syndroms

Der Internist volume 44, pages 1075–1082 (2002)Cite this article

Zusammenfassung

Das nephrotische Syndrom ist gekennzeichnet durch Eiweißverlust im Urin, Hypoalbuminämie, Hyperlipidämie und Ödeme. Verschiedene Erkrankungen verursachen ein nephrotisches Syndrom, indem sie zu einer Schädigung des glomerulären Podozyten führen. Diese spezialisierte Epithelzelle bildet zusammen mit dem kapillären Endothel und der glomerulären Basalmembran die Filtrationsbarriere, welche den Übertritt von Eiweiß aus der Zirkulation in das Primärfiltrat verhindert. Störungen dieses Filters führen zur Proteinurie. Die 3 häufigsten primären glomerulären Erkrankungen, welche ein nephrotisches Syndrom auslösen, sind Minimal-change-Disease, membranöse Glomerulonephritis und die primäre fokale segmentale Glomerulosklerose. Selten sind die familiären Formen des nephrotischen Syndroms; die Charakterisierung der genetischen Defekte hat jedoch wesentlich zum Verständnis der Podozytenfunktion und der Pathogenese des nephrotischen Syndroms beigetragen.

Abstract

Nephrotic syndrome is characterized by protein loss in the urine, hypoalbuminemia, hyperlipidemia and edema. Several diseases cause a nephrotic syndrome, as they damage the glomerular podocytes. These specialized epithelial cells, together with endothelial cells of the glomerular capillaries and the basal membrane, form a filter that retains plasma proteins in the circulation. A disturbance of this filter causes proteinuria. The three most common primary glomerular diseases are minimal change, membranous glomerulonephritis, and the primary focal segmental glomerulosclerosis. The familiar forms are rare; however, the identification of the relevant gene defects has greatly advanced our understanding of podocyte function as well as the pathogenesis of nephrotic syndrome.

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Danksagung

Ich möchte an dieser Stelle meinen Lehrern in Boston, Dr. Helmut Rennke, Brigham and Women's Hospital, und Dr. Bud Rose, Beth Israel Deaconess Medical Center, danken.

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Affiliations

  1. Medizinische Klinik IV, Universitätsklinikum , Freiburg

    G. Walz

  2. Medizinische Klinik IV, Universitätsklinikum , Hugstetter Str., 79106, Freiburg

    G. Walz

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  1. G. Walz
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Correspondence to G. Walz.

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Walz, G. Pathogenetische Aspekte des nephrotischen Syndroms. Internist 44, 1075–1082 (2002). https://doi.org/10.1007/s00108-003-1031-4

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  • DOI: https://doi.org/10.1007/s00108-003-1031-4

Schlüsselwörter

  • Nephrotisches Syndrom
  • Podozyte
  • Schlitzmembran
  • Ödeme
  • Nephrin

Keywords

  • Nephrotic syndrome
  • Podocyte
  • Slit diaphragm
  • Edema
  • Nephrin