Menu Expand
Primary Glomerular Diseases - ECAB

Primary Glomerular Diseases - ECAB

Ramadas Pisharody

(2013)

Additional Information

Book Details

Abstract

The basic functional unit of kidney is the glomerulus. Any renal derangement at that level can lead to a multitude of diseases. The renal as well as glomerular disorders secondary to other causes such as hypertension, diabetes, other vasculites, drugs, malignancies, etc. have been adequately addressed. However, the primary glomerular disorders, due to an ambiguity regarding their genesis, have yet to be explored properly. Though these diseases are clubbed under the common head of "primary", they consist of various distinct subtypes with markedly different mechanisms of pathogenesis, course of the disease, response to treatment and prognoses. This clinical update explores the various types of pimary glomerular diseases with an aim to enhance the reader’s knowledge and aid in timely recognition and precise diagnosis of glomerular diseases to help chart the appropriate treatment of each individual condition.

The first chapter elucidates the clinicopathological correlation of primary glomerular diseases to help differentiate them from each other and from other similar differential diagnoses without resorting to renal biopsy in all the cases. The second, third and fourth, chapters explore the various aspects of Proliferative Glomerulonephritis, Idiopathic Nephrotic Syndrome and Heredofamilial Glomerular Diseases. The last chapter summarizes the clinically relevant current perspectives regarding the pathogenesis and treatment modalities of all the primary glomerular diseases.

This book will help the readers understand the intricacies of the aspects mentioned above and guide the practitioner to diagnose and manage Primary Glomerular Diseases with special reference to practical experience in India. The authors have put together the most relevant facts about the disease for an easy comprehension and understanding of primary glomerular diseases by practitioners and students across the specialty.


