
Lesley Rees
et al.
Published online:
01 March 2019
Published in print:
01 February 2019
Online ISBN:
9780191826863
Print ISBN:
9780198784272
Contents
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Overview of inherited glomerular diseases Overview of inherited glomerular diseases
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Basic principles Basic principles
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Nephrotic syndrome Nephrotic syndrome
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Further reading Further reading
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Further reading Further reading
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Alport syndrome and thin basement membrane nephropathy Alport syndrome and thin basement membrane nephropathy
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Definitions Definitions
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Genetics Genetics
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Histology Histology
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Clinical features Clinical features
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Treatment Treatment
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Further reading Further reading
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Further reading Further reading
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Female carriers of X-linked Alport syndrome Female carriers of X-linked Alport syndrome
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Further reading Further reading
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Acute nephritis (acute nephritic syndrome) Acute nephritis (acute nephritic syndrome)
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Definition Definition
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Differential diagnosis of acute nephritis in children Differential diagnosis of acute nephritis in children
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Post-streptococcal glomerulonephritis Post-streptococcal glomerulonephritis
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Causes of hypocomplementaemic nephritis Causes of hypocomplementaemic nephritis
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Investigation of acute nephritis: generic investigations for all patients Investigation of acute nephritis: generic investigations for all patients
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Specific tests for certain patients Specific tests for certain patients
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Indications/considerations for renal biopsy Indications/considerations for renal biopsy
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Management of acute nephritis Management of acute nephritis
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General management of acute nephritis General management of acute nephritis
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Immunoglobulin A nephropathy Immunoglobulin A nephropathy
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Background Background
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Aetiology Aetiology
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Clinical and laboratory findings Clinical and laboratory findings
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Diagnosis Diagnosis
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Treatment Treatment
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Doses Doses
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Further reading Further reading
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Further reading Further reading
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Nephrotic syndromes: definitions Nephrotic syndromes: definitions
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Congenital and infantile nephrotic syndromes Congenital and infantile nephrotic syndromes
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Definitions Definitions
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Congenital nephrotic syndrome Congenital nephrotic syndrome
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NPHS1 (Finnish type) CNS NPHS1 (Finnish type) CNS
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Other primary causes of CNS Other primary causes of CNS
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Diffuse mesangial sclerosis Diffuse mesangial sclerosis
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Minimal change disease and focal segmental glomerulosclerosis Minimal change disease and focal segmental glomerulosclerosis
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Secondary causes of CNS Secondary causes of CNS
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The role of renal biopsy in CNS and infantile nephrotic syndrome The role of renal biopsy in CNS and infantile nephrotic syndrome
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Management of NPHS1 (Finnish type) CNS Management of NPHS1 (Finnish type) CNS
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Reduction of urinary protein losses Reduction of urinary protein losses
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Replacement of albumin Replacement of albumin
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Symptom treatment Symptom treatment
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Transplantation Transplantation
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Other important factors Other important factors
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Management of other forms of CNS Management of other forms of CNS
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Transient neonatal nephrosis (congenital membranous nephropathy) Transient neonatal nephrosis (congenital membranous nephropathy)
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Steroid-sensitive nephrotic syndrome: presenting episode Steroid-sensitive nephrotic syndrome: presenting episode
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Introduction Introduction
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Key points about minimal change disease Key points about minimal change disease
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Investigations at first presentation Investigations at first presentation
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Indication for renal biopsy at disease presentation Indication for renal biopsy at disease presentation
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Management Management
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Steroids Steroids
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Infection Infection
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Fluid balance, hypovolaemia, and hypervolaemia Fluid balance, hypovolaemia, and hypervolaemia
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Diet Diet
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Hypercoagulability Hypercoagulability
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Information Information
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Steroid-sensitive nephrotic syndrome: relapsing disease Steroid-sensitive nephrotic syndrome: relapsing disease
