Book contents
- Pediatric Nephropathology & Childhood Kidney Tumors
- Diagnostic Pediatric Pathology
- Pediatric Nephropathology & Childhood Kidney Tumors
- Copyright page
- Dedication
- Contents
- Contributors
- Preface
- Section 1 Normal and Abnormal Human Kidney Development
- Section 2 Glomerular Diseases
- Chapter 3 Idiopathic and Hereditary Proteinuric Glomerular Diseases
- Chapter 4 Haematuric Glomerular Diseases (Collagenopathies)
- Chapter 5 Immune Complex Mediated Glomerular Diseases
- Chapter 6 Inherited Metabolic Diseases Affecting the Kidney
- Chapter 7 Glomerular Diseases with Fibrillary Deposits
- Chapter 8 Mitochondrial Diseases in the Pediatric Kidney
- Section 3 Tubulointerstitial Diseases
- Section 4 Vascular Diseases
- Section 5 Infectious Diseases
- Section 6 Cystic Diseases
- Section 7 Solid Tumors of the Kidney
- Section 8 Transplant Pathology of the Kidney
- Index
- References
Chapter 3 - Idiopathic and Hereditary Proteinuric Glomerular Diseases
from Section 2 - Glomerular Diseases
Published online by Cambridge University Press: 10 August 2023
- Pediatric Nephropathology & Childhood Kidney Tumors
- Diagnostic Pediatric Pathology
- Pediatric Nephropathology & Childhood Kidney Tumors
- Copyright page
- Dedication
- Contents
- Contributors
- Preface
- Section 1 Normal and Abnormal Human Kidney Development
- Section 2 Glomerular Diseases
- Chapter 3 Idiopathic and Hereditary Proteinuric Glomerular Diseases
- Chapter 4 Haematuric Glomerular Diseases (Collagenopathies)
- Chapter 5 Immune Complex Mediated Glomerular Diseases
- Chapter 6 Inherited Metabolic Diseases Affecting the Kidney
- Chapter 7 Glomerular Diseases with Fibrillary Deposits
- Chapter 8 Mitochondrial Diseases in the Pediatric Kidney
- Section 3 Tubulointerstitial Diseases
- Section 4 Vascular Diseases
- Section 5 Infectious Diseases
- Section 6 Cystic Diseases
- Section 7 Solid Tumors of the Kidney
- Section 8 Transplant Pathology of the Kidney
- Index
- References
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.”
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- Pediatric Nephropathology & Childhood Kidney Tumors , pp. 27 - 55Publisher: Cambridge University PressPrint publication year: 2023