Table of Contents

Section Title Page Action Price
Front Cover\r Front Cover
Front Matter \r ia
Copyright id
ECAB Clinical Update:Nephrology ie
Primary Glomerular Diseases if
About the Authors ig
Contents im
ECAB Clinical Update InformationPrimary Glomerular Diseases i
Foreword vi
Primary Glomerular Diseases: Clinicopathologic Correlation 1a
ABSTRACT: 1a
KEYWORDS 1b
Introduction 1a
Clinical Markers of Glomerular Injury 2
Proteinuria 2
Hematuria 3
Reduced Glomerular Filtration Rate 4
Hypertension 4
Syndromes of Glomerular Disease 5
Nephrotic Syndrome 5
Acute Nephritic Syndrome 6
Rapidly Progressive Glomerulonephritis (RPGN) 6
Asymptomatic Urinary Abnormalities 6
Differenting Features of Primary Glomerular Diseases 7
Common Primary Glomerular Diseases 9
Minimal Change Disease 9
Focal Segmental Glomerulosclerosis 10
Membranous Nephropathy (MN) 10
Membranoproliferative (Mesangiocapillary) Glomerulonephritis 11
IgA Nephropathy 11
Post-infectious Glomerulonephritis 12
Conclusion 12
Proliferative Glomerulonephritis 14a
ABSTRACT 14a
KEYWORDS 14b
Introduction 14a
Pathogenesis 14a
Approach to A Possible Case of Proliferative Glomerulonephritis 16
Membranoproliferative Glomerulonephritis 17
Types of Membranoproliferative Glomerulonephritis 21
Type I Membranoproliferative Glomerulonephritis 21
Type II Membranoproliferative Glomerulonephritis 21
Type III Membranoproliferative Glomerulonephritis 21
Etiology 21
Treatment 22
Corticosteroids 22
Antiplatelet Agents 23
Prognosis 23
IgA Nephropathy 24
Pathology 24
Clinical Features 27
Synpharyngitic Hematuria 27
Microscopic Hematuria 27
Nephrotic Syndrome 27
Rare Presentations 27
Clinical Associations 27
Diagnosis 28
Treatment 28
Prognosis 28
Rapidly Progressive (Crescentic) Glomerulonephritis 29
Types of Crescentic Glomerulonephritis 29
Clinical Presentation 33
Treatment 34
Post-Streptococcal Glomerulonephritis 34
Pathogenesis 35
Pathology 35
Clinical Manifestations 37
Laboratory Findings 37
Diagnosis 38
Management 38
Course of Disease 39
Prognosis 39
Conclusion 42
Case Studies 45
Proliferative Glomerulonephritis 45
Case 1 45
History 45
Examination 45
Discussion 46
Case 2 47
History 47
Examination 47
Discussion 48
Idiopathic Nephrotic Syndrome 49a
ABSTRACT 49a
KEYWORDS 49b
Introduction 49a
Epidemiology and Demography 50
Epidemiology 50
Age and Gender Predominance 51
Familial Occurrence 52
Pathogenesis and Pathology 52
Pathogenesis of Idiopathic Nephrotic Syndrome 52
Cell-mediated Immunity (CMI) 52
Humoral Immunity 52
Circulating Immune Complexes 53
Complement Pathway Activation 53
Circulating Permeability Factors 53
Allergy and Atopy 53
Genetics in Minimal Change Disease 53
Association with HLA 54
Minimal Change Disease as a Disease of Podocytes 54
Pathology 54
Clinical Features 55
Evaluation 58
Management of Childhood Idiopathic Nephrotic Syndrome 63
Specific Therapy for Induction of Remission 63
Supportive Care 68
Immunization 69
Treatment of Relapse 70
Alternate Treatment in Steroid-dependent Nephrotic Syndrome and Frequently-relapsing Nephrotic Syndrome 71
Levamisole 71
Cyclophosphamide (CYC) 71
Cyclosporine (CsA) 72
Tacrolimus (TAC) 73
Mycophenolate Mofetil (MMF) 73
Rituximab (RTX) 74
Steroid-Resistant Nephrotic Syndrome 74
Management of Steroid Resistance 74
Renoprotection Strategies in Steroid-Resistant Nephrotic Syndrome 75
Complications 76
Infections 76
Hypovolemia 77
Hypertension 77
Thrombosis 78
Acute Renal Failure (ARF) 78
Malnutrition 79
Steroid Toxicity 79
Indian Scenario 80
Personal Experience 80
Prognosis and Outcome 82
Conclusion 83
Heredofamilial Glomerular Diseases 86a
ABSTRACT 86a
KEYWORDS 86b
Introduction 86a
Importance of Podocytes and Glomerular Basement Membrane 88
Podocytopathies 89
Heredofamilial Minimal Change Nephritic Syndrome 89
Heredofamilial Focal Segmental Glomerulosclerosis 92
Congenital Nephrotic Syndrome 96
Infantile and Childhood Nephrotic Syndrome 97
Diffuse Mesangial Hypercellularity 98
Diffuse Mesangial Sclerosis 98
Collapsing Glomerulopathy 100
GBM Abnormality in Alport's Syndrome 100
Heredofamilial Glomerular Diseases in India 102
Therapeutic Implications 104
Role of Genetic Counseling 105
Recurrence of Proteinuria After Renal Transplantation 105
Conclusion 107
Primary Glomerular Diseases-An Overview of Current Perspectives 109a
ABSTRACT 109a
KEYWORDS 109b
Introduction 109a
IgA Nephropathy 110
Pathogenesis 110
Prognostic Factors 111
Management 112
General Guidelines 112
Specific Management 112
Patients with Rapidly Declining Glomerular Filtration Rate (GFR): 112
Patients with Nephrotic Syndrome: 112
Patients with Slowly Progressive Renal Impairment: 112
Membranoproliferative Glomerulonephritis 115
Pathogenesis 115
Type I Membranoproliferative Glomerulonephritis 115
Type II Membranoproliferative Glomerulonephritis 116
Type III Membranoproliferative Glomerulonephritis 117
Clinical Features and Natural History 117
Treatment of Membranoproliferative Glomerulonephritis 117
Corticosteroid Therapy 119
Antiplatelet Agents and Anticoagulants 119
Cytotoxic Therapy 119
Complement Modulating Therapies 119
Membranous Nephropathy 120
Pathology 120
Pathogenesis 121
Clinical Course and Prognosis 121
Treatment of Membranous Nephropathy 122
General Strategy 122
Nephrotic Syndrome without Renal Insufficiency 124
Recommended Approaches: 124
Alternate Therapy: 124
Nephrotic Syndrome with Renal Insufficiency 125
Cyclosporine (CsA): 125
Alternate Month Corticosteroids and Cyclophosphamide for 6 Months: 125
Alternate Month Steroids and Chlorambucil for 6 Months: 125
Daily Oral Cyclophosphamide and Steroids for 1 Year: 125
Newer Agents: 125
Minimal Change Nephrotic Syndrome or Minimal Change Disease 126
Pathophysiology 126
Natural History of Minimal Change Disease in Adults 126
Management 126
Focal Segmental Glomerulosclerosis 127
Pathology 127
Pathogenesis 128
Clinical Features and Risk Factors for Progression 128
Therapeutic Options 128
Idiopathic Focal Segmental Glomerulosclerosis 129
Postadaptive Focal Segmental Glomerulosclerosis: 129
Genetic Focal Segmental Glomerulosclerosis: 129
Response to Treatment 130
Conclusion 130