Sindrome nefrotico esteroide resistente

	Kidney Int. 2008 Nov;74(9):1209-15. Epub 2008 Jul 2. Steroid-resistant nephrotic syndrome. Kitamura A, Tsukaguchi H, Maruyama K, Shono A, Iijima K, Kagami S, Doi T. Department of Pediatrics, University of Tokushima Graduate School, Tokushima, Japan. This case illustrates the usefulness of genetic approaches to explore the pathological basis for SRNS children. A screening for the NPSH2 mutations is clinically important in nonresponder children to an initial steroid therapy even in the absence of family history. Such genetic information helps clinicians to choose therapeutic options to reduce a risk of unnecessary side effects. A genetic defect in podocyte slit diaphragm complex and/or its anchorage to actin cytoskeleton leads to FSGS. In this context, some researchers proposed a new nosological approach, classifying certain histological patterns with an etiological commonality involving podocyte injury as ‘podocytopathies’.5,20 Delineation of the various forms of FSGS on the genetic basis may allow better understanding of pathophysiology and improve therapeutic approaches. The genetic testing will help guide treatment decision to avoid a prolonged unnecessary steroid therapy, even before obtaining a diagnostic FSGS lesion.
	PMID: 18596732 [PubMed - indexed for MEDLINE]