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1 results

  • Chapter 3 - Idiopathic and Hereditary Proteinuric Glomerular Diseases

  • from Section 2 - Glomerular Diseases
  • Edited by Helen Liapis, Ludwig Maximilian University, Nephrology Center, Munich, Adjunct Professor and Washington University St Louis, Department of Pathology and Immunology, Retired Professor
  • Book:
    Pediatric Nephropathology & Childhood Kidney Tumors
    Published online:
    10 August 2023
    Print publication:
    09 March 2023, pp 27-55

  • 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.”