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Management of initial relapses Management of initial relapses
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Infrequently relapsing disease Infrequently relapsing disease
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Management of frequently relapsing and steroid-dependent SSNS Management of frequently relapsing and steroid-dependent SSNS
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Notes on drug therapy Notes on drug therapy
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Long-term, low-dose maintenance steroid therapy Long-term, low-dose maintenance steroid therapy
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Levamisole Levamisole
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Alkylating agents: cyclophosphamide and chlorambucil Alkylating agents: cyclophosphamide and chlorambucil
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Ciclosporin Ciclosporin
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Longer-term adverse effects Longer-term adverse effects
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Other agents Other agents
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Secondary steroid resistance Secondary steroid resistance
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Further reading Further reading
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Further reading Further reading
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Steroid-resistant nephrotic syndrome Steroid-resistant nephrotic syndrome
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Primary steroid-resistant nephrotic syndrome Primary steroid-resistant nephrotic syndrome
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Introduction Introduction
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Histology Histology
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Genetics Genetics
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Outcomes Outcomes
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Treatment Treatment
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Immunosuppression Immunosuppression
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Non-immunosuppressive treatment Non-immunosuppressive treatment
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Secondary steroid-resistant nephrotic syndrome Secondary steroid-resistant nephrotic syndrome
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Further reading Further reading
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Further reading Further reading
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Fluid balance, hypovolaemia, and hypervolaemia in nephrotic syndrome Fluid balance, hypovolaemia, and hypervolaemia in nephrotic syndrome
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C3 glomerulopathy and immune complex-mediated glomerulonephritis C3 glomerulopathy and immune complex-mediated glomerulonephritis
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C3 glomerulopathy C3 glomerulopathy
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Differentiation of C3GN from PIGN Differentiation of C3GN from PIGN
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Treatment of C3G Treatment of C3G
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Non-evidence-based suggestions for treatment Non-evidence-based suggestions for treatment
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Renal transplantation Renal transplantation
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Immune complex-mediated glomerulonephritis (ICGN) Immune complex-mediated glomerulonephritis (ICGN)
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Treatment of ICGN Treatment of ICGN
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Reference Reference
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Reference Reference
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Membranous nephropathy Membranous nephropathy
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Introduction Introduction
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Presenting clinical features Presenting clinical features
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Diagnosis Diagnosis
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Treatment Treatment
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Secondary MN Secondary MN
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Idiopathic MN Idiopathic MN
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Alkylating agents Alkylating agents
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Calcineurin inhibitors (the authors’ personal preference) Calcineurin inhibitors (the authors’ personal preference)
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Corticosteroid monotherapy Corticosteroid monotherapy
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Other immunosuppressive treatments Other immunosuppressive treatments
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Non-immunosuppressive therapy Non-immunosuppressive therapy
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Children with asymptomatic proteinuria alone (i.e. no hypertension, renal impairment, or nephrotic syndrome) Children with asymptomatic proteinuria alone (i.e. no hypertension, renal impairment, or nephrotic syndrome)
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References References
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References References
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Chapter
9 C9Glomerular disease
Get access
Pages
C9–C9.P690
-
Published:February 2019
Cite
Rees, Lesley, and others, 'Glomerular disease', Paediatric Nephrology, 3 edn, Oxford Specialist Handbooks in Paediatrics (Oxford , 2019; online edn, Oxford Academic, 1 Mar. 2019), https://doi.org/10.1093/med/9780198784272.003.0009, accessed 3 May 2025.
Abstract
Understanding of the pathogenesis of nephrotic syndrome is rapidly increasing and new treatments are evolving. However, these diseases remain difficult to manage, with little evidence to help decide on optimum treatment strategies.
Keywords:
Alport syndrome, thin basement membrane nephropathy, acute nephritis, IgA nephropathy, nephrotic syndrome, steroid-sensitive nephrotic syndrome, steroid-resistant nephrotic syndrome, fluid balance, C3 glomerulopathy, membranous nephropathy
Subject
Paediatrics
Collection:
Oxford Medicine Online
Disclaimer
Oxford University Press makes no representation, express or implied, that the drug dosages in this book are correct.
Readers must therefore always …
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Oxford University Press makes no representation, express or implied, that the drug dosages in this book are correct.
Readers must therefore always check the product information and clinical procedures with the most up to date published product information and data sheets
provided by the manufacturers and the most recent codes of conduct and safety regulations. The authors and the publishers do not accept responsibility or
legal liability for any errors in the text or for the misuse or misapplication of material in this work. Except where otherwise stated, drug dosages
and recommendations are for the non-pregnant adult who is not breastfeeding.
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