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Chapter 3 - Idiopathic and Hereditary Proteinuric Glomerular Diseases

from Section 2 - Glomerular Diseases

Published online by Cambridge University Press:  10 August 2023

By
Danica Galešić Ljubanović  and
Parker Wilson
Edited by
Helen Liapis
Helen Liapis
Affiliation:
Ludwig Maximilian University, Nephrology Center, Munich, Adjunct Professor and Washington University St Louis, Department of Pathology and Immunology, Retired Professor
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Summary

This chapter discusses exclusively the pathology and molecular genetics of non-immune-complex mediated glomerular diseases characterized predominantly by proteinuria due to defects in the podocyte filtration barrier. The clinical classification of steroid sensitive and steroid resistant nephrotic syndrome and histopathology patterns are discussed in parallel or interchangeably. More than 50 podocyte gene mutations associated with nephrotic syndrome have been identified. These have changed the management and general outlook of childhood nephrotic glomerular diseases. A detail account of the genetics of nephrotic syndrome associated glomerular pathologies is provided in the section “Molecular Pathology.”

Keywords

Podocytopathynephrotic syndrome (NS)steroid sensitive NS (SSNS)steroid resistant NS (SRNS)minimal change disease (MCD)focal segmental glomerulosclerosis (FSGS)collapsing glomerulopathy (CGP)diffuse mesangial sclerosis (DMS)congenital nephrotic syndrome (CNS)nephrin (NPHS1)podocin (NPHS2)
Type
Chapter
Information
Pediatric Nephropathology & Childhood Kidney Tumors , pp. 27 - 55
Publisher: Cambridge University Press
Print publication year: 2023

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