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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 |